All Publications from Prof. Dr. med. Albert C. Ludolph

• Abdelhak A, Foschi M, Abu-Rumeileh S, Yue JK, D'Anna L, Huss A, Oeckl P, Ludolph AC, Kuhle J, Petzold A, Manley GT, Green AJ, Otto M, Tumani H. Blood GFAP as an emerging biomarker in brain and spinal cord disorders. Nat Rev Neurol. 2022 Mar;18(3):158-172. doi: 10.1038/s41582-021-00616-3. Epub 2022 Feb 3.
• Albanese A, Ludolph AC, McDermott CJ, Corcia P, Van Damme P, Van den Berg LH, Hardiman O, Rinaldi G, Vanacore N, Dickie B; TUDCA-ALS Study Group. Tauroursodeoxycholic acid in patients with amyotrophic lateral sclerosis: The TUDCA-ALS trial protocol. Front Neurol. 2022 Sep 27;13:1009113. doi: 10.3389/fneur.2022.1009113. eCollection 2022.
• Althaus K, Kasel M, Ludolph AC, Kassubek J, Kassubek R. HARM revisited: Etiology of subarachnoid hyperintensities in brain FLAIR MRI. Int J Stroke. 2022 Jan 5:17474930211064754. doi: 10.1177/17474930211064754. Online ahead of print.
• Andersen PM, Kuźma-Kozakiewicz M, Keller J, Maksymowicz-Śliwińska A, Barć K, Nieporęcki K, Finsel J, Vazquez C, Helczyk O, Linse K, Häggström AC, Stenberg E, Semb O, Ciećwierska K, Szejko N, Uttner I, Herrmann A, Petri S, Meyer T, Ludolph AC, Lulé D. Caregivers' divergent perspectives on patients' well-being and attitudes towards hastened death in Germany, Poland and Sweden. Amyotroph Lateral Scler Frontotemporal Degener. 2022 May;23(3-4):252-262. doi: 10.1080/21678421.2021.1936064. Epub 2021 Jun 30.
• Barć K, Lulé D, Finsel J, Helczyk O, Baader S, Aho-Özhan H, Ludolph AC, Kuźma-Kozakiewicz M. Well-being on supportive techniques in amyotrophic lateral sclerosis: from neurologists' perspective. Ann Clin Transl Neurol. 2022 Nov 29. doi: 10.1002/acn3.51663. Online ahead of print.
• Barschke P, Abu-Rumeileh S, Al Shweiki MHDR, Barba L, Paolini Paoletti F, Oeckl P, Steinacker P, Halbgebauer S, Gaetani L, Lewerenz J, Ludolph AC, Landwehrmeyer GB, Parnetti L, Otto M. Cerebrospinal fluid levels of proenkephalin and prodynorphin are differentially altered in Huntington's and Parkinson's disease. J Neurol. 2022 Sep;269(9):5136-5143. doi: 10.1007/s00415-022-11187-8. Epub 2022 Jun 23.
• Behler A, Müller HP, Del Tredici K, Braak H, Ludolph AC, Lulé D, Kassubek J. Multimodal in vivo staging in amyotrophic lateral sclerosis using artificial intelligence. Ann Clin Transl Neurol. 2022 Jul;9(7):1069-1079. doi: 10.1002/acn3.51601. Epub 2022 Jun 9.
• Behler A, Müller HP, Ludolph AC, Lulé D, Kassubek A multivariate Bayesian classification algorithm for cerebral stage prediction by diffusion tensor imaging in amyotrophic lateral sclerosis. J. Neuroimage Clin. 2022 Jun 21;35:103094. doi: 10.1016/j.nicl.2022.103094. Online ahead of print.
• Behler A, Lulé D, Ludolph AC, Kassubek J, Müller HP. Longitudinal monitoring of amyotrophic lateral sclerosis by diffusion tensor imaging: Power calculations for group studies. Front Neurosci. 2022 Aug 10;16:929151. doi: 10.3389/fnins.2022.929151. eCollection 2022.
• Binz C, Osmanovic A, Thomas NH, Stolte B, Freigang M, Cordts I, Griep R, Uzelac Z, Wurster CD, Kamm C, Siegler HA, Wieselmann G, Hermann A, Lingor P, Deschauer M, Ludolph AC, Meyer T, Günther R, Hagenacker T, Petri S, Schreiber-Katz O. Validity and reliability of the German multidimensional fatigue inventory in spinal muscular atrophy. Ann Clin Transl Neurol. 2022 Mar;9(3):351-362. doi: 10.1002/acn3.51520. Epub 2022 Feb 22.
• Borroni B, Graff C, Hardiman O, Ludolph AC, Moreno F, Otto M, Piccininni M, Remes AM, Rowe JB, Seelaar H, Stefanova E, Traykov L, Logroscino G; FRONTIERS. FRONTotemporal dementia Incidence European Research Study-FRONTIERS: Rationale and design. Alzheimers Dement. 2022 Mar;18(3):498-506. doi: 10.1002/alz.12414. Epub 2021 Aug 2.
• Brenner D, Müller K, Lattante S, Yilmaz R, Knehr A, Freischmidt A, Ludolph AC, Andersen PM, Weishaupt JH. FUS mutations dominate TBK1 mutations in FUS/TBK1 double-mutant ALS/FTD pedigrees. Neurogenetics. 2022 Jan;23(1):59-65. doi: 10.1007/s10048-021-00671-4. Epub 2021 Sep 13. PMID: 34518945
• Buck E, Oeckl P, Grozdanov V, Bopp V, Kühlwein JK, Ruf WP, Wiesner D, Roselli F, Weishaupt JH, Ludolph AC, Otto M, Danzer KM. Increased NF-L levels in the TDP-43G298S ALS mouse model resemble NF-L levels in ALS patients.Acta Neuropathol. 2022 Jul;144(1):161-164. doi: 10.1007/s00401-022-02436-1. Epub 2022 May 18.
• Dam T, Boxer AL, Golbe LI, Höglinger GU, Morris HR, Litvan I, Lang AE, Corvol JC, Aiba I, Grundman M, Yang L, Tidemann-Miller B, Kupferman J, Harper K, Kamisoglu K, Wald MJ, Graham DL, Gedney L, O'Gorman J, Haeberlein SB; PASSPORT Study Group. Author Correction: Safety and efficacy of anti-tau monoclonal antibody gosuranemab in progressive supranuclear palsy: a phase 2, randomized, placebo-controlled trial. Nat Med. 2022 Oct 17. doi: 10.1038/s41591-022-02076-8. Online ahead of print.
• Dorst J, Doenz J, Kandler K, Dreyhaupt J, Tumani H, Witzel S, Schuster J, Ludolph AC. Fat-rich versus carbohydrate-rich nutrition in ALS: a randomised controlled study. J Neurol Neurosurg Psychiatry. 2022 Mar;93(3):298-302. doi: 10.1136/jnnp-2021-328331. Epub 2022 Jan 12.
• Erhart DK, Ludolph AC, Althaus K. Cerebral infarctions following an increase in corticosteroids: an atypical case of reversible cerebral vasoconstriction syndrome. J Neurol. 2022 May 16. doi: 10.1007/s00415-022-11170-3. Online ahead of print.
• Erhart DK, Ludolph AC, Althaus K. RCVS: by clinicians for clinicians-a narrative review.J Neurol. 2022 Oct 28:1-16. doi: 10.1007/s00415-022-11425-z. Online ahead of print.
• Fahmy N, Müller K, Andersen PM, Marklund SL, Otto M, Ludolph AC, Hamdi N. A novel homozygous p.Ser69Pro SOD1 mutation causes severe young-onset ALS with decreased enzyme activity. J Neurol. 2022 Dec 6. doi: 10.1007/s00415-022-11489-x. Online ahead of print.
• Finsel J, Uttner I, Vázquez Medrano CR, Ludolph AC, Lulé D. Cognition in the course of ALS-a meta-analysis.  Amyotroph Lateral Scler Frontotemporal Degener. 2022 Jul 22:1-12. doi: 10.1080/21678421.2022.2101379. Online ahead of print. PMID: 35866707
• Freigang M, Steinacker P, Wurster CD, Schreiber-Katz O, Osmanovic A, Petri S, Koch JC, Rostásy K, Huss A, Tumani H, Winter B, Falkenburger B, Ludolph AC, Otto M, Hermann A, Günther R. Glial fibrillary acidic protein in cerebrospinal fluid of patients with spinal muscular atrophy.Ann Clin Transl Neurol. 2022 Aug 11. doi: 10.1002/acn3.51645. Online ahead of print.
• Halbgebauer S, Steinacker P, Verde F, Weishaupt J, Oeckl P, von Arnim C, Dorst J, Feneberg E, Mayer B, Rosenbohm A, Silani V, Ludolph AC, Otto M. Comparison of CSF and serum neurofilament light and heavy chain as differential diagnostic biomarkers for ALS. J Neurol Neurosurg Psychiatry. 2022 Jan;93(1):68-74. doi: 10.1136/jnnp-2021-327129. Epub 2021 Aug 20.
• Halbgebauer S, Steinacker P, Riedel D, Oeckl P, Anderl-Straub S, Lombardi J, von Arnim CAF, Nagl M, Giese A, Ludolph AC, Otto M. Visinin-like protein 1 levels in blood and CSF as emerging markers for Alzheimer's and other neurodegenerative diseases. Alzheimers Res Ther. 2022 Nov 22;14(1):175. doi: 10.1186/s13195-022-01122-4.
• Halbgebauer S, Abu-Rumeileh S, Oeckl P, Steinacker P, Roselli F, Wiesner D, Mammana A, Beekes M, Kortazar-Zubizarreta I, Perez de Nanclares G, Capellari S, Giese A, Castilla J, Ludolph AC, Žáková D, Parchi P, Otto M. Blood β-Synuclein and Neurofilament Light Chain During the Course of Prion Disease.Neurology. 2022 Feb 2:10.1212/WNL.0000000000200002. doi: 10.1212/WNL.0000000000200002. Online ahead of print.
• Halbgebauer S, Steinacker P, Hengge S, Oeckl P, Abu Rumeileh S, Anderl-Straub S, Lombardi J, Von Arnim CAF, Giese A, Ludolph AC, Otto M. CSF levels of SNAP-25 are increased early in Creutzfeldt-Jakob and Alzheimer's disease. J Neurol Neurosurg Psychiatry. 2022 Aug 22:jnnp-2021-328646. doi: 10.1136/jnnp-2021-328646. Online ahead of print.
• Hebestreit H, Zeidler C, Schippers C, de Zwaan M, Deckert J, Heuschmann P, Krauth C, Bullinger M, Berger A, Berneburg M, Brandstetter L, Deibele A, Dieris-Hirche J, Graessner H, Gündel H, Herpertz S, Heuft G, Lapstich AM, Lücke T, Maisch T, Mundlos C, Petermann-Meyer A, Müller S, Ott S, Pfister L, Quitmann J, Romanos M, Rutsch F, Schaubert K, Schubert K, Schulz JB, Schweiger S, Tüscher O, Ungethüm K, Wagner TOF, Haas K; ZSE-DUO working group. Dual guidance structure for evaluation of patients with unclear diagnosis in centers for rare diseases (ZSE-DUO): study protocol for a controlled multi-center cohort study.  Orphanet J Rare Dis. 2022 Feb 14;17(1):47. doi: 10.1186/s13023-022-02176-1.
• Herrmann C, Schradt F, Lindner-Pfleghar B, Schuster J, Ludolph AC, Dorst J. Pharyngeal electrical stimulation in amyotrophic lateral sclerosis: a pilot study. Ther Adv Neurol Disord. 2022 Feb 8;15:17562864211068394. doi: 10.1177/17562864211068394. eCollection 2022.
• Hörner R, Kassubek J, Dreyhaupt J, Ludolph AC. The spectrum and differential diagnosis of acquired ocular motor nerve palsies: a clinical study of 502 patients. J Neurol. 2022 Apr;269(4):2140-2148. doi: 10.1007/s00415-021-10761-w. Epub 2021 Sep 19.
• Huss A, Abdelhak A, Mayer B, Tumani H, Müller HP, Althaus K, Kassubek J, Otto M, Ludolph AC, Yilmazer-Hanke D, Neugebauer H. Association of Serum GFAP with Functional and Neurocognitive Outcome in Sporadic Small Vessel Disease. Biomedicines. 2022 Aug 2;10(8):1869. doi: 10.3390/biomedicines10081869.
• Kandler K, Witzel S, Eder K, Rothenbacher D, Nagel G, Peter RS, Schuster J, Dorst J, Rosenbohm A, Ludolph AC; ALS Registry Study Group. Phenotyping of the thoracic-onset variant of amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 2022 May;93(5):563-565. doi: 10.1136/jnnp-2021-326712. Epub 2021 Oct 19.
• Kassubek R, Weinstock D, Behler A, Müller HP, Dupuis L, Kassubek J, Ludolph AC. Morphological alterations of the hypothalamus in idiopathic intracranial hypertension. Ther Adv Chronic Dis. 2022 Dec 2;13:20406223221141354. doi: 10.1177/20406223221141354. eCollection 2022.
• Kocar TD, Behler A, Leinert C, Denkinger M, Ludolph AC, Müller HP, Kassubek Artificial neural networks for non-linear age correction of diffusion metrics in the brain. J. Front Aging Neurosci. 2022 Oct 20;14:999787. doi: 10.3389/fnagi.2022.999787. eCollection 2022.
• Ludolph AC. Editorial: Amyotrophic lateral sclerosis. Curr Opin Neurol. 2022 Oct 1;35(5):658-659. doi: 10.1097/WCO.0000000000001100.
• Lulé D, Michels S, Finsel J, Braak H, Del Tredici K, Strobel J, Beer AJ, Uttner I, Müller HP, Kassubek J, Juengling FD, Ludolph AC. Clinicoanatomical substrates of selfish behaviour in amyotrophic lateral sclerosis - An observational cohort study. Cortex. 2022 Jan;146:261-270. doi: 10.1016/j.cortex.2021.11.009. Epub 2021 Nov 27.
• Maier A, Gaudlitz M, Grehl T, Weyen U, Steinbach R, Grosskreutz J, Rödiger A, Koch JC, Lengenfeld T, Weydt P, Günther R, Wolf J, Baum P, Metelmann M, Dorst J, Ludolph AC, Kettemann D, Norden J, Koc RY, Walter B, Hildebrandt B, Münch C, Meyer T, Spittel S. Use and subjective experience of the impact of motor-assisted movement exercisers in people with amyotrophic lateral sclerosis: a multicenter observational study. Sci Rep. 2022 Jun 10;12(1):9657. doi: 10.1038/s41598-022-13761-6.
• Michels S, Widmann P, Rapp D, Willkomm F, Ludolph AC, Dorst. Predictive parameters of early respiratory decline in amyotrophic lateral sclerosis. J. Eur J Neurol. 2022 Jul 9. doi: 10.1111/ene.15486. Online ahead of print.
• Miller TM, Cudkowicz ME, Genge A, Shaw PJ, Sobue G, Bucelli RC, Chiò A, Van Damme P, Ludolph AC, Glass JD, Andrews JA, Babu S, Benatar M, McDermott CJ, Cochrane T, Chary S, Chew S, Zhu H, Wu F, Nestorov I, Graham D, Sun P, McNeill M, Fanning L, Ferguson TA, Fradette S; VALOR and OLE Working Group. Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS. N Engl J Med. 2022 Sep 22;387(12):1099-1110. doi: 10.1056/NEJMoa2204705.
• Müller HP, Nagel AM, Keidel F, Wunderlich A, Hübers A, Gast LV, Ludolph AC, Beer M, Kassubek J. Relaxation-weighted 23Na magnetic resonance imaging maps regional patterns of abnormal sodium concentrations in amyotrophic lateral sclerosis. Ther Adv Chronic Dis. 2022 Jul 8;13:20406223221109480. doi: 10.1177/20406223221109480. eCollection 2022.
• Münch M, Müller HP, Behler A, Ludolph AC, Kassubek J. Segmental alterations of the corpus callosum in motor neuron disease: A DTI and texture analysis in 575 patients. Neuroimage Clin. 2022 May 28;35:103061. doi: 10.1016/j.nicl.2022.103061. Online ahead of print.
• Müller K, Oh KW, Nordin A, Panthi S, Kim SH, Nordin F, Freischmidt A, Ludolph AC, Ki CS, Forsberg K, Weishaupt J, Kim YE, Andersen PM. De novo mutations in SOD1 are a cause of ALS. J Neurol Neurosurg Psychiatry. 2022 Feb;93(2):201-206. doi: 10.1136/jnnp-2021-327520. Epub 2021 Sep 13.
• Oeckl P, Wagemann O, Halbgebauer S, Anderl-Straub S, Nuebling G, Prix C, Loosli SV, Wlasich E, Danek A, Steinacker P, Ludolph AC, Levin J, Otto M. Serum Beta-Synuclein Is Higher in Down Syndrome and Precedes Rise of pTau181.  Ann Neurol. 2022 Jul;92(1):6-10. doi: 10.1002/ana.26360. Epub 2022 Apr 12.
• Oeckl P, Anderl-Straub S, Von Arnim CAF, Baldeiras I, Diehl-Schmid J, Grimmer T, Halbgebauer S, Kort AM, Lima M, Marques TM, Ortner M, Santana I, Steinacker P, Verbeek MM, Volk AE, Ludolph AC, Otto M. Serum GFAP differentiates Alzheimer's disease from frontotemporal dementia and predicts MCI-to-dementia conversion. J Neurol Neurosurg Psychiatry. 2022 Apr 27:jnnp-2021-328547. doi: 10.1136/jnnp-2021-328547. Online ahead of print.
• Oeckl P, Anderl-Straub S, Danek A, Diehl-Schmid J, Fassbender K, Fliessbach K, Halbgebauer S, Huppertz HJ, Jahn H, Kassubek J, Kornhuber J, Landwehrmeyer B, Lauer M, Prudlo J, Schneider A, Schroeter ML, Steinacker P, Volk AE, Wagner M, Winkelmann J, Wiltfang J, Ludolph AC, Otto M; FTLD Consortium. Relationship of serum beta-synuclein with blood biomarkers and brain atrophy. Alzheimers Dement. 2022 Sep 21. doi: 10.1002/alz.12790. Online ahead of print.
• Raber FP, Gmeiner FV, Dreyhaupt J, Wolf A, Ludolph AC, Werner JU, Kassubek J, Althaus K. J Thrombolysis in central retinal artery occlusion: a retrospective observational study. Neurol. 2022 Oct 28. doi: 10.1007/s00415-022-11439-7. Online ahead of print.
• Rauch S, Müller HP, Dreyhaupt J, Ludolph AC, Kassubek J, Althaus K. Andexanet Alfa for Reversal of Factor Xa Inhibitors in Intracranial Hemorrhage: Observational Cohort Study. J Clin Med. 2022 Jun 13;11(12):3399. doi: 10.3390/jcm11123399.
• van Rheenen W, van der Spek RAA, Bakker MK, van Vugt JJFA, Hop PJ, Zwamborn RAJ, de Klein N, Westra HJ, Bakker OB, Deelen P, Shireby G, Hannon E, Moisse M, Baird D, Restuadi R, Dolzhenko E, Dekker AM, Gawor K, Westeneng HJ, Tazelaar GHP, van Eijk KR, Kooyman M, Byrne RP, Doherty M, Heverin M, Al Khleifat A, Iacoangeli A, Shatunov A, Ticozzi N, Cooper-Knock J, Smith BN, Gromicho M, Chandran S, Pal S, Morrison KE, Shaw PJ, Hardy J, Orrell RW, Sendtner M, Meyer T, Başak N, van der Kooi AJ, Ratti A, Fogh I, Gellera C, Lauria G, Corti S, Cereda C, Sproviero D, D'Alfonso S, Sorarù G, Siciliano G, Filosto M, Padovani A, Chiò A, Calvo A, Moglia C, Brunetti M, Canosa A, Grassano M, Beghi E, Pupillo E, Logroscino G, Nefussy B, Osmanovic A, Nordin A, Lerner Y, Zabari M, Gotkine M, Baloh RH, Bell S, Vourc'h P, Corcia P, Couratier P, Millecamps S, Meininger V, Salachas F, Mora Pardina JS, Assialioui A, Rojas-García R, Dion PA, Ross JP, Ludolph AC, Weishaupt JH, Brenner D, Freischmidt A, Bensimon G, Brice A, Durr A, Payan CAM, Saker-Delye S, Wood NW, Topp S, Rademakers R, Tittmann L, Lieb W, Franke A, Ripke S, Braun A, Kraft J, Whiteman DC, Olsen CM, Uitterlinden AG, Hofman A, Rietschel M, Cichon S, Nöthen MM, Amouyel P; SLALOM Consortium; PARALS Consortium; SLAGEN Consortium; SLAP Consortium, Traynor BJ, Singleton AB, Mitne Neto M, Cauchi RJ, Ophoff RA, Wiedau-Pazos M, Lomen-Hoerth C, van Deerlin VM, Grosskreutz J, Roediger A, Gaur N, Jörk A, Barthel T, Theele E, Ilse B, Stubendorff B, Witte OW, Steinbach R, Hübner CA, Graff C, Brylev L, Fominykh V, Demeshonok V, Ataulina A, Rogelj B, Koritnik B, Zidar J, Ravnik-Glavač M, Glavač D, Stević Z, Drory V, Povedano M, Blair IP, Kiernan MC, Benyamin B, Henderson RD, Furlong S, Mathers S, McCombe PA, Needham M, Ngo ST, Nicholson GA, Pamphlett R, Rowe DB, Steyn FJ, Williams KL, Mather KA, Sachdev PS, Henders AK, Wallace L, de Carvalho M, Pinto S, Petri S, Weber M, Rouleau GA, Silani V, Curtis CJ, Breen G, Glass JD, Brown RH Jr, Landers JE, Shaw CE, Andersen PM, Groen EJN, van Es MA, Pasterkamp RJ, Fan D, Garton FC, McRae AF, Davey Smith G, Gaunt TR, Eberle MA, Mill J, McLaughlin RL, Hardiman O, Kenna KP, Wray NR, Tsai E, Runz H, Franke L, Al-Chalabi A, Van Damme P, van den Berg LH, Veldink JH. Author Correction: Common and rare variant association analyses in amyotrophic lateral sclerosis identify 15 risk loci with distinct genetic architectures and neuron-specific biology. Nat Genet. 2022 Mar;54(3):361. doi: 10.1038/s41588-022-01020-3.
• Rosenbohm A, Del Tredici K, Braak H, Huppertz HJ, Ludolph AC, Müller HP, Kassubek J. Involvement of cortico-efferent tracts in flail arm syndrome: a tract-of-interest-based DTI study.J Neurol. 2022 May;269(5):2619-2626. doi: 10.1007/s00415-021-10854-6. Epub 2021 Oct 21.
• Rosenbohm A, Pott H, Thomsen M, Rafehi H, Kaya S, Szymczak S, Volk AE, Mueller K, Silveira I, Weishaupt JH, Tönnies H, Seibler P, Zschiedrich K, Schaake S, Westenberger A, Zühlke C, Depienne C, Trinh J, Ludolph AC, Klein C, Bahlo M, Lohmann K. Familial Cerebellar Ataxia and Amyotrophic Lateral Sclerosis/Frontotemporal Dementia with DAB1 and C9ORF72 Repeat Expansions: An 18-Year Study. Mov Disord. 2022 Sep 23. doi: 10.1002/mds.29221. Online ahead of print.
• Ruf WP, Hannon E, Freischmidt A, Grozdanov V, Brenner D, Müller K, Knehr A, Günther K, Dorst J, Ammerpohl O, Danzer KM, Mill J, Ludolph AC, Weishaupt JH. Methylome analysis of ALS patients and presymptomatic mutation carriers in blood cells. Neurobiol Aging. 2022 Aug;116:16-24. doi: 10.1016/j.neurobiolaging.2022.04.003. Epub 2022 Apr 20.
• Ruf WP, Palmer A, Dörfer L, Wiesner D, Buck E, Grozdanov V, Kassubek J, Dimou L, Ludolph AC, Huber-Lang M, Danzer KM. Thoracic trauma promotes alpha-Synuclein oligomerization in murine Parkinson's disease. Neurobiol Dis. 2022 Nov;174:105877. doi: 10.1016/j.nbd.2022.105877. Epub 2022 Sep 24.
• Schrempf T, Finsel J, Uttner I, Ludolph AC, Lulé D. Neuropsychological deficits have only limited impact on psychological well-being in amyotrophic lateral sclerosis.J Neurol. 2022 Mar;269(3):1369-1374. doi: 10.1007/s00415-021-10690-8. Epub 2021 Jul 2.
• Streit L, Kuhn T, Vomhof T, Bopp V, Ludolph AC, Weishaupt JH, Gebhardt JCM, Michaelis J, Danzer KM. Stress induced TDP-43 mobility loss independent of stress granules. Nat Commun. 2022 Sep 19;13(1):5480. doi: 10.1038/s41467-022-32939-0.
• Van Schoor E, Ospitalieri S, Moonen S, Tomé SO, Ronisz A, Ok O, Weishaupt J, Ludolph AC, Van Damme P, Van Den Bosch L, Thal DR. Increased pyroptosis activation in white matter microglia is associated with neuronal loss in ALS motor cortex. Acta Neuropathol. 2022 Jul 22. doi: 10.1007/s00401-022-02466-9. Online ahead of print.
• Vernikouskaya I, Müller HP, Felbel D, Roselli F, Ludolph AC, Kassubek J, Rasche V. Body fat compartment determination by encoder-decoder convolutional neural network: application to amyotrophic lateral sclerosis. Sci Rep. 2022 Apr 1;12(1):5513. doi: 10.1038/s41598-022-09518-w.
• Witzel S, Maier A, Steinbach R, Grosskreutz J, Koch JC, Sarikidi A, Petri S, Günther R, Wolf J, Hermann A, Prudlo J, Cordts I, Lingor P, Löscher WN, Kohl Z, Hagenacker T, Ruckes C, Koch B, Spittel S, Günther K, Michels S, Dorst J, Meyer T, Ludolph AC; German Motor Neuron Disease Network (MND-NET). Safety and Effectiveness of Long-term Intravenous Administration of Edaravone for Treatment of Patients With Amyotrophic Lateral Sclerosis. JAMA Neurol. 2022 Jan 10:e214893. doi: 10.1001/jamaneurol.2021.4893. Online ahead of print.
• Witzel S, Wagner M, Zhao C, Kandler K, Graf E, Berutti R, Oexle K, Brenner D, Winkelmann J, Ludolph AC. Fast versus slow disease progression in amyotrophic lateral sclerosis-clinical and genetic factors at the edges of the survival spectrum. Neurobiol Aging. 2022 Jul 16:S0197-4580(22)00158-0. doi: 10.1016/j.neurobiolaging.2022.07.005. Online ahead of print.
• Xia K, Witzel S, Witzel C, Klose V, Fan D, Ludolph AC, Dorst Mutation-specific metabolic profiles in presymptomatic amyotrophic lateral sclerosis. J. Eur J Neurol. 2022 Sep 28. doi: 10.1111/ene.15584. Online ahead of print.

• Al Shweiki MR, Oeckl P, Pachollek A, Steinacker P, Barschke P, Halbgebauer S, Anderl-Straub S, Lewerenz J, Ludolph AC, Bernhard Landwehrmeyer G, Otto M. Cerebrospinal Fluid Levels of Prodynorphin-Derived Peptides are Decreased in Huntington's Disease. Mov Disord. 2021 Feb;36(2):492-497. doi: 10.1002/mds.28300. Epub 2020 Oct 2.
• Anderl-Straub S, Schuster J, Dorst J, Ludolph AC. Amyotrophic lateral sclerosis and frontotemporal dementia-On the way to common gene-specific treatment approaches. Nervenarzt. 2021 Oct 26. doi: 10.1007/s00115-021-01209-7. Online ahead of print.
• Andersen PM, Kuźma-Kozakiewicz M, Keller J, Maksymowicz-Śliwińska A, Barć K, Nieporęcki K, Finsel J, Vazquez C, Helczyk O, Linse K, Häggström AC, Stenberg E, Semb O, Ciećwierska K, Szejko N, Uttner I, Herrmann A, Petri S, Meyer T, Ludolph AC, Lulé D. Caregivers' divergent perspectives on patients' well-being and attitudes towards hastened death in Germany, Poland and Sweden. Amyotroph Lateral Scler Frontotemporal Degener. 2021 Jun 30:1-11. doi: 10.1080/21678421.2021.1936064. Online ahead of print. PMID: 34187249
• Althaus K, Dreyhaupt J, Hyrenbach S, Pinkhardt EH, Kassubek J, Ludolph AC. MRI as a first-line imaging modality in acute ischemic stroke: a sustainable concept. Ther Adv Neurol Disord. 2021 Aug 27;14:17562864211030363. doi: 10.1177/17562864211030363. eCollection 2021.
• Bayer D, Antonucci S, Müller HP, Saad R, Dupuis L, Rasche V, Böckers TM, Ludolph AC, Kassubek J, Roselli F. Disruption of orbitofrontal-hypothalamic projections in a murine ALS model and in human patients.  Transl Neurodegener. 2021 May 31;10(1):17. doi: 10.1186/s40035-021-00241-6. PMID: 34059131
• Behler A, Knehr A, Finsel J, Kunz MS, Lang C, Müller K, Müller HP, Pinkhardt EH, Ludolph AC, Lulé D, Kassubek J. Eye movement alterations in presymptomatic C9orf72 expansion gene carriers.J Neurol. 2021 Mar 11. doi: 10.1007/s00415-021-10510-z. Online ahead of print.
• Bhatia D, Grozdanov V, Ruf WP, Kassubek J, Ludolph AC, Weishaupt JH, Danzer KM. T-cell dysregulation is associated with disease severity in Parkinson's Disease. J Neuroinflammation. 2021 Oct 30;18(1):250. doi: 10.1186/s12974-021-02296-8.
• Borroni B, Graff C, Hardiman O, Ludolph AC, Moreno F, Otto M, Piccininni M, Remes AM, Rowe JB, Seelaar H, Stefanova E, Traykov L, Logroscino G; FRONTIERS. FRONTotemporal dementia Incidence European Research Study-FRONTIERS: Rationale and design. Alzheimers Dement. 2021 Aug 2. doi: 10.1002/alz.12414. Online ahead of print.
• Brenner D, Müller K, Lattante S, Yilmaz R, Knehr A, Freischmidt A, Ludolph AC, Andersen PM, Weishaupt JH. FUS mutations dominate TBK1 mutations in FUS/TBK1 double-mutant ALS/FTD pedigrees.Neurogenetics. 2021 Sep 13. doi: 10.1007/s10048-021-00671-4. Online ahead of print.
• Freigang M, Wurster CD, Hagenacker T, Stolte B, Weiler M, Kamm C, Schreiber-Katz O, Osmanovic A, Petri S, Kowski A, Meyer T, Koch JC, Cordts I, Deschauer M, Lingor P, Aust E, Petzold D, Ludolph AC, Falkenburger B, Hermann A, Günther R. Serum creatine kinase and creatinine in adult spinal muscular atrophy under nusinersen treatment. Ann Clin Transl Neurol. 2021 May;8(5):1049-1063. doi: 10.1002/acn3.51340. Epub 2021 Mar 31. PMID: 33792208 Free PMC article.
• Freigang M, Steinacker P, Wurster CD, Schreiber-Katz O, Osmanovic A, Petri S, Koch JC, Rostásy K, Falkenburger B, Ludolph AC, Otto M, Hermann A, Günther R. Increased chitotriosidase 1 concentration following nusinersen treatment in spinal muscular atrophy. Orphanet J Rare Dis. 2021 Jul 28;16(1):330. doi: 10.1186/s13023-021-01961-8.
• Freischmidt A, Goswami A, Limm K, Zimyanin VL, Demestre M, Glaß H, Holzmann K, Helferich AM, Brockmann SJ, Tripathi P, Yamoah A, Poser I, Oefner PJ, Böckers TM, Aronica E, Ludolph AC, Andersen PM, Hermann A, Weis J, Reinders J, Danzer KM, Weishaupt JH. A serum microRNA sequence reveals fragile X protein pathology in amyotrophic lateral sclerosis. Brain. 2021 May 7;144(4):1214-1229. doi: 10.1093/brain/awab018. PMID: 33871026
• Halbgebauer S, Steinacker P, Verde F, Weishaupt J, Oeckl P, von Arnim C, Dorst J, Feneberg E, Mayer B, Rosenbohm A, Silani V, Ludolph AC, Otto M. Comparison of CSF and serum neurofilament light and heavy chain as differential diagnostic biomarkers for ALS. J Neurol Neurosurg Psychiatry. 2021 Aug 20:jnnp-2021-327129. doi: 10.1136/jnnp-2021-327129. Online ahead of print.
• Hörner R, Kassubek J, Dreyhaupt J, Ludolph AC. The spectrum and differential diagnosis of acquired ocular motor nerve palsies: a clinical study of 502 patients. J Neurol. 2021 Sep 19. doi: 10.1007/s00415-021-10761-w. Online ahead of print.
• Hübers A, Böckler B, Abaei A, Rasche V, Lulé D, Ercan E, Doorenweerd N, Müller HP, Dreyhaupt J, Kammer T, Ludolph AC, Ronen I, Kassubek J. Functional and structural impairment of transcallosal motor fibres in ALS: a study using transcranial magnetic stimulation, diffusion tensor imaging, and diffusion weighted spectroscopy.  Brain Imaging Behav. 2021 Apr;15(2):748-757. doi: 10.1007/s11682-020-00282-x. PMID: 32306281
• Hübers A, Kassubek J, Müller HP, Broc N, Dreyhaupt J, Ludolph AC. The ipsilateral silent period: an early diagnostic marker of callosal disconnection in ALS. Ther Adv Chronic Dis. 2021 Sep 13;12:20406223211044072. doi: 10.1177/20406223211044072. eCollection 2021.
• Jesse S, Huppertz HJ, Ludolph AC, Kassubek J. Pediatr Neurol. 2021 Focal Cortical Dysplasia: Relevant for Seizures in Phelan-McDermid Syndrome? Feb;115:7-9. doi: 10.1016/j.pediatrneurol.2020.11.005. Epub 2020 Nov 14.
• Kandler K, Witzel S, Eder K, Rothenbacher D, Nagel G, Peter RS, Schuster J, Dorst J, Rosenbohm A, Ludolph AC; ALS Registry Study Group. Phenotyping of the thoracic-onset variant of amyotrophic lateral sclerosis.  J Neurol Neurosurg Psychiatry. 2021 Oct 19:jnnp-2021-326712. doi: 10.1136/jnnp-2021-326712. Online ahead of print.
• Kersten J, Güleroglu AM, Rosenbohm A, Buckert D, Ludolph AC, Hackenbroch C, Beer M, Bernhardt P. Myocardial involvement and deformation abnormalities in idiopathic inflammatory myopathy assessed by CMR feature tracking. Int J Cardiovasc Imaging. 2021 Feb;37(2):597-603. doi: 10.1007/s10554-020-02020-2. Epub 2020 Sep 17.
• Kocar TD, Müller HP, Ludolph AC, Kassubek J. Feature selection from magnetic resonance imaging data in ALS: a systematic review. Ther Adv Chronic Dis. 2021 Oct 13;12:20406223211051002. doi: 10.1177/20406223211051002. eCollection 2021.
• Lehmann Urban D, Mohamed M, Ludolph AC, Kassubek J, Rosenbohm A. The value of qualitative muscle MRI in the diagnostic procedures of myopathies: a biopsy-controlled study in 191 patients.  Ther Adv Neurol Disord. 2021 Feb 26;14:1756286420985256. doi: 10.1177/1756286420985256. eCollection 2021.
• Lohmann L, Lammerskitten A, Korsen M, Dodel R, Gaul C, Hamer HM, Kleineberg NN, Ludolph AC, Mayer G, Poli S, Saur D, Steinhoff BJ, Timmermann L, Klotz L, Meuth SG. Status of Clinical Research in Neurology in Germany - A National Survey. Eur J Neurol. 2021 Feb 4. doi: 10.1111/ene.14763. Online ahead of print.
• Månberg A, Skene N, Sanders F, Trusohamn M, Remnestål J, Szczepińska A, Aksoylu IS, Lönnerberg P, Ebarasi L, Wouters S, Lehmann M, Olofsson J, von Gohren Antequera I, Domaniku A, De Schaepdryver M, De Vocht J, Poesen K, Uhlén M, Anink J, Mijnsbergen C, Vergunst-Bosch H, Hübers A, Kläppe U, Rodriguez-Vieitez E, Gilthorpe JD, Hedlund E, Harris RA, Aronica E, Van Damme P, Ludolph AC, Veldink J, Ingre C, Nilsson P, Lewandowski SA. Altered perivascular fibroblast activity precedes ALS disease onset. Nat Med. 2021 Apr;27(4):640-646.
• Meyer T, Maier A, Uzelac Z, Hagenacker T, Günther R, Schreiber-Katz O, Weiler M, Steinbach R, Weyen U, Koch JC, Kettemann D, Norden J, Dorst J, Wurster C, Ludolph AC, Stolte B, Freigang M, Osmanovic A, Petri S, Grosskreutz J, Rödiger A, Griep R, Gaudlitz M, Walter B, Münch C, Spittel S. Treatment expectations and perception of therapy in adult patients with spinal muscular atrophy receiving nusinersen. Eur J Neurol. 2021 May 7. doi: 10.1111/ene.14902. Online ahead of print. PMID: 33960080
• Mix L, Winter B, Wurster CD, Platen S, Witzel S, Uzelac Z, Graf H, Ludolph AC, Lulé D. Quality of Life in SMA Patients Under Treatment With Nusinersen. Front Neurol. 2021 Mar 29;12:626787. doi: 10.3389/fneur.2021.626787. eCollection 2021. PMID: 33854472
• Mix L, Schreiber-Katz O, Wurster CD, Uzelac Z, Platen S, Gipperich C, Ranxha G, Wieselmann G, Osmanovic A, Ludolph AC, Petri S, Lulé D. Orphanet Executive function is inversely correlated with physical function: the cognitive profile of adult Spinal Muscular Atrophy (SMA).  J Rare Dis. 2021 Jan 6;16(1):10. doi: 10.1186/s13023-020-01661-9.
• Mora JS, Bradley WG, Chaverri D, Hernández-Barral M, Mascias J, Gamez J, Gargiulo-Monachelli GM, Moussy A, Mansfield CD, Hermine O, Ludolph AC. Long-term survival analysis of masitinib in amyotrophic lateral sclerosis. Ther Adv Neurol Disord. 2021 Jul 19;14:17562864211030365. doi: 10.1177/17562864211030365. eCollection 2021.
• Müller HP, Lulé D, Roselli F, Behler A, Ludolph AC, Kassubek J. Segmental involvement of the corpus callosum in C9orf72-associated ALS: a tract of interest-based DTI study. Ther Adv Chronic Dis. 2021 Mar 21;12:20406223211002969. doi: 10.1177/20406223211002969. eCollection 2021. PMID: 33815737 Free PMC article.
• Müller K, Oh KW, Nordin A, Panthi S, Kim SH, Nordin F, Freischmidt A, Ludolph AC, Ki CS, Forsberg K, Weishaupt J, Kim YE, Andersen PM. De novo mutations in SOD1 are a cause of ALS. J Neurol Neurosurg Psychiatry. 2021 Sep 13:jnnp-2021-327520. doi: 10.1136/jnnp-2021-327520. Online ahead of print.
• Osmanovic A, Ranxha G, Kumpe M, Wurster CD, Stolte B, Cordts I, Günther R, Freigang M, Müschen LH, Binz C, Hermann A, Deschauer M, Lingor P, Ludolph AC, Hagenacker T, Schreiber-Katz O, Petri S. Treatment satisfaction in 5q-spinal muscular atrophy under nusinersen therapy. Ther Adv Neurol Disord. 2021 Mar 5;14:1756286421998902. doi: 10.1177/1756286421998902. eCollection 2021. PMID: 33747131 Free PMC article.
• Povedano Panades M, Couratier P, Sidle K, Sorarù G, Tsivgoulis G, Ludolph AC. Administration of Riluzole Oral Suspension During the Different Stages of Amyotrophic Lateral Sclerosis.  Front Neurol. 2021 Jul 8;12:633854. doi: 10.3389/fneur.2021.633854. eCollection 2021. PMID: 34305774
• Peseschkian T, Cordts I, Günther R, Stolte B, Zeller D, Schröter C, Weyen U, Regensburger M, Wolf J, Schneider I, Hermann A, Metelmann M, Kohl Z, Linker RA, Koch JC, Büchner B, Weiland U, Schönfelder E, Heinrich F, Osmanovic A, Klopstock T, Dorst J, Ludolph AC, Boentert M, Hagenacker T, Deschauer M, Lingor P, Petri S, Schreiber-Katz O. A Nation-Wide, Multi-Center Study on the Quality of Life of ALS Patients in Germany. Brain Sci. 2021 Mar 14;11(3):372. doi: 10.3390/brainsci11030372. PMID: 33799476 Free PMC article.
• Rosenbohm A, Del Tredici K, Braak H, Huppertz HJ, Ludolph AC, Müller HP, Kassubek J. Involvement of cortico-efferent tracts in flail arm syndrome: a tract-of-interest-based DTI study. J Neurol. 2021 Oct 21. doi: 10.1007/s00415-021-10854-6. Online ahead of print.
• Rosenbohm A, Peter R, Dorst J, Kassubek J, Rothenbacher D, Nagel G, Ludolph AC; ALS Registry Swabia Study Group. Life Course of Physical Activity and Risk and Prognosis of Amyotrophic Lateral Sclerosis in a German ALS Registry. Neurology. 2021 Nov 9;97(19):e1955-e1963. doi: 10.1212/WNL.0000000000012829. Epub 2021 Oct 20.
• Ruf WP, Freischmidt A, Grozdanov V, Roth V, Brockmann SJ, Mollenhauer B, Martin D, Haslinger B, Fundel-Clemens K, Otto M, Arnim CV, Holzmann K, Ludolph AC, Weishaupt JH, Danzer KM. Protein Binding Partners of Dysregulated miRNAs in Parkinson's Disease Serum.  Cells. 2021 Apr 2;10(4):791. doi: 10.3390/cells10040791. PMID: 33918274 Free PMC article.
• Sieverding K, Ulmer J, Bruno C, Satoh T, Tsao W, Freischmidt A, Akira S, Wong PC, Ludolph AC, Danzer KM, Lobsiger CS, Brenner D, Weishaupt JH. Hemizygous deletion of Tbk1 worsens neuromuscular junction pathology in TDP-43G298S transgenic mice. Exp Neurol. 2021 Jan;335:113496. doi: 10.1016/j.expneurol.2020.113496. Epub 2020 Oct 8.
• Schischlevskij P, Cordts I, Günther R, Stolte B, Zeller D, Schröter C, Weyen U, Regensburger M, Wolf J, Schneider I, Hermann A, Metelmann M, Kohl Z, Linker RA, Koch JC, Stendel C, Müschen LH, Osmanovic A, Binz C, Klopstock T, Dorst J, Ludolph AC, Boentert M, Hagenacker T, Deschauer M, Lingor P, Petri S, Schreiber-Katz O. Informal Caregiving in Amyotrophic Lateral Sclerosis (ALS): A High Caregiver Burden and Drastic Consequences on Caregivers' Lives. Brain Sci. 2021 Jun 4;11(6):748. doi: 10.3390/brainsci11060748.
• Schrempf T, Finsel J, Uttner I, Ludolph AC, Lulé D. Neuropsychological deficits have only limited impact on psychological well-being in amyotrophic lateral sclerosis. J Neurol. 2021 Jul 2. doi: 10.1007/s00415-021-10690-8. Online ahead of print. PMID: 34215919
• Steinacker P, Feneberg E, Halbgebauer S, Witzel S, Verde F, Oeckl P, Van Damme P, Gaur N, Gray E, Grosskreutz J, Jardel CG, Kachanov M, Kuhle J, Lamari F, Maceski A, Del Mar Amador M, Mayer B, Morelli C, Petri S, Poesen K, Raaphorst J, Salachas F, Silani V, Turner MR, Verbeek MM, Volk AE, Weishaupt JH, Weydt P, Ludolph AC, Otto M. Chitotriosidase as biomarker for early stage amyotrophic lateral sclerosis: a multicenter study. Amyotroph Lateral Scler Frontotemporal Degener. 2021 May;22(3-4):276-286. doi: 10.1080/21678421.2020.1861023. Epub 2021 Feb 12. PMID: 33576252
• Steinacker P, Al Shweiki MR, Oeckl P, Graf H, Ludolph AC, Schönfeldt-Lecuona C, Otto M. Glial fibrillary acidic protein as blood biomarker for differential diagnosis and severity of major depressive disorder J Psychiatr Res. 2021 Sep 2;144:54-58. doi: 10.1016/j.jpsychires.2021.09.012. Online ahead of print.
• Tang L, Dorst J, Chen L, Liu X, Ma Y, Günther K, Michels S, Müller K, Freischmidt A, Weishaupt JH, Fan D, Ludolph AC. A natural history comparison of SOD1-mutant patients with amyotrophic lateral sclerosis between Chinese and German populations. Transl Neurodegener. 2021 Oct 28;10(1):42. doi: 10.1186/s40035-021-00266-x.
• Van Schoor E, Koper MJ, Ospitalieri S, Dedeene L, Tomé SO, Vandenberghe R, Brenner D, Otto M, Weishaupt J, Ludolph AC, Van Damme P, Van Den Bosch L, Thal DR. Necrosome-positive granulovacuolar degeneration is associated with TDP-43 pathological lesions in the hippocampus of ALS/FTLD cases. Neuropathol Appl Neurobiol. 2021 Feb;47(2):328-345. doi: 10.1111/nan.12668. Epub 2020 Oct 20.
• Wagner M, Lorenz G, Volk AE, Brunet T, Edbauer D, Berutti R, Zhao C, Anderl-Straub S, Bertram L, Danek A, Deschauer M, Dill V, Fassbender K, Fliessbach K, Götze KS, Jahn H, Kornhuber J, Landwehrmeyer B, Lauer M, Obrig H, Prudlo J, Schneider A, Schroeter ML, Uttner I, Vukovich R, Wiltfang J, Winkler AS, Zhou Q, Ludolph AC; German FTLD consortium, Oexle K, Otto M, Diehl-Schmid J, Clinico-genetic findings in 509 frontotemporal dementia patients. Winkelmann J. Mol Psychiatry. 2021 Sep 24. doi: 10.1038/s41380-021-01271-2. Online ahead of print.
• Witzel S, Frauhammer F, Steinacker P, Devos D, Pradat PF, Meininger V, Halbgebauer S, Oeckl P, Schuster J, Anders S, Dorst J, Otto M, Ludolph AC. Neurofilament light and heterogeneity of disease progression in amyotrophic lateral sclerosis: development and validation of a prediction model to improve interventional trials. Transl Neurodegener. 2021 Aug 26;10(1):31. doi: 10.1186/s40035-021-00257-y.
• Wunderlich J, Behler A, Dreyhaupt J, Ludolph AC, Pinkhardt EH, Kassubek J. Diagnostic value of video-oculography in progressive supranuclear palsy: a controlled study in 100 patients.  J Neurol. 2021 Mar 21. doi: 10.1007/s00415-021-10522-9. Online ahead of print.

• Abdelhak A, Huss A, Brück A, Sebert U, Mayer B, Müller HP, Tumani H, Otto M, Yilmazer-Hanke D, Ludolph AC, Kassubek J, Pinkhardt E, Neugebauer H. Optical coherence tomography-based assessment of retinal vascular pathology in cerebral small vessel disease. Neurol Res Pract 2020;2:13

• Abdelhak A, Huss A, Stahmann A, Senel M, Krumbholz M, Kowarik MC, Havla J, Kümpfel T, Kleiter I, Wüstinger I, Zettl UK, Schwartz M, Roesler R, Friede T, Ludolph AC, Ziemann U, Tumani H. Explorative study of emerging blood biomarkers in progressive multiple sclerosis (EmBioProMS): Design of a prospective observational multicentre pilot study. Contemp Clin Trials Commun 2020;18:100574

• Abu-Rumeileh S, Halbgebauer S, Steinacker P, Anderl-Straub S, Polischi B, Ludolph AC, Capellari S, Parchi P, Otto M. CSF SerpinA1 in Creutzfeldt-Jakob disease and frontotemporal lobar degeneration.Ann Clin Transl Neurol 2020;7(2):191-199

• Al Shweiki MR, Oeckl P, Pachollek A, Steinacker P, Barschke P, Halbgebauer S, Anderl-Straub S, Lewerenz J, Ludolph AC, Bernhard Landwehrmeyer G, Otto M. Cerebrospinal Fluid Levels of Prodynorphin-Derived Peptides Are Decreased in Huntington's Disease. Mov Disord 2020. doi: 10.1002/mds.28300. Online ahead of print.

• Barć K, Weber C, Maksymowicz-Śliwińska A, Ciećwierska K, Helczyk O, Ludolph AC, Kuźma-Kozakiewicz M, Lulé D. Healthcare provision in amyotrophic lateral sclerosis: procedures, queries and pitfalls in Germany and Poland. Amyotroph Lateral Scler Frontotemporal Degener 2020;21(3-4):193-202

• Barschke P, Oeckl P, Steinacker P, Al Shweiki MR, Weishaupt JH, Landwehrmeyer GB, Anderl-Straub S, Weydt P, Diehl-Schmid J, Danek A, Kornhuber J, Schroeter ML, Prudlo J, Jahn H, Fassbender K, Lauer M, van der Ende EL, van Swieten JC, Volk AE, Ludolph AC, Otto M; German FTLD consortium. Different CSF protein profiles in amyotrophic lateral sclerosis and frontotemporal dementia with C9orf72 hexanucleotide repeat expansion. J Neurol Neurosurg Psych 2020;91(5):503-511

• Brenner D, Freischmidt A, Ludolph AC, Weishaupt JH. [Gene-specific treatment approaches in amyotrophic lateral sclerosis in the present and future]. Nervenarzt 2020 ;91(4):287-293

• Brenner D, Ludolph AC, Weishaupt JH. Gene specific therapies - the next therapeutic milestone in neurology. Neurol Res Pract 2020;2:25

• Brenner D, Ludolph AC. [Gene-specific treatment of neurological diseases-current state and perspectives]. Nervenarzt 2020;91(4):285-286

• Bruno C, Sieverding K, Freischmidt A, Satoh T, Walther P, Mayer B, Ludolph AC, Akira S, Yilmazer-Hanke D, Danzer KM, Lobsiger CS, Brenner D, Weishaupt JH. Haploinsufficiency of TANK-binding kinase 1 prepones age-associated neuroinflammatory changes without causing motor neuron degeneration in aged mice. Brain Commun 2020;2(2):fcaa133

• Dedeene L, Van Schoor E, Ospitalieri S, Ronisz A, Weishaupt JH, Otto M, Ludolph AC, Scheuerle A, Vandenberghe R, Van Damme P, Poesen K, Thal DR. Dipeptide repeat protein and TDP-43 pathology along the hypothalamic-pituitary axis in C9orf72 and non-C9orf72 ALS and FTLD-TDP cases. Acta Neuropathol 2020;140(5):777-781

• Del Tredici K, Ludolph AC, Feldengut S, Jacob C, Reichmann H, Bohl JR, Braak H. Fabry Disease With Concomitant Lewy Body Disease. J Neuropathol Exp Neurol 2020;79(4):378-392

• Dorst J, Schuster J, Dreyhaupt J, Witzel S, Weishaupt JH, Kassubek J, Weiland U, Petri S, Meyer T, Grehl T, Hermann A, Jordan B, Grosskreutz J, Zeller D, Boentert M, Schrank B, Prudlo J, Winkler AS, Gorbulev S, Roselli F, Dupuis L, Otto M, Ludolph AC. Effect of high-caloric nutrition on serum neurofilament light chain levels in amyotrophic lateral sclerosis. J Neurol Neurosurg Psych 2020;91(9):1007-1009

• Freischmidt A, Brenner D, Ludolph AC, Andersen PM, Weishaupt JH. Commentary: Effects of ALS-associated TANK binding kinase 1 mutations on protein-protein interactions and kinase activity. Front Neurosci 2020;14:551006

• Gray E, Oeckl P, Amador MDM, Andreasson U, An J, Blennow K, Bowser R, De Schaepdryver M, Heslegrave A, Kuhle J, Maceski A, Koel-Simmelink M, Lamari F, Lombardi V, Malaspina A, Nilsson I, Poesen K, Salachas F, Steinacker P, Teunissen CE, Van Damme P, Zetterberg H, Ludolph A, Jeromin A, Turner MR, Otto M. A multi-center study of neurofilament assay reliability and inter-laboratory variability. Amyotroph Lateral Scler Frontotemporal Degener 2020;21(5-6):452-458

• Hagenacker T, Wurster CD, Günther R, Schreiber-Katz O, Osmanovic A, Petri S, Weiler M, Ziegler A, Kuttler J, Koch JC, Schneider I, Wunderlich G, Schloss N, Lehmann HC, Cordts I, Deschauer M, Lingor P, Kamm C, Stolte B, Pietruck L, Totzeck A, Kizina K, Mönninghoff C, von Velsen O, Ose C, Reichmann H, Forsting M, Pechmann A, Kirschner J, Ludolph AC, Hermann A, Kleinschnitz C. Nusinersen in adults with 5q spinal muscular atrophy: a non-interventional, multicentre, observational cohort study. Lancet Neurol 2020;19(4):317-325

• Hübers A, Böckler B, Abaei A, Rasche V, Lulé D, Ercan E, Doorenweerd N, Müller HP, Dreyhaupt J, Kammer T, Ludolph AC, Ronen I, Kassubek J. Functional and structural impairment of transcallosal motor fibres in ALS: a study using transcranial magnetic stimulation, diffusion tensor imaging, and diffusion weighted spectroscopy. Brain Imaging Behav. 2020 Apr 18. doi: 10.1007/s11682-020-00282-x. Online ahead of print.

• Huss A, Otto M, Senel M, Ludolph AC, Abdelhak A, Tumani H. A Score Based on NfL and Glial Markers May Differentiate Between Relapsing-Remitting and Progressive MS Course. Front Neurol 2020;11:608

• Huss A, Senel M, Abdelhak A, Mayer B, Kassubek J, Ludolph AC, Otto M, Tumani H. Longitudinal Serum Neurofilament Levels of Multiple Sclerosis Patients Before and After Treatment with First-Line Immunomodulatory Therapies. Biomedicines 2020;8(9):312

• Jesse S, Müller HP, Schoen M, Asoglu H, Bockmann J, Huppertz HJ, Rasche V, Ludolph AC, Boeckers TM, Kassubek J. Severe white matter damage in SHANK3 deficiency: a human and translational study. Ann Clin Transl Neuro 2020;7(1):46-58

• Joilin G, Gray E, Thompson AG, Bobeva Y, Talbot K, Weishaupt J, Ludolph A, Malaspina A, Leigh PN, Newbury SF, Turner MR, Hafezparast M. Identification of a potential non-coding RNA biomarker signature for amyotrophic lateral sclerosis. Brain Commun 2020;2(1):fcaa053

• Kersten J, Güleroglu AM, Rosenbohm A, Buckert D, Ludolph AC, Hackenbroch C, Beer M, Bernhardt P. Myocardial involvement and deformation abnormalities in idiopathic inflammatory myopathy assessed by CMR feature tracking. Int J Cardiovasc Imaging. 2020 Sep 17. doi: 10.1007/s10554-020-02020-2. Online ahead of print.

• Kruse T, Heller R, Wirth B, Glöggler J, Wurster CD, Ludolph AC, Braumann B. Maximum bite force in patients with spinal muscular atrophy during the first year of nusinersen therapy - A pilot study. Acta Myol 2020 1;39(2):83-89

• Lehmann Urban D, Motlagh Scholle L, Alt K, Ludolph AC, Rosenbohm A. Camptocormia as a Novel Phenotype in a Heterozygous POLG2 Mutation. Diagnostics (Basel) 2020;10(2):68

• Ludolph AC, Dorst J, Dreyhaupt J, Weishaupt JH, Kassubek J, Weiland U, Meyer T, Petri S, Hermann A, Emmer A, Grosskreutz J, Grehl T, Zeller D, Boentert M, Schrank B, Prudlo J, Winkler AS, Gorbulev S, Roselli F, Schuster J, Dupuis L; LIPCAL-ALS Study Group. Effect of High-Caloric Nutrition on Survival in Amyotrophic Lateral Sclerosis. Ann Neurol 2020;87(2):206-216

• Ludolph AC, Emilian S, Dreyhaupt J, Rosenbohm A, Kraskov A, Lemon RN, Del Tredici K, Braak H. Pattern of paresis in ALS is consistent with the physiology of the corticomotoneuronal projections to different muscle groups. J Neurol Neurosurg Psych 2020;(9):991-998

• Lulé DE, Ludolph AC. In vivo tracking of TDP43 in ALS: cognition as a new biomarker for brain pathology. J Neurol Neurosurg Psych 2020;91(2):125

• Lulé DE, Müller HP, Finsel J, Weydt P, Knehr A, Winroth I, Andersen P, Weishaupt J, Uttner I, Kassubek J, Ludolph AC. Deficits in verbal fluency in presymptomatic C9orf72 mutation gene carriers-a developmental disorder. J Neurol Neurosurg Psych 2020;91(11):1195-1200

• Lutz AK, Pfaender S, Incearap B, Ioannidis V, Ottonelli I, Föhr KJ, Cammerer J, Zoller M, Higelin J, Giona F, Stetter M, Stoecker N, Alami NO, Schön M, Orth M, Liebau S, Barbi G, Grabrucker AM, Delorme R, Fauler M, Mayer B, Jesse S, Roselli F, Ludolph AC, Bourgeron T, Verpelli C, Demestre M, Boeckers TM. Autism-associated SHANK3 mutations impair maturation of neuromuscular junctions and striated muscles. Sci Transl Med 2020;12(547):eaaz3267

• Meyer T, Kettemann D, Maier A, Grehl T, Weyen U, Grosskreutz J, Steinbach R, Norden J, George A, Hermann A, Guenther R, Petri S, Schreiber-Katz O, Dorst J, Ludolph AC, Walter B, Münch C, Spittel S. Symptomatic pharmacotherapy in ALS: data analysis from a platform-based medication management programme. J Neurol Neurosurg Psych 2020;91(7):783-785

• Miller T, Cudkowicz M, Shaw PJ, Andersen PM, Atassi N, Bucelli RC, Genge A, Glass J, Ladha S, Ludolph AC, Maragakis NJ, McDermott CJ, Pestronk A, Ravits J, Salachas F, Trudell R, Van Damme P, Zinman L, Bennett CF, Lane R, Sandrock A, Runz H, Graham D, Houshyar H, McCampbell A, Nestorov I, Chang I, McNeill M, Fanning L, Fradette S, Ferguson TA. Phase 1-2 Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS. N Engl J Med 2020;383(2):109-119

• Müller HP, Del Tredici K, Lulé D, Müller K, Weishaupt JH, Ludolph AC, Kassubek J. In vivo histopathological staging in C9orf72-associated ALS: A tract of interest DTI study. Neuroimage Clin 2020;27:102298

• Müller HP, Dreyhaupt J, Roselli F, Schlecht M, Ludolph AC, Huppertz HJ, Kassubek J. Focal alterations of the callosal area III in primary lateral sclerosis: An MRI planimetry and texture analysis. Neuroimage Clin 2020;26:10222

• Nagel G, Peter RS, Rosenbohm A, Koenig W, Dupuis L, Rothenbacher D, Ludolph AC. Association of Insulin-like Growth Factor 1 Concentrations with Risk for and Prognosis of Amyotrophic Lateral Sclerosis - Results from the ALS Registry Swabia. Sci Rep 2020;10(1):736

• Oeckl P, Halbgebauer S, Anderl-Straub S, von Arnim CAF, Diehl-Schmid J, Froelich L, Grimmer T, Hausner L, Denk J, Jahn H, Steinacker P, Weishaupt JH, Ludolph AC, Otto M. Targeted Mass Spectrometry Suggests Beta-Synuclein as Synaptic Blood Marker in Alzheimer's Disease. J Proteome Res 2020;19(3):1310-1318

• Oeckl P, Weydt P, Thal DR, Weishaupt JH, Ludolph AC, Otto M. Proteomics in cerebrospinal fluid and spinal cord suggests UCHL1, MAP2 and GPNMB as biomarkers and underpins importance of transcriptional pathways in amyotrophic lateral sclerosis. Acta Neuropathol 2020;139(1):119-134

• Ouali Alami N, Tang L, Wiesner D, Commisso B, Bayer D, Weishaupt J, Dupuis L, Wong P, Baumann B, Wirth T, Boeckers TM, Yilmazer-Hanke D, Ludolph A, Roselli F. Multiplexed chemogenetics in astrocytes and motoneurons restore blood-spinal cord barrier in ALS. Life Sci Alliance 2020;3(11):e201900571

• Pinkhardt EH, Ding Y, Slotnick S, Kassubek J, Ludolph AC, Glazman S, Selesnick I, Bodis-Wollner I. The intrinsically restructured fovea is correlated with contrast sensitivity loss in Parkinson's disease. Neural Transm (Vienna) 2020;127(9):1275-1283

• Rada A, Birnbacher R, Gobbi C, Kurthen M, Ludolph A, Naumann M, Neirich U, von Oertzen TJ, Ransmayr G, Riepe M, Schimmel M, Schwartz O, Surges R, Bien CG. Seizures associated with antibodies against cell surface antigens are acute symptomatic and not indicative of epilepsy: insights from long-term data. J Neurol 2020 Oct 6. doi: 10.1007/s00415-020-10250-6. Online ahead of print.

• Rosenbohm A, Buckert D, Kassubek J, Rottbauer W, Ludolph AC, Bernhardt P. Sporadic inclusion body myositis: no specific cardiac involvement in cardiac magnetic resonance tomography. J Neurol 2020;267(5):1407-1413

• Schumacher J, Peter RS, Nagel G, Rothenbacher D, Rosenbohm A, Ludolph AC, Dorst J; ALS Registry Swabia Study Group. Statins, diabetes mellitus and prognosis of amyotrophic lateral sclerosis: data from 501 patients of a population-based registry in southwest Germany. Eur J Neurol 2020;27(8):1405-1414

• Senderek J, Lassuthova P, Kabzińska D, Abreu L, Baets J, Beetz C, Braathen GJ, Brenner D, Dalton J, Dankwa L, Deconinck T, De Jonghe P, Dräger B, Eggermann K, Ellis M, Fischer C, Stojkovic T, Herrmann DN, Horvath R, Høyer H, Iglseder S, Kennerson M, Kinslechner K, Kohler JN, Kurth I, Laing NG, Lamont PJ, Wolfgang N L, Ludolph A, Marques W Jr, Nicholson G, Ong R, Petri S, Ravenscroft G, Rebelo A, Ricci G, Rudnik-Schöneborn S, Schirmacher A, Schlotter-Weigel B, Schoels L, Schüle R, Synofzik M, Francou B, Strom TM, Wagner J, Walk D, Wanschitz J, Weinmann D, Weishaupt J, Wiessner M, Windhager R, Young P, Züchner S, Toegel S, Seeman P, Kochański A, Auer-Grumbach M. The genetic landscape of axonal neuropathies in the middle-aged and elderly: Focus on MME. Neurology 2020;95(24):e3163-e3179

• Sieverding K, Ulmer J, Bruno C, Satoh T, Tsao W, Freischmidt A, Akira S, Wong PC, Ludolph AC, Danzer KM, Lobsiger CS, Brenner D, Weishaupt JH. Hemizygous deletion of Tbk1 worsens neuromuscular junction pathology in TDP-43G298S transgenic mice. Exp Neuro. 2021 Jan;335:113496

• Stösser S, Ullrich L, Kassubek J, Ludolph AC, Schocke M, Neugebauer H. Recent silent infarcts do not increase the risk of haemorrhage after intravenous thrombolysis. Eur J Neurol 2020;27(12):2483-2490

• Unrath A., Hans‐Peter Müller, Axel Riecker, Albert C. Ludolph, Anne‐Dorte Sperfeld, Jan Kassubek Whole brain‐based analysis of regional white matter tract alterations in rare motor neuron diseases by diffusion tensor imaging. Hum Brain Mapp. 2010 Nov; 31(11): 1727–1740. Published online 2010 Mar 24. doi:10.1002/hbm.20971 PMCID: PMC6871219

• Urban DL, Scholle LM, Wagner M, Ludolph AC, Rosenbohm A. The m.9143T>C Variant: Recurrent Infections and Immunodeficiency as an Extension of the Phenotypic Spectrum in MT-ATP6 Mutations? Diseases 2020;8(2):19

• Van Schoor E, Koper MJ, Ospitalieri S, Dedeene L, Tomé SO, Vandenberghe R, Brenner D, Otto M, Weishaupt J, Ludolph AC, Van Damme P, Van Den Bosch L, Thal DR. Necrosome-positive granulovacuolar degeneration is associated with TDP-43 pathological lesions in the hippocampus of ALS/FTLD cases. Neuropathol Appl Neurobiol 2020 Sep 18. doi: 10.1111/nan.12668. Online ahead of print.

• Vázquez Medrano CR, Aho-Özhan HEA, Weiland U, Uttner I, Ludolph AC, Lulé D. Disease progression but not physical state per se determines mental wellbeing in ALS. Neurol 2020;267(12):3593-3601

• Vollmuth C, Stoesser S, Neugebauer H, Hansel A, Dreyhaupt J, Ludolph AC, Kassubek J, Althaus K. Correction to: MR imaging pattern is not a predictor of occult atrial fibrillation in patients with cryptogenic stroke. J Neurol 2020;267(10):3117

• Wurster CD, Steinacker P, Günther R, Koch JC, Lingor P, Uzelac Z, Witzel S, Wollinsky K, Winter B, Osmanovic A, Schreiber-Katz O, Al Shweiki R, Ludolph AC, Petri S, Hermann A, Otto M; MND-Net. Neurofilament light chain in serum of adolescent and adult SMA patients under treatment with nusinersen. J Neuro. 2020;267(1):36-44

• Yilmaz R, Müller K, Brenner D, Volk AE, Borck G, Hermann A, Meitinger T, Strom TM, Danzer KM, Ludolph AC, Andersen PM, Weishaupt JH; German ALS Network MND-NET. SQSTM1/p62 variants in 486 patients with familial ALS from Germany and Sweden. Neurobiol Aging 2020;87:139.e9-139.e1

• Yilmazer-Hanke D, Mayer T, Müller HP, Neugebauer H, Abaei A, Scheuerle A, Weis J, Forsberg KME, Althaus K, Meier J, Ludolph AC, Del Tredici K, Braak H, Kassubek J, Rasche V. Histological correlates of postmortem ultra-high-resolution single-section MRI in cortical cerebral microinfarcts. Acta Neuropathol Commun 2020;8(1):33

• Al Shweiki MR, Steinacker P, Oeckl P, Hengerer B, Danek A, Fassbender K, Diehl-Schmid J, Jahn H, Anderl-Straub S, Ludolph AC, Schönfeldt-Lecuona C, Otto M. Neurofilament light chain as a blood biomarker to differentiate psychiatric disorders from behavioural variant frontotemporal dementia. J Psychiatr Res 2019;113:137-140
• Albrecht F, Mueller K, Ballarini T, Lampe L, Diehl-Schmid J, Fassbender K, Fliessbach K, Jahn H, Jech R, Kassubek J, Kornhuber J, Landwehrmeyer B, Lauer M, Ludolph AC, Lyros E, Prudlo J, Schneider A, Synofzik M, Wiltfang J, Danek A, Otto M; FTLD-Consortium, Schroeter ML. Unraveling corticobasal syndrome and alien limb syndrome with structural brain imaging. Cortex 2019;117:33-40
• Al-Chalabi A, Shaw P, Leigh PN, van den Berg L, Hardiman O, Ludolph AC, Aho VV, Sarapohja T, Kuoppamäki M. Oral levosimendan in amyotrophic lateral sclerosis: a phase II multicentre, randomised, double-blind, placebo-controlled trial. J Neurol Neurosurg Psych 2019;90(10):1165-1170
• Becker W, Gorges M, Lulé D, Pinkhardt E,  Ludolph AC, Kassubek J. Saccadic intrusions in amyotrophic lateral sclerosis (ALS). J Eye Movement Research 2019;12 (6):81-17
• Brenner  D, Baumgartner T, von Spiczak S, Lewerenz J, Weis R, Grimmer A, Gaspirova P,  Wurster CD,  Kunz WS, Wagner J,  Minassian BA, Elger C, Ludolph AC, Biskup S, Döcker D. Genotypes and phenotypes of patients with Lafora disease living in Germany. Neurological Research and Practice 2019;1:34
• Brenner D, Müller K, Gastl R, Gorges M, Otto M, Pinkhardt EH, Kassubek J, Weishaupt JH, Ludolph AC. Analysis of CACNA1A CAG repeat lengths in patients with familial ALS. Neurobiol Aging 2019;74:235.e5-235.e8
• Brenner D, Rosenbohm A, Yilmaz R, Müller K, Grehl T, Petri S, Meyer T, Grosskreutz J, Weydt P, Ruf W, Neuwirth C, Weber M, Pinto S, Claeys KG, Schrank B, Jordan B, Knehr A, Günther K, Hübers A, Zeller D, Kubisch C, Jablonka S, Sendtner M, Klopstock T, de Carvalho M, Sperfeld A, Borck G, Volk AE, Dorst J, Weis J, Otto M, Schuster J, Del Tredici K, Braak H, Danzer KM, Freischmidt A, Meitinger T, Ludolph AC, Andersen PM, Weishaupt JH; German ALS network MND-NET. Reply: Adult-onset distal spinal muscular atrophy: a new phenotype associated with KIF5A mutations. Brain 2019;142(12):e67
• Brenner D, Sieverding K, Bruno C, Lüningschrör P, Buck E, Mungwa S, Fischer L, Brockmann SJ, Ulmer J, Bliederhäuser C, Philibert CE, Satoh T, Akira S, Boillée S, Mayer B, Sendtner M, Ludolph AC, Danzer KM, Lobsiger CS, Freischmidt A, Weishaupt JH. Heterozygous Tbk1 loss has opposing effects in early and late stages of ALS in mice. J Exp Med 2019;216(2):267-278
• Brittain C, McCarthy A, Irizarry MC, McDermott D, Biglan K, Höglinger GU, Lorenzl S, Del Ser T, Boxer AL; AL-108-231 Study Group; PROPSPERA investigators; 4RNTI-1authors; Tau Restoration on PSP (TAUROS) Investigators [incl. Ludolph AC, Kassubek J]. Severity dependent distribution of impairments in PSP and CBS: Interactive visualizations. Parkinsonism Relat Disord 2019;60:138-145
• Catanese A, Olde Heuvel F, Mulaw M, Demestre M, Higelin J, Barbi G, Freischmidt A, Weishaupt JH, Ludolph AC, Roselli F, Boeckers TM. Retinoic acid worsens ATG10-dependent autophagy impairment in TBK1-mutant hiPSC-derived motoneurons through SQSTM1/p62 accumulation. Autophagy 2019;15(10):1719-1737
• Chandrasekar A, Heuvel FO, Tar L, Hagenston AM, Palmer A, Linkus B, Ludolph AC, Huber-Lang M, Boeckers T, Bading H, Roselli F. Parvalbumin Interneurons Shape Neuronal Vulnerability in Blunt TBI. Cereb Cortex 2019;29(6):2701-2715
• Chen Z, Chen JA, Shatunov A, Jones AR, Kravitz SN, Huang AY, Lawrence L, Lowe JK, Lewis CM, Payan CAM, Lieb W, Franke A, Deloukas P, Amouyel P, Tzourio C, Dartigues JF; NNIPPS and BBBIPPS Study Groups, Ludolph AC, Bensimon G, Leigh PN, Bronstein JM, Coppola G, Geschwind DH, Al-Chalabi A. Genome-wide survey of copy number variants finds MAPT duplications in progressive supranuclear palsy. Mov Disord 2019;34(7):1049-1059
• Daria T, Müller K, Oidovdorj G, Baatar K, Boldbaatar P, Sarangerel J, Rentsenbat M, Turbat S, Yadamsuren E, Weydt P, Dambasuren B, Bosookhuu O, Banzrai C, Damchaa B, Pinkhardt EH, Rosenbohm A, Högel J, Andersen P, Borck G, Batmunkh M, Ludolph AC, Weishaupt JH. Genotypes of amyotrophic lateral sclerosis in Mongolia. J Neurol Neurosurg Psych;90(11):1300-1302
• Dekker AM, Diekstra FP, Pulit SL, Tazelaar GHP, van der Spek RA, van Rheenen W, van Eijk KR, Calvo A, Brunetti M, Damme PV, Robberecht W, Hardiman O, McLaughlin R, Chiò A, Sendtner M, Ludolph AC, Weishaupt JH, Pardina JSM, van den Berg LH, Veldink JH. Exome array analysis of rare and low frequency variants in amyotrophic lateral sclerosis. Sci Rep 2019;9(1):5931
• Diehl-Schmid J, Licata A, Goldhardt O, Förstl H, Yakushew I, Otto M, Anderl-Straub S, Beer A, Ludolph AC, Landwehrmeyer GB, Levin J, Danek A, Fliessbach K, Spottke A, Fassbender K, Lyros E, Prudlo J, Krause BJ, Volk A, Edbauer D, Schroeter ML, Drzezga A, Kornhuber J, Lauer M; FTLDc Study Group, Grimmer T. FDG-PET underscores the key role of the thalamus in frontotemporal lobar degeneration caused by C9ORF72 mutations. Transl Psychiatry 2019;9(1):54
• Dorst J, Behrendt G, Ludolph AC. Non-invasive ventilation and hypercapnia-associated symptoms in amyotrophic lateral sclerosis. Acta Neurol Scand 2019;139(2):128-134
• Dorst J, Chen L, Rosenbohm A, Dreyhaupt J, Hübers A, Schuster J, Weishaupt JH, Kassubek J, Gess B, Meyer T, Weyen U, Hermann A, Winkler J, Grehl T, Hagenacker T, Lingor P, Koch JC, Sperfeld A, Petri S, Großkreutz J, Metelmann M, Wolf J, Winkler AS, Klopstock T, Boentert M, Johannesen S, Storch A, Schrank B, Zeller D, Liu XL, Tang L, Fan DS, Ludolph AC. Prognostic factors in ALS: a comparison between Germany and China. J Neurol 2019;266(6):1516-1525
• Dorst J, Fangerau T, Taranu D, Eichele P, Dreyhaupt J, Michels S, Schuster J, Ludolph AC, Senel M, Tumani H. Safety and efficacy of immunoadsorption versus plasma exchange in steroid-refractory relapse of multiple sclerosis and clinically isolated syndrome: A randomised, parallel-group, controlled trial. EClinicalMedicine 2019;16:98-106
• Dorst J, Ludolph AC. Non-invasive ventilation in amyotrophic lateral sclerosis. Ther Adv Neurol Disord 2019;12:1756286419857040
• Forouhideh Y, Müller K, Ruf W, Assi M, Seker T, Tunca C, Knehr A, Strom TM, Gorges M, Schradt F, Meitinger T, Ludolph AC, Pinkhardt EH, Basak AN, Kassubek J, Uttner I, Weishaupt JH. A biallelic mutation links MYORG to autosomal-recessive primary familial brain calcification. Brain 2019 1;142(2):e4
• Gerner ST, Kuramatsu JB, Sembill JA, Sprügel MI, Hagen M, Knappe RU, Endres M, Haeusler KG, Sobesky J, Schurig J, Zweynert S, Bauer M, Vajkoczy P, Ringleb PA, Purrucker JC, Rizos T, Volkmann J, Müllges W, Kraft P, Schubert AL, Erbguth F, Nueckel M, Schellinger PD, Glahn J, Knappe UJ, Fink GR, Dohmen C, Stetefeld H, Fisse AL, Minnerup J, Hagemann G, Rakers F, Reichmann H, Schneider H, Rahmig J, Ludolph AC, Stösser S, Neugebauer H, Röther J, Michels P, Schwarz M, Reimann G, Bäzner H, Schwert H, Claßen J, Michalski D, Grau A, Palm F, Urbanek C, Wöhrle JC, Alshammari F, Horn M, Bahner D, Witte OW, Günther A, Hamann GF, Engelhorn T, Lücking H, Dörfler A, Schwab S, Huttner HB. Characteristics in Non-Vitamin K Antagonist Oral Anticoagulant-Related Intracerebral Hemorrhage. Stroke 2019;50(6):1392-1402
• Gorges M, Kuntz B, Del Tredici K, Schmidt DG, Müller HP, Ludolph AC, Dupuis L, Kassubek J. Morphological MRI investigations of the hypothalamus in 232 individuals with Parkinson's disease. Mov Disord 2019;34(10):1566-1570
• Grozdanov V, Bousset L, Hoffmeister M, Bliederhaeuser C, Meier C, Madiona K, Pieri L, Kiechle M, McLean PJ, Kassubek J, Behrends C, Ludolph AC, Weishaupt JH, Melki R, Danzer KM. Increased Immune Activation by Pathologic α-Synuclein in Parkinson's Disease. Ann Neurol 2019;86(4):593-606
• Günther R, Wurster CD, Cordts I, Koch JC, Kamm C, Petzold D, Aust E, Deschauer M, Lingor P, Ludolph AC, Hermann A. Patient-Reported Prevalence of Non-motor Symptoms Is Low in Adult Patients Suffering From 5q Spinal Muscular Atrophy. Front Neurol 2019;10:1098
• Kiechle M, von Einem B, Höfs L, Voehringer P, Grozdanov V, Markx D, Parlato R, Wiesner D, Mayer B, Sakk O, Baumann B, Lukassen S, Liss B, Ekici AB, Ludolph AC, Walther P, Ferger B, McLean PJ, Falkenburger BH, Weishaupt JH, Danzer KM. In Vivo Protein Complementation Demonstrates Presynaptic α-Synuclein Oligomerization and Age-Dependent Accumulation of 8-16-mer Oligomer Species. Cell Rep 2019;29(9):2862-2874
• Klenk J, Peter RS, Rapp K, Dallmeier D, Rothenbacher D, Denkinger M, Büchele G; ActiFE Study Group, Becker T, Böhm B, Scharffetter-Kochanek K, Stingl J, Koenig W, Riepe M, Peter R, Geiger H, Ludolph AC, von Arnim C, Nagel G, Weinmayr G, Steinacker JM, Laszlo R. Lazy Sundays: role of day of the week and reactivity on objectively measured physical activity in older people. Eur Rev Aging Phys Act 2019;16:18
• Kuramatsu JB, Biffi A, Gerner ST, Sembill JA, Sprügel MI, Leasure A, Sansing L, Matouk C, Falcone GJ, Endres M, Haeusler KG, Sobesky J, Schurig J, Zweynert S, Bauer M, Vajkoczy P, Ringleb PA, Purrucker J, Rizos T, Volkmann J, Müllges W, Kraft P, Schubert AL, Erbguth F, Nueckel M, Schellinger PD, Glahn J, Knappe UJ, Fink GR, Dohmen C, Stetefeld H, Fisse AL, Minnerup J, Hagemann G, Rakers F, Reichmann H, Schneider H, Rahmig J, Ludolph AC, Stösser S, Neugebauer H, Röther J, Michels P, Schwarz M, Reimann G, Bäzner H, Schwert H, Claßen J, Michalski D, Grau A, Palm F, Urbanek C, Wöhrle JC, Alshammari F, Horn M, Bahner D, Witte OW, Günther A, Hamann GF, Hagen M, Roeder SS, Lücking H, Dörfler A, Testai FD, Woo D, Schwab S, Sheth KN, Huttner HB. Association of Surgical Hematoma Evacuation vs Conservative Treatment With Functional Outcome in Patients With Cerebellar Intracerebral Hemorrhage. JAMA 2019;322:1392-1403
• Kuzma-Kozakiewicz M, Andersen PM, Ciecwierska K, Vázquez C, Helczyk O, Loose M, Uttner I, Ludolph AC, Lulé D. An observational study on quality of life and preferences to sustain life in locked-in state. Neurology 2019;93(10):e938-e945
• Lingor P, Weber M, Camu W, Friede T, Hilgers R, Leha A, Neuwirth C, Günther R, Benatar M, Kuzma-Kozakiewicz M, Bidner H, Blankenstein C, Frontini R, Ludolph AC, Koch JC; ROCK-ALS Investigators. ROCK-ALS: Protocol for a Randomized, Placebo-Controlled, Double-Blind Phase IIa Trial of Safety, Tolerability and Efficacy of the Rho Kinase (ROCK) Inhibitor Fasudil in Amyotrophic Lateral Sclerosis. Front Neurol 2019;10:293
• Ludolph AC, Dorst J, Dreyhaupt J, Weishaupt JH, Kassubek J, Weiland U, Meyer T, Petri S, Hermann A, Emmer A, Grosskreutz J, Grehl T, Zeller D, Boentert M, Schrank B, Prudlo J, Winkler AS, Gorbulev S, Roselli F, Schuster J, Dupuis L; LIPCAL-ALS Study Group. Effect of High-Caloric Nutrition on Survival in Amyotrophic Lateral Sclerosis. Ann Neurol. 2019 Dec 17 [Epub ahead of print]
• Ludolph AC, Wurster CD. Therapeutic advances in SMA. Curr Opin Neurol 2019;32(5):777-781
• Ludolph AC. Can we turn mice into men?: An Editorial for 'Effects of cannabinoids in Amyotrophic Lateral Sclerosis (ALS) murine models: a systematic review and meta-analysis' page 284. J Neurochem 2019; 149(2): 168-169
• Lulé D, Kübler A, Ludolph AC. Ethical Principles in Patient-Centered Medical Care to Support Quality of Life in Amyotrophic Lateral Sclerosis. Front Neurol 2019;10:259
• Lulé DE, Aho-Özhan HEA, Vázquez C, Weiland U, Weishaupt JH, Otto M, Anderl-Straub S, Semler E, Uttner I, Ludolph AC. Story of the ALS-FTD continuum retold: rather two distinct entities. J Neurol Neurosurg Psych 2019;90(5):586-589
• Lulé DE, Ludolph AC. In vivo tracking of TDP43 in ALS: cognition as a new biomarker for brain pathology. J Neurol Neurosurg Psych 2019 Okt  31. pii: jnnp-2019-321940. doi: 10.1136/jnnp-2019-321940. [Epub ahead of print]
• Müller HP, Brenner D, Roselli F, Wiesner D, Abaei A, Gorges M, Danzer KM, Ludolph AC, Tsao W, Wong PC, Rasche V, Weishaupt JH, Kassubek J. Longitudinal diffusion tensor magnetic resonance imaging analysis at the cohort level reveals disturbed cortical and callosal microstructure with spared corticospinal tract in the TDP-43 G298S ALS mouse model. Transl Neurodegener 2019;8:27
• Müller HP, Gorges M, Del Tredici K, Ludolph AC, Kassubek J. The same cortico-efferent tract involvement in progressive bulbar palsy and in 'classical' ALS: A tract of interest-based MRI study. Neuroimage Clin 2019;24:101979
• Müller HP, Huppertz HJ, Dreyhaupt J, Ludolph AC, Tabrizi SJ, Roos RAC, Durr A, Landwehrmeyer GB, Kassubek J. Combined cerebral atrophy score in Huntington's disease based on atlas-based MRI volumetry: Sample size calculations for clinical trials. Parkinsonism Relat Disord 2019;63:179-184
• Naumann M, Peikert K, Günther R, van der Kooi AJ, Aronica E, Hübers A, Danel V, Corcia P, Pan-Montojo F, Cirak S, Haliloglu G, Ludolph AC, Goswami A, Andersen PM, Prudlo J, Wegner F, Van Damme P, Weishaupt JH, Hermann A. Phenotypes and malignancy risk of different FUS mutations in genetic amyotrophic lateral sclerosis. Ann Clin Transl Neurol 2019;6(12):2384-2394
• Oeckl P, Halbgebauer S, Anderl-Straub S, Steinacker P, Huss AM, Neugebauer H, von Arnim CAF, Diehl-Schmid J, Grimmer T, Kornhuber J, Lewczuk P, Danek A; Consortium for Frontotemporal Lobar Degeneration German, Ludolph AC, Otto M. Glial Fibrillary Acidic Protein in Serum is Increased in Alzheimer's Disease and Correlates with Cognitive Impairment. J Alzheimers Dis 2019;67(2):481-488
• Oeckl P, Weydt P, Steinacker P, Anderl-Straub S, Nordin F, Volk AE, Diehl-Schmid J, Andersen PM, Kornhuber J, Danek A, Fassbender K, Fliessbach K; German Consortium for Frontotemporal Lobar Degeneration, Jahn H, Lauer M, Müller K, Knehr A, Prudlo J, Schneider A, Thal DR, Yilmazer-Hanke D, Weishaupt JH, Ludolph AC, Otto M. Different neuroinflammatory profile in amyotrophic lateral sclerosis and frontotemporal dementia is linked to the clinical phase. J Neurol Neurosurg Psychiatry 2019;90(1):4-10
• Olde Heuvel F, Holl S, Chandrasekar A, Li Z, Wang Y, Rehman R, Förstner P, Sinske D, Palmer A, Wiesner D, Ludolph AC, Huber-Lang M, Relja B, Wirth T, Röszer T, Baumann B, Boeckers T, Knöll B, Roselli F. STAT6 mediates the effect of ethanol on neuroinflammatory response in TBI. Brain Behav Immun 2019;81:228-246
• Picchiarelli G, Demestre M, Zuko A, Been M, Higelin J, Dieterlé S, Goy MA, Mallik M, Sellier C, Scekic-Zahirovic J, Zhang L, Rosenbohm A, Sijlmans C, Aly A, Mersmann S, Sanjuan-Ruiz I, Hübers A, Messaddeq N, Wagner M, van Bakel N, Boutillier AL, Ludolph AC, Lagier-Tourenne C, Boeckers TM, Dupuis L, Storkebaum E. FUS-mediated regulation of acetylcholine receptor transcription at neuromuscular junctions is compromised in amyotrophic lateral sclerosis. Nat Neurosci 2019;22(11):1793-1805
• Pinkhardt EH, Liu H, Ma D, Chen J, Pachollek A, Kunz MS, Kassubek J, Ludolph AC, Huang Y, Chen H, Landwehrmeyer GB, Wang Z, Su W. Olfactory screening of Parkinson's Disease patients and healthy subjects in China and Germany: A study of cross-cultural adaptation of the Sniffin' Sticks 12-identification test. PLoS One 2019;14(11):e0224331
• Rué L, Oeckl P, Timmers M, Lenaerts A, van der Vos J, Smolders S, Poppe L, de Boer A, Van Den Bosch L, Van Damme P, Weishaupt JH, Ludolph AC, Otto M, Robberecht W, Lemmens R. Reduction of ephrin-A5 aggravates disease progression in amyotrophic lateral sclerosis. Acta Neuropathol Commun 2019;7(1):114
• Schönecker S, Hell F, Bötzel K, Wlasich E, Ackl N, Süßmair C; German FTLD Consortium, Otto M, Anderl-Straub S, Ludolph AC, Kassubek J, Huppertz HJ, Diehl-Schmid J, Riedl L, Roßmeier C, Fassbender K, Lyros E, Kornhuber J, Oberstein TJ, Fliessbach K, Schneider A, Schroeter ML, Prudlo J, Lauer M, Jahn H, Levin J, Danek A. The applause sign in frontotemporal lobar degeneration and related conditions.  J Neurol 2019;266(2):330-338
• Semler E, Petersdorff L, Anderl-Straub S, Böhm S, Lulé D, Fangerau H, Ludolph AC, Otto M, Uttner I. Moral judgment in patients with behavioral variant of frontotemporal dementia and amyotrophic lateral sclerosis: no impairment of the moral position, but rather its execution. Amyotroph Lateral Scler Frontotemporal Degener 2019;20(1-2):12-18
• Senel M, Mojib-Yezdani F, Braisch U, Bachhuber F, Lewerenz J, Ludolph AC, Otto M, Tumani H. CSF Free Light Chains as a Marker of Intrathecal Immunoglobulin Synthesis in Multiple Sclerosis: A Blood-CSF Barrier Related Evaluation in a Large Cohort. Front Immunol 2019;10:641
• Shefner JM, Cudkowicz ME, Hardiman O, Cockcroft BM, Lee JH, Malik FI, Meng L, Rudnicki SA, Wolff AA, Andrews JA; VITALITY-ALS Study Group [incl Ludolph AC]. A phase III trial of tirasemtiv as a potential treatment for amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2019;0(0):1-11
• Shu J, Neugebauer H, Li F, Lulé D, Müller HP, Zhang J, Ludolph AC, Huang Y, Kassubek J, Zhang W. Clinical and neuroimaging disparity between Chinese and German patients with cerebral small vessel disease: a comparative study. Sci Rep 2019;9(1):20015
• Sprügel MI, Sembill JA, Kuramatsu JB, Gerner ST, Hagen M, Roeder SS, Endres M, Haeusler KG, Sobesky J, Schurig J, Zweynert S, Bauer M, Vajkoczy P, Ringleb PA, Purrucker JC, Rizos T, Volkmann J, Muellges W, Kraft P, Schubert AL, Erbguth F, Nueckel M, Schellinger PD, Glahn J, Knappe UJ, Fink GR, Dohmen C, Stetefeld H, Fisse AL, Minnerup J, Hagemann G, Rakers F, Reichmann H, Schneider H, Wöpking S, Ludolph AC, Stösser S, Neugebauer H, Röther J, Michels P, Schwarz M, Reimann G, Bäzner H, Schwert H, Classen J, Michalski D, Grau A, Palm F, Urbanek C, Wöhrle JC, Alshammari F, Horn M, Bahner D, Witte OW, Guenther A, Hamann GF, Lücking H, Dörfler A, Schwab S, Huttner HB. Heparin for prophylaxis of venous thromboembolism in intracerebral haemorrhage. J Neurol Neurosurg Psych 2019;90(7):783-791
• Stösser S, Böckler S, Ludolph AC, Kassubek J, Neugebauer H. Juxtacortical lesions are associated with seizures in cerebral small vessel disease. J Neurol 2019;266(5):1230-1235
• Tazelaar GHP, Dekker AM, van Vugt JJFA, van der Spek RA, Westeneng HJ, Kool LJBG, Kenna KP, van Rheenen W, Pulit SL, McLaughlin RL, Sproviero W, Iacoangeli A, Hübers A, Brenner D, Morrison KE, Shaw PJ, Shaw CE, Panadés MP, Mora Pardina JS, Glass JD, Hardiman O, Al-Chalabi A, van Damme P, Robberecht W, Landers JE, Ludolph AC, Weishaupt JH, van den Berg LH, Veldink JH, van Es MA; Project MinE ALS Sequencing Consortium. Association of NIPA1 repeat expansions with amyotrophic lateral sclerosis in a large international cohort. Neurobiol Aging 2019;74:234.e9-234.e15
• Verde F, Steinacker P, Weishaupt JH, Kassubek J, Oeckl P, Halbgebauer S, Tumani H, von Arnim CAF, Dorst J, Feneberg E, Mayer B, Müller HP, Gorges M, Rosenbohm A, Volk AE, Silani V, Ludolph AC, Otto M. Neurofilament light chain in serum for the diagnosis of amyotrophic lateral sclerosis. J Neurol Neurosurg Psych 2019;90(2):157-164
• Vollmuth C, Stoesser S, Neugebauer H, Hansel A, Dreyhaupt J, Ludolph AC, Kassubek J, Althaus K. MR-imaging pattern is not a predictor of occult atrial fibrillation in patients with cryptogenic stroke. J Neurol 2019;266(12):3058-3064
• Walter MC, Dräger B, Günther R, Hermann A, Hagenacker T, Kleinschnitz C, Löscher W, Meyer T, Schrank B, Schwersenz I, Wurster CD, Ludolph AC, Kirschner J. [Treatment evaluation in patients with 5q-associated spinal muscular atrophy : Real-world experience]. Nervenarzt 2019;90(4):343-351
• Winter B, Guenther R, Ludolph AC, Hermann A, Otto M, Wurster CD. Neurofilaments and tau in CSF in an infant with SMA type 1 treated with nusinersen. J Neurol Neurosurg Psych 2019;90(9):1068-1069
• Winzel S, Ludolph AC. Aktuelle und zukünftige Therapieansätze bei der amyotrophen Lateralsklerose. Info Neurol Psych 2019;21(9):30-37
• Winzel S, Ludolph AC. Amyotrophe Lateralsklerose früh erkennen und behandeln. Der Neurologe & Psychiater 2019;20(2):54-61
• Wurster CD, Günther R, Steinacker P, Dreyhaupt J, Wollinsky K, Uzelac Z, Witzel S, Kocak T, Winter B, Koch JC, Lingor P, Petri S, Ludolph AC, Hermann A, Otto M. Neurochemical markers in CSF of adolescent and adult SMA patients undergoing nusinersen treatment. Ther Adv Neurol Disord 2019;12:1756286419846058
• Wurster CD, Koch JC, Cordts I, Dreyhaupt J, Otto M, Uzelac Z, Witzel S, Winter B, Kocak T, Schocke M, Weydt P, Wollinsky K, Ludolph AC, Deschauer M, Lingor P, Tumani H, Hermann A, Günther R. Routine Cerebrospinal Fluid (CSF) Parameters in Patients With Spinal Muscular Atrophy (SMA) Treated With Nusinersen. Front Neurol 2019;10:1179
• Wurster CD, Steinacker P, Günther R, Koch JC, Lingor P, Uzelac Z, Witzel S, Wollinsky K, Winter B, Osmanovic A, Schreiber-Katz O, Al Shweiki R, Ludolph AC, Petri S, Hermann A, Otto M; MND-Net. Neurofilament light chain in serum of adolescent and adult SMA patients under treatment with nusinersen. J Neurol. 2019 Sep 24. doi: 10.1007/s00415-019-09547-y. [Epub ahead of print]
• Wurster CD, Winter B, Wollinsky K, Ludolph AC, Uzelac Z, Witzel S, Schocke M, Schneider R, Kocak T. Intrathecal administration of nusinersen in adolescent and adult SMA type 2 and 3 patients. J Neurol 2019;266(1):183-194
• Ye S, Rosenbohm A, Böhm S, Uttner I, Ji Y, Ludolph AC, Lulé D, Fan D. Cognitive and behavioral impairments in German and Chinese ALS populations - a post-hoc comparison of national study data. Amyotroph Lateral Scler Frontotemporal Degener 2019;20(1-2):28-36
• Yilmaz R, Müller K, Brenner D, Volk AE, Borck G, Hermann A, Meitinger T, Strom TM, Danzer KM, Ludolph AC, Andersen PM, Weishaupt JH; German ALS Network MND-NET. SQSTM1/p62 variants in 486 patients with familial ALS from Germany and Sweden.  Neurobiol Aging. 2019 Nov 2. [Epub ahead of print]
• Zimmermann J, Jesse S, Kassubek J, Pinkhardt E, Ludolph AC. Differential diagnosis of peripheral facial nerve palsy: a retrospective clinical, MRI and CSF-based study. J Neurol 2019;266(10):2488-2494

• Abdelhak A, Hübers A, Böhm K, Ludolph AC, Kassubek J, Pinkhardt EH. In vivo assessment of retinal vessel pathology in amyotrophic lateral sclerosis. J Neurol 2018;265(4):949-953
• Andersen PM, Kuzma-Kozakiewicz M, Keller J, Aho-Oezhan HEA, Ciecwierska K, Szejko N, Vázquez C, Böhm S, Badura-Lotter G, Meyer T, Petri S, Linse K, Hermann A, Semb O, Stenberg E, Nackberg S, Dorst J, Uttner I, Häggström AC, Ludolph AC, Lulé D. Therapeutic decisions in ALS patients: cross-cultural differences and clinical implications. J Neurol 2018;265(7):1600-1606
• Brenner D, Hiergeist A, Adis C, Mayer B, Gessner A, Ludolph AC, Weishaupt JH. The fecal microbiome of ALS patients. Neurobiol Aging 2018;61:132-137
• Brenner D, Yilmaz R, Müller K, Grehl T, Petri S, Meyer T, Grosskreutz J, Weydt P, Ruf W, Neuwirth C, Weber M, Pinto S, Claeys KG, Schrank B, Jordan B, Knehr A, Günther K, Hübers A, Zeller D, Kubisch C, Jablonka S, Sendtner M, Klopstock T, de Carvalho M, Sperfeld A, Borck G, Volk AE, Dorst J, Weis J, Otto M, Schuster J, Del Tredici K, Braak H, Danzer KM, Freischmidt A, Meitinger T, Strom TM, Ludolph AC, Andersen PM, Weishaupt JH; German ALS network MND-NET. Hot-spot KIF5A mutations cause familial ALS. Brain 2018;141(3):688-697
• Brockmann SJ, Freischmidt A, Oeckl P, Müller K, Ponna SK, Helferich AM, Paone C, Reinders J, Kojer K, Orth M, Jokela M, Auranen M, Udd B, Hermann A, Danzer KM, Lichtner P, Walther P, Ludolph AC, Andersen PM, Otto M, Kursula P, Just S, Weishaupt JH. CHCHD10 mutations p.R15L and p.G66V cause motoneuron disease by haploinsufficiency. Hum Mol Genet 2018;27(4):706-715
• Chandrasekar A, Aksan B, Heuvel FO, Förstner P, Sinske D, Rehman R, Palmer A, Ludolph AC, Huber-Lang M, Böckers T, Mauceri D, Knöll B, Roselli F. Neuroprotective effect of acute ethanol intoxication in TBI is associated to the hierarchical modulation of early transcriptional responses. Exp Neurol 2018;302:34-45
• Chandrasekar A, Olde Heuvel F, Wepler M, Rehman R, Palmer A, Catanese A, Linkus B, Ludolph AC, Boeckers T, Huber-Lang M, Radermacher P, Roselli F. The Neuroprotective Effect of Ethanol Intoxication in Traumatic Brain Injury Is Associated with the Suppression of ErbB Signaling in Parvalbumin-Positive Interneurons. J Neurotrauma 2018;35(22):2718-2735
• Chen JA, Chen Z, Won H, Huang AY, Lowe JK, Wojta K, Yokoyama JS, Bensimon G, Leigh PN, Payan C, Shatunov A, Jones AR, Lewis CM, Deloukas P, Amouyel P, Tzourio C, Dartigues JF, Ludolph AC, Boxer AL, Bronstein JM, Al-Chalabi A, Geschwind DH, Coppola G. Joint genome-wide association study of progressive supranuclear palsy identifies novel susceptibility loci and genetic correlation to neurodegenerative diseases. Mol Neurodegener 2018;13(1):41
• Commisso B, Ding L, Varadi K, Gorges M, Bayer D, Boeckers TM, Ludolph AC, Kassubek J, Müller OJ, Roselli F. Stage-dependent remodeling of projections to motor cortex in ALS mouse model revealed by a new variant retrograde-AAV9. Elife 2018;7: e36892
• Dorst J, Ludolph AC, Huebers A. Disease-modifying and symptomatic treatment of amyotrophic lateral sclerosis. Ther Adv Neurol Disord 2018; 11:1756285617734734
• Dorst J, Ludolph AC, Senel M, Tumani H. Short-term and long-term effects of immunoadsorption in refractory chronic inflammatory demyelinating polyneuropathy: a prospective study in 17 patients. J Neurol 2018;265(12):2906-2915
• Feneberg E, Oeckl P, Steinacker P, Verde F, Barro C, Van Damme P, Gray E, Grosskreutz J, Jardel C, Kuhle J, Koerner S, Lamari F, Amador MDM, Mayer B, Morelli C, Muckova P, Petri S, Poesen K, Raaphorst J, Salachas F, Silani V, Stubendorff B, Turner MR, Verbeek MM, Weishaupt JH, Weydt P, Ludolph AC, Otto M. Multicenter evaluation of neurofilaments in early symptom onset amyotrophic lateral sclerosis. Neurology 2018;90(1):e22-e30
• Fissler P, Küster OC, Laptinskaya D, Loy LS, von Arnim CAF, Kolassa IT. Jigsaw Puzzling Taps Multiple Cognitive Abilities and Is a Potential Protective Factor for Cognitive Aging. Front Aging Neurosci 2018;10:299
• Forsberg KME, Zhang Y, Reiners J, Ander M, Niedermayer A, Fang L, Neugebauer H, Kassubek J, Katona I, Weis J, Ludolph AC, Del Tredici K, Braak H, Yilmazer-Hanke D. Endothelial damage, vascular bagging and remodeling of the microvascular bed in human microangiopathy with deep white matter lesions. Acta Neuropathol Commun 2018;6(1):128
• Funke A, Spittel S, Grehl T, Grosskreutz J, Kettemann D, Petri S, Weyen U, Weydt P, Dorst J, Ludolph AC, Baum P, Oberstadt M, Jordan B, Hermann A, Wolf J, Boentert M, Walter B, Gajewski N, Maier A, Münch C, Meyer T. Provision of assistive technology devices among people with ALS in Germany: a platform-case management approach. Amyotroph Lateral Scler Frontotemporal Degener 2018;19(5-6):342-350
• Gorges M, Del Tredici K, Dreyhaupt J, Braak H, Ludolph AC, Müller HP, Kassubek J. Corticoefferent pathology distribution in amyotrophic lateral sclerosis: in vivo evidence from a meta-analysis of diffusion tensor imaging data. Sci Rep 2018;8(1):15389
• Graf H, Lehmann D, Ludolph AC, Wurster CD. Antisense-Oligonukleotide II. Therapeutisches Potenzial bei neuropsychiatrischen Erkrankungen sowie ethische und gesundheitspolitische Aspekte. Nervenheilkunde 2018;18:818-823
• Grunseich C, Miller R, Swan T, Glass DJ, El Mouelhi M, Fornaro M, Petricoul O, Vostiar I, Roubenoff R, Meriggioli MN, Kokkinis A, Guber RD, Budron MS, Vissing J, Soraru G, Mozaffar T, Ludolph AC, Kissel JT, Fischbeck KH; BVS857 study group. Safety, tolerability, and preliminary efficacy of an IGF-1 mimetic in patients with spinal and bulbar muscular atrophy: a randomised, placebo-controlled trial. Lancet Neurol 2018;17(12):1043-1052
• Helferich AM, Brockmann SJ, Reinders J, Deshpande D, Holzmann K, Brenner D, Andersen PM, Petri S, Thal DR, Michaelis J, Otto M, Just S, Ludolph AC, Danzer KM, Freischmidt A, Weishaupt JH. Dysregulation of a novel miR-1825/TBCB/TUBA4A pathway in sporadic and familial ALS. Cell Mol Life Sci 2018;75(23):4301-4319
• Henstridge CM, Sideris DI, Carroll E, Rotariu S, Salomonsson S, Tzioras M, McKenzie CA, Smith C, von Arnim CAF, Ludolph AC, Lulé D, Leighton D, Warner J, Cleary E, Newton J, Swingler R, Chandran S, Gillingwater TH, Abrahams S, Spires-Jones TL. Synapse loss in the prefrontal cortex is associated with cognitive decline in amyotrophic lateral sclerosis. Acta Neuropathol 2018;135(2):213-226
• Higelin J, Catanese A, Semelink-Sedlacek LL, Oeztuerk S, Lutz AK, Bausinger J, Barbi G, Speit G, Andersen PM, Ludolph AC, Demestre M, Boeckers TM. NEK1 loss-of-function mutation induces DNA damage accumulation in ALS patient-derived motoneurons. Stem Cell Res 2018;30:150-162
• Hübers A, Thoma K, Schocke M, Fauser S, Ludolph AC, Kassubek J, Pinkhardt EH. Acute DWI Reductions In Patients After Single Epileptic Seizures - More Common Than Assumed. Front Neurol 2018;9:550
• Jeck J, Kassubek R, Coburger J, Edenhofer S, Schönsteiner SS, Ludolph AC, Schmitz B, Engelke J, Mayer-Steinacker R, Lewerenz J, Bullinger L. Bevacizumab in temozolomide refractory high-grade gliomas: single-centre experience and review of the literature. Ther Adv Neurol Disord 2018;11:1756285617753597
• Kassubek J, Müller HP, Del Tredici K, Hornberger M, Schroeter ML, Müller K, Anderl-Straub S, Uttner I, Grossman M, Braak H, Hodges JR, Piguet O, Otto M, Ludolph AC. Longitudinal Diffusion Tensor Imaging Resembles Patterns of Pathology Progression in Behavioral Variant Frontotemporal Dementia (bvFTD). Front Aging Neurosci 2018;10:47
• Kassubek J, Müller HP, Del Tredici K, Lulé D, Gorges M, Braak H, Ludolph AC. Imaging the pathoanatomy of amyotrophic lateral sclerosis in vivo: targeting a propagation-based biological marker. J Neurol Neurosurg Psych 2018;89(4):374-381
• Keller J, Böhm S, Aho-Özhan HEA, Loose M, Gorges M, Kassubek J, Uttner I, Abrahams S, Ludolph AC, Lulé D. Functional reorganization during cognitive function tasks in patients with amyotrophic lateral sclerosis. Brain Imaging Behav 2018;12(3):771-784
• Krämer LM, Brettschneider J, Lennerz JK, Walcher D, Fang L, Rosenbohm A, Balakrishnan K, Benckendorff J, Möller P, Just S, Willem M, Ludolph AC, Thal DR. Amyloid precursor protein-fragments-containing inclusions in cardiomyocytes with basophilic degeneration and its association with cerebral amyloid angiopathy and myocardial fibrosis. Sci Rep 2018;8(1):16594
• Kuramatsu JB, Sembill JA, Gerner ST, Sprügel MI, Hagen M, Roeder SS, Endres M, Haeusler KG, Sobesky J, Schurig J, Zweynert S, Bauer M, Vajkoczy P, Ringleb PA, Purrucker J, Rizos T, Volkmann J, Müllges W, Kraft P, Schubert AL, Erbguth F, Nueckel M, Schellinger PD, Glahn J, Knappe UJ, Fink GR, Dohmen C, Stetefeld H, Fisse AL, Minnerup J, Hagemann G, Rakers F, Reichmann H, Schneider H, Wöpking S, Ludolph AC, Stösser S, Neugebauer H, Röther J, Michels P, Schwarz M, Reimann G, Bäzner H, Schwert H, Claßen J, Michalski D, Grau A, Palm F, Urbanek C, Wöhrle JC, Alshammari F, Horn M, Bahner D, Witte OW, Günther A, Hamann GF, Lücking H, Dörfler A, Achenbach S, Schwab S, Huttner HB. Management of therapeutic anticoagulation in patients with intracerebral haemorrhage and mechanical heart valves. Eur Heart J 2018;39(19):1709-1723
• Ludolph AC, Schuster J, Dorst J, Dupuis L, Dreyhaupt J, Weishaupt JH, Kassubek J, Weiland U, Petri S, Meyer T, Grosskreutz J, Schrank B, Boentert M, Emmer A, Hermann A, Zeller D, Prudlo J, Winkler AS, Grehl T, Heneka MT, Wollebæk Johannesen S, Göricke B; RAS-ALS Study Group. Safety and efficacy of rasagiline as an add-on therapy to riluzole in patients with amyotrophic lateral sclerosis: a randomised, double-blind, parallel-group, placebo-controlled, phase 2 trial. Lancet Neurol 2018;17(8):681-688
• Ludolph AC. Neurodegenerative Erkrankungen. Klinikarzt 2018;47:442-443
• Lulé D, Böhm S, Müller HP, Aho-Özhan H, Keller J, Gorges M, Loose M, Weishaupt JH, Uttner I, Pinkhardt E, Kassubek J, Del Tredici K, Braak H, Abrahams S, Ludolph AC. Cognitive phenotypes of sequential staging in amyotrophic lateral sclerosis. Cortex 2018;101:163-171
• Lulé D, Hörner K, Vazquez C, Aho-Özhan H, Keller J, Gorges M, Uttner I, Ludolph AC. Screening for Cognitive Function in Complete Immobility Using Brain-Machine Interfaces: A Proof of Principle Study. Front Neurosci 2018;12:517
• Müller HP, Agosta F, Gorges M, Kassubek R, Spinelli EG, Riva N, Ludolph AC, Filippi M, Kassubek J. Cortico-efferent tract involvement in primary lateral sclerosis and amyotrophic lateral sclerosis: A two-centre tract of interest-based DTI analysis. Neuroimage Clin 2018;20:1062-1069
• Müller HP, Agosta F, Riva N, Spinelli EG, Comi G, Ludolph AC, Filippi M, Kassubek J. Fast progressive lower motor neuron disease is an ALS variant: A two-centre tract of interest-based MRI data analysis. Neuroimage Clin 2018;17:145-152
• Müller HP, Gorges M, Kassubek R, Dorst J, Ludolph AC, Kassubek J. Identical patterns of cortico-efferent tract involvement in primary lateral sclerosis and amyotrophic lateral sclerosis: A tract of interest-based MRI study. Neuroimage Clin 2018;18:762-769
• Müller K, Brenner D, Weydt P, Meyer T, Grehl T, Petri S, Grosskreutz J, Schuster J, Volk AE, Borck G, Kubisch C, Klopstock T, Zeller D, Jablonka S, Sendtner M, Klebe S, Knehr A, Günther K, Weis J, Claeys KG, Schrank B, Sperfeld AD, Hübers A, Otto M, Dorst J, Meitinger T, Strom TM, Andersen PM, Ludolph AC, Weishaupt JH; German ALS network MND-NET. Comprehensive analysis of the mutation spectrum in 301 German ALS families. J Neurol Neurosurg Psych 2018;89(8):817-827
• Nagel G, Rothenbacher D, Ludolph AC. Retinoids and Amyotrophic Lateral Sclerosis. JAMA Neurol 2018;75(9):1153-1154
• Naumann M, Pal A, Goswami A, Lojewski X, Japtok J, Vehlow A, Naujock M, Günther R, Jin M, Stanslowsky N, Reinhardt P, Sterneckert J, Frickenhaus M, Pan-Montojo F, Storkebaum E, Poser I, Freischmidt A, Weishaupt JH, Holzmann K, Troost D, Ludolph AC, Boeckers TM, Liebau S, Petri S, Cordes N, Hyman AA, Wegner F, Grill SW, Weis J, Storch A, Hermann A. Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation. Nat Commun 2018;9(1):335
• Nicolas A, Kenna KP, Renton AE, Ticozzi N, Faghri F, Chia R, Dominov JA, Kenna BJ, Nalls MA, Keagle P, Rivera AM, van Rheenen W, Murphy NA, van Vugt JJFA, Geiger JT, Van der Spek RA, Pliner HA, Shankaracharya, Smith BN, Marangi G, Topp SD, Abramzon Y, Gkazi AS, Eicher JD, Kenna A; ITALSGEN Consortium, Mora G, Calvo A, Mazzini L, Riva N, Mandrioli J, Caponnetto C, Battistini S, Volanti P, La Bella V, Conforti FL, Borghero G, Messina S, Simone IL, Trojsi F, Salvi F, Logullo FO, D'Alfonso S, Corrado L, Capasso M, Ferrucci L; Genomic Translation for ALS Care (GTAC) Consortium, Moreno CAM, Kamalakaran S, Goldstein DB; ALS Sequencing Consortium, Gitler AD, Harris T, Myers RM; NYGC ALS Consortium, Phatnani H, Musunuri RL, Evani US, Abhyankar A, Zody MC; Answer ALS Foundation, Kaye J, Finkbeiner S, Wyman SK, LeNail A, Lima L, Fraenkel E, Svendsen CN, Thompson LM, Van Eyk JE, Berry JD, Miller TM, Kolb SJ, Cudkowicz M, Baxi E; Clinical Research in ALS and Related Disorders for Therapeutic Development (CReATe) Consortium, Benatar M, Taylor JP, Rampersaud E, Wu G, Wuu J; SLAGEN Consortium, Lauria G, Verde F, Fogh I, Tiloca C, Comi GP, Sorarù G, Cereda C; French ALS Consortium, Corcia P, Laaksovirta H, Myllykangas L, Jansson L, Valori M, Ealing J, Hamdalla H, Rollinson S, Pickering-Brown S, Orrell RW, Sidle KC, Malaspina A, Hardy J, Singleton AB, Johnson JO, Arepalli S, Sapp PC, McKenna-Yasek D, Polak M, Asress S, Al-Sarraj S, King A, Troakes C, Vance C, de Belleroche J, Baas F, Ten Asbroek ALMA, Muñoz-Blanco JL, Hernandez DG, Ding J, Gibbs JR, Scholz SW, Floeter MK, Campbell RH, Landi F, Bowser R, Pulst SM, Ravits JM, MacGowan DJL, Kirby J, Pioro EP, Pamphlett R, Broach J, Gerhard G, Dunckley TL, Brady CB, Kowall NW, Troncoso JC, Le Ber I, Mouzat K, Lumbroso S, Heiman-Patterson TD, Kamel F, Van Den Bosch L, Baloh RH, Strom TM, Meitinger T, Shatunov A, Van Eijk KR, de Carvalho M, Kooyman M, Middelkoop B, Moisse M, McLaughlin RL, Van Es MA, Weber M, Boylan KB, Van Blitterswijk M, Rademakers R, Morrison KE, Basak AN, Mora JS, Drory VE, Shaw PJ, Turner MR, Talbot K, Hardiman O, Williams KL, Fifita JA, Nicholson GA, Blair IP, Rouleau GA, Esteban-Pérez J, García-Redondo A, Al-Chalabi A; Project MinE ALS Sequencing Consortium, Rogaeva E, Zinman L, Ostrow LW, Maragakis NJ, Rothstein JD, Simmons Z, Cooper-Knock J, Brice A, Goutman SA, Feldman EL, Gibson SB, Taroni F, Ratti A, Gellera C, Van Damme P, Robberecht W, Fratta P, Sabatelli M, Lunetta C, Ludolph AC, Andersen PM, Weishaupt JH, Camu W, Trojanowski JQ, Van Deerlin VM, Brown RH Jr, van den Berg LH, Veldink JH, Harms MB, Glass JD, Stone DJ, Tienari P, Silani V, Chiò A, Shaw CE, Traynor BJ, Landers JE. Genome-wide Analyses Identify KIF5A as a Novel ALS Gene. Neuron 2018;97(6):1268-1283
• Ouali Alami N, Schurr C, Olde Heuvel F, Tang L, Li Q, Tasdogan A, Kimbara A, Nettekoven M, Ottaviani G, Raposo C, Röver S, Rogers-Evans M, Rothenhäusler B, Ullmer C, Fingerle J, Grether U, Knuesel I, Boeckers TM, Ludolph AC, Wirth T, Roselli F, Baumann B. NF-κB activation in astrocytes drives a stage-specific beneficial neuroimmunological response in ALS. EMBO J 2018;37(16) pii:e98697
• Rojas JC, Bang J, Lobach IV, Tsai RM, Rabinovici GD, Miller BL, Boxer AL; AL-108-231 Investigators [incl. Ludolph AC). CSF neurofilament light chain and phosphorylated tau 181 predict disease progression in PSP. Neurology 2018;90(4):e273-e281
• Rosenbohm A, Hirsch S, Volk AE, Grehl T, Grosskreutz J, Hanisch F, Herrmann A, Kollewe K, Kress W, Meyer T, Petri S, Prudlo J, Wessig C, Müller HP, Dreyhaupt J, Weishaupt J, Kubisch C, Kassubek J, Weydt P, Ludolph AC. The metabolic and endocrine characteristics in spinal and bulbar muscular atrophy. J Neurol 2018;265(5):1026-1036
• Rosenbohm A, Liu M, Nagel G, Peter RS, Cui B, Li X, Kassubek J, Rothenbacher D, Lulé D, Cui L, Ludolph AC; ALS Registry Swabia Study Group. Phenotypic differences of amyotrophic lateral sclerosis (ALS) in China and Germany. J Neurol 2018;265(4):774-782
• Rosenbohm A, Nagel G, Peter RS, Brehme T, Koenig W, Dupuis L, Rothenbacher D, Ludolph AC; ALS Registry Study Group. Association of Serum Retinol-Binding Protein 4 Concentration With Risk for and Prognosis of Amyotrophic Lateral Sclerosis. JAMA Neurol 2018;75(5):600-607
• Rosskopf J, Gorges M, Müller HP, Pinkhardt EH, Ludolph AC, Kassubek J. Hyperconnective and hypoconnective cortical and subcortical functional networks in multiple system atrophy. Parkinsonism Relat Disord 2018;49:75-80
• Schmid S, Goldberg-Bockhorn E, Schwarz S, Rotter N, Kassubek J, Del Tredici K, Pinkhardt E, Otto M, Ludolph AC, Oeckl P. Alpha-synuclein is present in dental calculus but not altered in Parkinson's disease patients in comparison to controls. J Neurol 2018;265(6):1334-1337
• Schönecker S, Neuhofer C, Otto M, Ludolph AC, Kassubek J, Landwehrmeyer B, Anderl-Straub S, Semler E, Diehl-Schmid J, Prix C, Vollmar C, Fortea J; Deutsches FTLD-Konsortium, Huppertz HJ, Arzberger T, Edbauer D, Feddersen B, Dieterich M, Schroeter ML, Volk AE, Fließbach K, Schneider A, Kornhuber J, Maler M, Prudlo J, Jahn H, Boeckh-Behrens T, Danek A, Klopstock T, Levin J. Atrophy in the Thalamus But Not Cerebellum Is Specific for C9orf72 FTD and ALS Patients - An Atlas-Based Volumetric MRI Study. Front Aging Neurosci 2018;10:45
• Semler E, Anderl-Straub S, Uttner I, Diehl-Schmid J, Danek A, Einsiedler B, Fassbender K, Fliessbach K, Huppertz HJ, Jahn H, Kornhuber J, Landwehrmeyer B, Lauer M, Muche R, Prudlo J, Schneider A, Schroeter ML, Ludolph AC, Otto M; FTLD consortium. A language-based sum score for the course and therapeutic intervention in primary progressive aphasia. Alzheimers Res Ther 2018;10(1):41
• Steinacker P, Anderl-Straub S, Diehl-Schmid J, Semler E, Uttner I, von Arnim CAF, Barthel H, Danek A, Fassbender K, Fliessbach K, Foerstl H, Grimmer T, Huppertz HJ, Jahn H, Kassubek J, Kornhuber J, Landwehrmeyer B, Lauer M, Maler JM, Mayer B, Oeckl P, Prudlo J, Schneider A, Volk AE, Wiltfang J, Schroeter ML, Ludolph AC, Otto M; FTLDc study group. Serum neurofilament light chain in behavioral variant frontotemporal dementia. Neurology 2018;91(15):e1390-e1401
• Steinacker P, Verde F, Fang L, Feneberg E, Oeckl P, Roeber S, Anderl-Straub S, Danek A, Diehl-Schmid J, Fassbender K, Fliessbach K, Foerstl H, Giese A, Jahn H, Kassubek J, Kornhuber J, Landwehrmeyer GB, Lauer M, Pinkhardt EH, Prudlo J, Rosenbohm A, Schneider A, Schroeter ML, Tumani H, von Arnim CAF, Weishaupt J, Weydt P, Ludolph AC, Yilmazer Hanke D, Otto M; FTLDc study group. Chitotriosidase (CHIT1) is increased in microglia and macrophages in spinal cord of amyotrophic lateral sclerosis and cerebrospinal fluid levels correlate with disease severity and progression. J Neurol Neurosurg Psych 2018;89(3):239-247
• Verde F, Steinacker P, Oeckl P, Weishaupt JH, Rosenbohm A, Silani V, Ludolph AC, Otto M. Chromogranin A levels in the cerebrospinal fluid of patients with amyotrophic lateral sclerosis. Neurobiol Aging 2018;67:21-22
• Volk AE, Weishaupt JH, Andersen PM, Ludolph AC, Kubisch C. Current knowledge and recent insights into the genetic basis of amyotrophic lateral sclerosis. Med Genet 2018;30(2):252-258
• Westeneng HJ, Debray TPA, Visser AE, van Eijk RPA, Rooney JPK, Calvo A, Martin S, McDermott CJ, Thompson AG, Pinto S, Kobeleva X, Rosenbohm A, Stubendorff B, Sommer H, Middelkoop BM, Dekker AM, van Vugt JJFA, van Rheenen W, Vajda A, Heverin M, Kazoka M, Hollinger H, Gromicho M, Körner S, Ringer TM, Rödiger A, Gunkel A, Shaw CE, Bredenoord AL, van Es MA, Corcia P, Couratier P, Weber M, Grosskreutz J, Ludolph AC, Petri S, de Carvalho M, Van Damme P, Talbot K, Turner MR, Shaw PJ, Al-Chalabi A, Chiò A, Hardiman O, Moons KGM, Veldink JH, van den Berg LH. Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model. Lancet Neurol 2018;17(5):423-433
• Wiesner D, Tar L, Linkus B, Chandrasekar A, Olde Heuvel F, Dupuis L, Tsao W, Wong PC, Ludolph AC, Roselli F. Reversible induction of TDP-43 granules in cortical neurons after traumatic injury. Exp Neurol 2018;299(Pt A):15-25
• Wurster CD, Ludolph AC, Lehmann D, Graf F. Antisense-Oligonukleotide I. Chemische Modifikationen, Wirkmechanismen und therapeutischer Einsatz bei neurodegenerative Erkrankungen. Nervenheilkunde 2018;18:809-817
• Wurster CD, Ludolph AC. Antisense oligonucleotides in neurological disorders. Ther Adv Neurol Disord 2018;11:1756286418776932
• Wurster CD, Ludolph AC. Nusinersen for spinal muscular atrophy. Ther Adv Neurol Disord 2018;11:1756285618754459

• Aho-Özhan HE, Böhm S, Keller J, Dorst J, Uttner I, Ludolph AC, Lulé D. Experience matters: neurologists' perspectives on ALS patients' well-being. J Neurol 2017;264(4):639-646
• Al-Chalabi A, Andersen PM, Chandran S, Chio A, Corcia P, Couratier P, Danielsson O, de Carvalho M, Desnuelle C, Grehl T, Grosskreutz J, Holmøy T, Ingre C, Karlsborg M, Kleveland G, Koch JC, Koritnik B, KuzmaKozakiewicz M, Laaksovirta H, Ludolph AC, McDermott C, Meyer T, Mitre Ropero B, Mora Pardina J, Nygren I, Petri S, Povedano Panades M, Salachas F, Shaw P, Silani V, Staaf G, Svenstrup K, Talbot K, Tysnes OB, Van Damme P, van der Kooi A, Weber M, Weydt P, Wolf J, Hardiman O, van den Berg LH. July 2017 ENCALS statement on edaravone. Amyotroph Lateral Scler Frontotemporal Degener 2017;18(7-8):471-474
• Barschke P, Oeckl P, Steinacker P, Ludolph AC, Otto M. Proteomic studies in the discovery of cerebrospinal fluid biomarkers for amyotrophic lateral sclerosis. Expert Rev Proteomics 2017;14(9):769-777
• Bayer H, Lang K, Buck E, Higelin J, Barteczko L, Pasquarelli N, Sprissler J, Lucas T, Holzmann K, Demestre M, Lindenberg KS, Danzer KM, Boeckers T, Ludolph AC, Dupuis L, Weydt P, Witting A. ALS-causing mutations differentially affect PGC-1α expression and function in the brain vs. peripheral tissues. Neurobiol Dis 2017;97(Pt A):36-45
• Bisenius S, Mueller K, Diehl-Schmid J, Fassbender K, Grimmer T, Jessen F, Kassubek J, Kornhuber J, Landwehrmeyer B, Ludolph AC, Schneider A, Anderl-Straub S, Stuke K, Danek A, Otto M, Schroeter ML; FTLDc study group. Predicting primary progressive aphasias with support vector machine approaches in structural MRI data. Neuroimage Clin 2017;14:334-343
• Braak H, Ludolph AC, Neumann M, Ravits J, Del Tredici K. Pathological TDP-43 changes in Betz cells differ from those in bulbar and spinal α-motoneurons in sporadic amyotrophic lateral sclerosis. Acta Neuropathol 2017;133(1):79-90
• Braak H, Neumann M, Ludolph AC, Del Tredici K. Does Sporadic Amyotrophic Lateral Sclerosis Spread via Axonal Connectivities? Neurology International Open 2017; 1: E136–E141
• Buck E, Bayer H, Lindenberg KS, Hanselmann J, Pasquarelli N, Ludolph AC, Weydt P, Witting A. Comparison of Sirtuin 3 Levels in ALS and Huntington's Disease-Differential Effects in Human Tissue Samples vs. Transgenic Mouse Models. Front Mol Neurosci 2017;10:156
• Chandrasekar A, Heuvel FO, Palmer A, Linkus B, Ludolph AC, Boeckers TM, Relja B, Huber-Lang M, Roselli F. Acute ethanol administration results in a protective cytokine and neuroinflammatory profile in traumatic brain injury. Int Immunopharmacol 2017;51:66-75
• Eisen A, Braak H, Del Tredici K, Lemon R, Ludolph AC, Kiernan MC. Cortical influences drive amyotrophic lateral sclerosis. J Neurol Neurosurg Psych 2017;88(11):917-924
• Endo S, Takada S, Honda RP, Müller K, Weishaupt JH, Andersen PM, Ludolph AC, Kamatari YO, Matsunaga T, Kuwata K, El-Kabbani O, Ikari A. Instability of C154Y variant of aldo-keto reductase 1C3. Chem Biol Interact 2017;276:194-202
• Freischmidt A, Müller K, Ludolph AC, Weishaupt JH, Andersen PM. Association of Mutations in TBK1 With Sporadic and Familial Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. JAMA Neurol 2017;74(1):110-113
• Geisler T, Poli S, Meisner C, Schreieck J, Zuern CS, Nägele T, Brachmann J, Jung W, Gahn G, Schmid E, Bäezner H, Keller T, Petzold GC, Schrickel JW, Liman J, Wachter R, Schön F, Schabet M, Lindner A, Ludolph AC, Kimmig H, Jander S, Schlegel U, Gawaz M, Ziemann U. Apixaban for treatment of embolic stroke of undetermined source (ATTICUS randomized trial): Rationale and study design. Int J Stroke 2017;12(9):985-990
• Gorges M, Maier MN, Rosskopf J, Vintonyak O, Pinkhardt EH, Ludolph AC, Müller HP, Kassubek J. Regional microstructural damage and patterns of eye movement impairment: a DTI and video-oculography study in neurodegenerative parkinsonian syndromes. J Neurol 2017;264(9):1919-1928
• Gorges M, Roselli F, Müller HP, Ludolph AC, Rasche V, Kassubek J. Functional Connectivity Mapping in the Animal Model: Principles and Applications of Resting-State fMRI. Front Neurol 2017;8:200
• Gorges M, Vercruysse P, Müller HP, Huppertz HJ, Rosenbohm A, Nagel G, Weydt P, Petersén Å, Ludolph AC, Kassubek J, Dupuis L. Hypothalamic atrophy is related to body mass index and age at onset in amyotrophic lateral sclerosis. J Neurol Neurosurg Psych 2017;88(12):1033-1041
• Jesse S, Bayer H, Alupei MC, Zügel M, Mulaw M, Tuorto F, Malmsheimer S, Singh K, Steinacker J, Schumann U, Ludolph AC, Scharffetter-Kochanek K, Witting A, Weydt P, Iben S. Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington's disease. Sci Rep 2017;7(1):8513
• Jesse S, Bråthen G, Ferrara M, Keindl M, Ben-Menachem E, Tanasescu R, Brodtkorb E, Hillbom M, Leone MA, Ludolph AC. Alcohol withdrawal syndrome: mechanisms, manifestations, and management. Acta Neurol Scand 2017;135(1):4-16
• Kassubek R, Bullinger L, Kassubek J, Dreyhaupt J, Ludolph AC, Althaus K, Lewerenz J. Identifying ischemic stroke associated with cancer: a multiple model derived from a case-control analysis. J Neurol 2017;264(4):781-791
• Kassubek R, Gorges M, Schocke M, Hagenston VAM, Huss A, Ludolph AC, Kassubek J, Tumani H. GFAP in early multiple sclerosis: A biomarker for inflammation. Neurosci Lett 2017;657:166-170
• Kassubek R, Gorges M, Westhoff MA, Ludolph AC, Kassubek J, Müller HP. Cerebral Microstructural Alterations after Radiation Therapy in High-Grade Glioma: A Diffusion Tensor Imaging-Based Study. Front Neurol 2017;8:286
• Keller J, Krimly A, Bauer L, Schulenburg S, Böhm S, Aho-Özhan HEA, Uttner I, Gorges M, Kassubek J, Pinkhardt EH, Abrahams S, Ludolph AC, Lulé D. A first approach to a neuropsychological screening tool using eye-tracking for bedside cognitive testing based on the Edinburgh Cognitive and Behavioural ALS Screen. Amyotroph Lateral Scler Frontotemporal Degener 2017;18(5-6):443-450
• Lange DJ, Shahbazi M, Silani V, Ludolph AC, Weishaupt JH, Ajroud-Driss S, Fields KG, Remanan R, Appel SH, Morelli C, Doretti A, Maderna L, Messina S, Weiland U, Marklund SL, Andersen PM. Pyrimethamine significantly lowers cerebrospinal fluid Cu/Zn superoxide dismutase in amyotrophic lateral sclerosis patients with SOD1 mutations. Ann Neurol 2017;81(6):837-848
• Lehmer C, Oeckl P, Weishaupt JH, Volk AE, Diehl-Schmid J, Schroeter ML, Lauer M, Kornhuber J, Levin J, Fassbender K, Landwehrmeyer B; German Consortium for Frontotemporal Lobar Degeneration, Schludi MH, Arzberger T, Kremmer E, Flatley A, Feederle R, Steinacker P, Weydt P, Ludolph AC, Edbauer D, Otto M. Poly-GP in cerebrospinal fluid links C9orf72-associated dipeptide repeat expression to the asymptomatic phase of ALS/FTD. EMBO Mol Med 2017;9(7):859-868
• Lindner-Pfleghar B, Neugebauer H, Stösser S, Kassubek J, Ludolph AC, Dziewas R, Prosiegel M, Riecker A. Dysphagiemanagement beim akuten Schlaganfall. Eine prospektive Studie zur Überprüfung der geltenden Empfehlungen. Nervenarzt 2017;88(2):173-179
• Ludolph AC. Edaravone: a baby in the bathwater? Ther Adv Neurol Disord 2017;10(9):313-314
• Ludolph AC. Motor neuron disease: Genetic testing in amyotrophic lateral sclerosis. Nat Rev Neurol 2017;13(5):262-263
• Meininger V, Genge A, van den Berg LH, Robberecht W, Ludolph AC, Chio A, Kim SH, Leigh PN, Kiernan MC, Shefner JM, Desnuelle C, Morrison KE, Petri S, Boswell D, Temple J, Mohindra R, Davies M, Bullman J, Rees P, Lavrov A; NOG112264 Study Group. Safety and efficacy of ozanezumab in patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled, phase 2 trial. Lancet Neurol 2017;16(3):208-216
• Meyer S, Mueller K, Stuke K, Bisenius S, Diehl-Schmid J, Jessen F, Kassubek J, Kornhuber J, Ludolph AC, Prudlo J, Schneider A, Schuemberg K, Yakushev I, Otto M, Schroeter ML; FTLDc Study Group. Predicting behavioral variant frontotemporal dementia with pattern classification in multi-center structural MRI data. Neuroimage Clin 2017;14:656-662
• Mueller K, Jech R, Bonnet C, Tintěra J, Hanuška J, Möller HE, Fassbender K, Ludolph AC, Kassubek J, Otto M, Růžička E, Schroeter ML; FTLDc Study Group. Disease-Specific Regions Outperform Whole-Brain Approaches in Identifying Progressive Supranuclear Palsy: A Multicentric MRI Study. Front Neurosci 2017;11:100
• Nagel G, Peter RS, Rosenbohm A, Koenig W, Dupuis L, Rothenbacher D, Ludolph AC. Adipokines, C-reactive protein and Amyotrophic Lateral Sclerosis - results from a population- based ALS registry in Germany. Sci Rep 2017;7(1):4374
• Nordin A, Akimoto C, Wuolikainen A, Alstermark H, Forsberg K, Baumann P, Pinto S, de Carvalho M, Hübers A, Nordin F, Ludolph AC, Weishaupt JH, Meyer T, Grehl T, Schweikert K, Weber M, Burkhardt C, Neuwirth C, Holmøy T, Morita M, Tysnes OB, Benatar M, Wuu J, Lange DJ, Bisgård C, Asgari N, Tarvainen I, Brännström T, Andersen PM. Sequence variations in C9orf72 downstream of the hexanucleotide repeat region and its effect on repeat-primed PCR interpretation: a large multinational screening study. Amyotroph Lateral Scler Frontotemporal Degener 2017;18(3-4):256-264
• Peter RS, Rosenbohm A, Dupuis L, Brehme T, Kassubek J, Rothenbacher D, Nagel G, Ludolph AC. Life course body mass index and risk and prognosis of amyotrophic lateral sclerosis: results from the ALS registry Swabia. Eur J Epidemiol 2017;32(10):901-908
• Poletti B, Carelli L, Solca F, Lafronza A, Pedroli E, Faini A, Ticozzi N, Ciammola A, Meriggi P, Cipresso P, Lulé D, Ludolph AC, Riva G, Silani V. An eye-tracker controlled cognitive battery: overcoming verbal-motor limitations in ALS. J Neurol 2017;264(6):1136-1145
• Poletti B, Carelli L, Solca F, Lafronza A, Pedroli E, Faini A, Zago S, Ticozzi N, Ciammola A, Morelli C, Meriggi P, Cipresso P, Lulé D, Ludolph AC, Riva G, Silani V. An eye-tracking controlled neuropsychological battery for cognitive assessment in neurological diseases. Neurol Sci 2017;38(4):595-603
• Rosenbohm A, Peter RS, Erhardt S, Lulé D, Rothenbacher D, Ludolph AC, Nagel G; ALS Registry Study Group. Epidemiology of amyotrophic lateral sclerosis in Southern Germany. J Neurol 2017;264(4):749-757
• Rosenbohm A, Schmid B, Buckert D, Rottbauer W, Kassubek J, Ludolph AC, Bernhardt P. Cardiac Findings in Amyotrophic Lateral Sclerosis: A Magnetic Resonance Imaging Study. Front Neurol 2017;8:479
• Rosskopf J, Gorges M, Müller HP, Lulé D, Uttner I, Ludolph AC, Pinkhardt E, Juengling FD, Kassubek J. Intrinsic functional connectivity alterations in progressive supranuclear palsy: Differential effects in frontal cortex, motor, and midbrain networks. Mov Disord 2017;32(7):1006-1015
• Scekic-Zahirovic J, Oussini HE, Mersmann S, Drenner K, Wagner M, Sun Y, Allmeroth K, Dieterlé S, Sinniger J, Dirrig-Grosch S, René F, Dormann D, Haass C, Ludolph AC, Lagier-Tourenne C, Storkebaum E, Dupuis L. Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis. Acta Neuropathol 2017;133(6):887-906
• Steinacker P, Huss A, Mayer B, Grehl T, Grosskreutz J, Borck G, Kuhle J, Lulé D, Meyer T, Oeckl P, Petri S, Weishaupt J, Ludolph AC, Otto M. Diagnostic and prognostic significance of neurofilament light chain NF-L, but not progranulin and S100B, in the course of amyotrophic lateral sclerosis: Data from the German MND-net. Amyotroph Lateral Scler Frontotemporal Degener 2017;18(1-2):112-119
• Steinacker P, Semler E, Anderl-Straub S, Diehl-Schmid J, Schroeter ML, Uttner I, Foerstl H, Landwehrmeyer B, von Arnim CA, Kassubek J, Oeckl P, Huppertz HJ, Fassbender K, Fliessbach K, Prudlo J, Roßmeier C, Kornhuber J, Schneider A, Volk AE, Lauer M, Danek A, Ludolph AC, Otto M; FTLDc Study Group. Neurofilament as a blood marker for diagnosis and monitoring of primary progressive aphasias. Neurology 2017;88(10):961-969
• Synofzik M, Otto M, Ludolph AC, Weishaupt JH. Genetic architecture of amyotrophic lateral sclerosis and frontotemporal dementia: Overlap and differences. Nervenarzt 2017;88 (7):728-735
• Tsoures E, Lewerenz J, Pinkhardt E, Ludolph AC, Fauser S. Electroencephalographic findings in patients with circumscribed thalamic lesions. Epilepsy Res 2017;135: 115-122
• Türk M, Schröder R, Khuller K, Hofmann A, Berwanger C, Ludolph AC, Dekomien G, Müller K, Weishaupt JH, Thiel CT, Clemen CS. Genetic analysis of VCP and WASH complex genes in a German cohort of sporadic ALS-FTD patients. Neurobiol Aging 2017;56:213.e1-213.e5
• van der Burg JMM, Gardiner SL, Ludolph AC, Landwehrmeyer GB, Roos RAC, Aziz NA. Body weight is a robust predictor of clinical progression in Huntington disease. Ann Neurol 2017;82(3):479-483
• van Doormaal PT, Ticozzi N, Weishaupt JH, Kenna K, Diekstra FP, Verde F, Andersen PM, Dekker AM, Tiloca C, Marroquin N, Overste DJ, Pensato V, Nürnberg P, Pulit SL, Schellevis RD, Calini D, Altmüller J, Francioli LC, Muller B, Castellotti B, Motameny S, Ratti A, Wolf J, Gellera C, Ludolph AC, van den Berg LH, Kubisch C, Landers JE, Veldink JH, Silani V, Volk AE. The role of de novo mutations in the development of amyotrophic lateral sclerosis. Hum Mutat 2017;38(11): 1534-1541
• Vintonyak O, Gorges M, Müller HP, Pinkhardt EH, Ludolph AC, Huppertz HJ, Kassubek J. Patterns of Eye Movement Impairment Correlate with Regional Brain Atrophy in Neurodegenerative Parkinsonism. Neurodegener Dis 2017;17(4-5):117-126
• von Einem B, Eschbach J, Kiechle M, Wahler A, Thal DR, McLean PJ, Weishaupt JH, Ludolph AC, von Arnim CAF, Danzer KM. The Golgi-localized, gamma ear-containing, ARF-binding (GGA) protein family alters alpha synuclein (α-syn) oligomerization and secretion. Aging (Albany NY) 2017;9(7):1677-1697
• Wais V, Rosenbohm A, Petri S, Kollewe K, Hermann A, Storch A, Hanisch F, Zierz S, Nagel G, Kassubek J, Weydt P, Brettschneider J, Weishaupt JH, Ludolph AC, Dorst J. The concept and diagnostic criteria of primary lateral sclerosis. Acta Neurol Scand 2017;136(3):204-211
• Weber C, Fijalkowska B, Ciecwierska K, Lindblad A, Badura-Lotter G, Andersen PM, Kuźma-Kozakiewicz M, Ludolph AC, Lulé D, Pasierski T, Lynöe N. Existential decision-making in a fatal progressive disease: how much do legal and medical frameworks matter? BMC Palliat Care 2017;16(1):80
• Zondler L, Feiler MS, Freischmidt A, Ruf WP, Ludolph AC, Danzer KM, Weishaupt JH. Impaired activation of ALS monocytes by exosomes. Immunol Cell Biol 2017;95(2):207-214

• Aho-Özhan HE, Keller J, Heimrath J, Uttner I, Kassubek J, Birbaumer N, Ludolph AC, Lulé D. Perception of Emotional Facial Expressions in Amyotrophic Lateral Sclerosis (ALS) at Behavioural and Brain Metabolic Level. PLoS One 2016;11(10):e0164655
• Auer-Grumbach M, Toegel S, Schabhüttl M, Weinmann D, Chiari C, Bennett DL, Beetz C, Klein D, Andersen PM, Böhme I, Fink-Puches R, Gonzalez M, Harms MB, Motley W, Reilly MM, Renner W, Rudnik-Schöneborn S, Schlotter-Weigel B, Themistocleous AC, Weishaupt JH, Ludolph AC, Wieland T, Tao F, Abreu L, Windhager R, Zitzelsberger M, Strom TM, Walther T, Scherer SS, Züchner S, Martini R, Senderek J. Rare Variants in MME, Encoding Metalloprotease Neprilysin, Are Linked to Late-Onset Autosomal-Dominant Axonal Polyneuropathies. Am J Hum Genet 2016;99(3):607-23
• Bernhardt D, Müller HP, Ludolph AC, Dupuis L, Kassubek J. Body fat distribution in Parkinson's disease: An MRI-based body fat quantification study. Parkinsonism Relat Disord 2016;33:84-89
• Bliederhaeuser C, Grozdanov V, Speidel A, Zondler L, Ruf WP, Bayer H, Kiechle M, Feiler MS, Freischmidt A, Brenner D, Witting A, Hengerer B, Fändrich M, Ludolph AC, Weishaupt JH, Gillardon F, Danzer KM. Age-dependent defects of alpha-synuclein oligomer uptake in microglia and monocytes. Acta Neuropathol 2016;131(3):379-91
• Bliederhaeuser C, Zondler L, Grozdanov V, Ruf WP, Brenner D, Melrose HL, Bauer P, Ludolph AC, Gillardon F, Kassubek J, Weishaupt JH, Danzer KM. LRRK2 contributes to monocyte dysregulation in Parkinson's disease. Acta Neuropathol Commun 2016;4(1):123
• Böhm S, Aho-Özhan HE, Keller J, Dorst J, Uttner I, Ludolph AC, Lulé D. Medical decisions are independent of cognitive impairment in amyotrophic lateral sclerosis. Neurology 2016;87(16):1737-1738
• Brenner D, Andersen PM, Ludolph AC, Weishaupt JH. Comment on "Cutting Edge: Inhibiting TBK1 by Compound II Ameliorates Autoimmune Disease in Mice". J Immunol 2016;196(2):530-1
• Brenner D, Müller K, Wieland T, Weydt P, Böhm S, Lulé D, Hübers A, Neuwirth C, Weber M, Borck G, Wahlqvist M, Danzer KM, Volk AE, Meitinger T, Strom TM, Otto M, Kassubek J, Ludolph AC, Andersen PM, Weishaupt JH. NEK1 mutations in familial amyotrophic lateral sclerosis. Brain 2016;139(Pt 5):e28
• El Oussini H, Bayer H, Scekic-Zahirovic J, Vercruysse P, Sinniger J, Dirrig-Grosch S, Dieterlé S, Echaniz-Laguna A, Larmet Y, Müller K, Weishaupt JH, Thal DR, van Rheenen W, van Eijk K, Lawson R, Monassier L, Maroteaux L, Roumier A, Wong PC, van den Berg LH, Ludolph AC, Veldink JH, Witting A, Dupuis L. Serotonin 2B receptor slows disease progression and prevents degeneration of spinal cord mononuclear phagocytes in amyotrophic lateral sclerosis. Acta Neuropathol 2016;131(3):465-80
• Feneberg E, Steinacker P, Volk AE, Weishaupt JH, Wollmer MA, Boxer A, Tumani H, Ludolph AC, Otto M. Progranulin as a candidate biomarker for therapeutic trial in patients with ALS and FTLD. J Neural Transm (Vienna) 2016;123(3):289-96
• Gorges M, Müller HP, Lulé D; LANDSCAPE Consortium, Pinkhardt EH, Ludolph AC, Kassubek J. The association between alterations of eye movement control and cerebral intrinsic functional connectivity in Parkinson's disease. Brain Imaging Behav 2016 Mar;10(1):79-91
• Halbgebauer S, Nagl M, Klafki H, Haußmann U, Steinacker P, Oeckl P, Kassubek J, Pinkhardt E, Ludolph AC, Soininen H, Herukka SK, Wiltfang J, Otto M. Modified serpinA1 as risk marker for Parkinson's disease dementia: Analysis of baseline data. Sci Rep 2016;6:26145
• Hübers A, Hildebrandt V, Petri S, Kollewe K, Hermann A, Storch A, Hanisch F, Zierz S, Rosenbohm A, Ludolph AC, Dorst J. Clinical features and differential diagnosis of flail arm syndrome. J Neurol 2016;263(2):390-5
• Hübers A, Kassubek J, Grön G, Gorges M, Aho-Oezhan H, Keller J, Horn H, Neugebauer H, Uttner I, Lulé D, Ludolph AC. Pathological laughing and crying in amyotrophic lateral sclerosis is related to frontal cortex function. J Neurol 2016;263(9):1788-95
• Hübers A, Ludolph AC, Rosenbohm A, Pinkhardt EH, Weishaupt JH, Dorst J. [Amyotrophic lateral sclerosis. Multisystem degeneration]. Nervenarzt 2016;87(2):179-88
• Hübers A, Müller HP, Dreyhaupt J, Böhm K, Lauda F, Tumani H, Kassubek J, Ludolph AC, Pinkhardt EH. Retinal involvement in amyotrophic lateral sclerosis: a study with optical coherence tomography and diffusion tensor imaging. J Neural Transm (Vienna) 2016;123(3):281-7
• Keller J, Gorges M, Aho-Özhan HE, Uttner I, Schneider E, Kassubek J, Pinkhardt EH, Ludolph AC, Lulé D. Eye-Tracking Control to Assess Cognitive Functions in Patients with Amyotrophic Lateral Sclerosis. J Vis Exp 2016 Oct 13;(116)
• Kenna KP, van Doormaal PT, Dekker AM, Ticozzi N, Kenna BJ, Diekstra FP, van Rheenen W, van Eijk KR, Jones AR, Keagle P, Shatunov A, Sproviero W, Smith BN, van Es MA, Topp SD, Kenna A, Miller JW, Fallini C, Tiloca C, McLaughlin RL, Vance C, Troakes C, Colombrita C, Mora G, Calvo A, Verde F, Al-Sarraj S, King A, Calini D, de Belleroche J, Baas F, van der Kooi AJ, de Visser M, Ten Asbroek AL, Sapp PC, McKenna-Yasek D, Polak M, Asress S, Muñoz-Blanco JL, Strom TM, Meitinger T, Morrison KE; SLAGEN Consortium, Lauria G, Williams KL, Leigh PN, Nicholson GA, Blair IP, Leblond CS, Dion PA, Rouleau GA, Pall H, Shaw PJ, Turner MR, Talbot K, Taroni F, Boylan KB, Van Blitterswijk M, Rademakers R, Esteban-Pérez J, García-Redondo A, Van Damme P, Robberecht W, Chio A, Gellera C, Drepper C, Sendtner M, Ratti A, Glass JD, Mora JS, Basak NA, Hardiman O, Ludolph AC, Andersen PM, Weishaupt JH, Brown RH Jr, Al-Chalabi A, Silani V, Shaw CE, van den Berg LH, Veldink JH, Landers JE. NEK1 variants confer susceptibility to amyotrophic lateral sclerosis. Nat Genet 2016;48(9):1037-42
• Lindemann K, Müller HP, Ludolph AC, Hornyak M, Kassubek J. Microstructure of the Midbrain and Cervical Spinal Cord in Idiopathic Restless Legs Syndrome: A Diffusion Tensor Imaging Study. Sleep 2016;39(2):423-8
• Linkus B, Wiesner D, Meßner M, Karabatsiakis A, Scheffold A, Rudolph KL, Thal DR, Weishaupt JH, Ludolph AC, Danzer KM. Telomere shortening leads to earlier age of onset in ALS mice. Aging (Albany NY) 2016;8(2):382-93
• Loose M, Burkhardt C, Aho-Özhan H, Keller J, Abdulla S, Böhm S, Kollewe K, Uttner I, Abrahams S, Petri S, Weber M, Ludolph AC, Lulé D. Age and education-matched cut-off scores for the revised German/Swiss-German version of ECAS. Amyotroph Lateral Scler Frontotemporal Degener 2016;17(5-6):374-6
• Ludolph AC. The TUDCA trial-innovative trial designs for amyotrophic lateral sclerosis drugs? Eur J Neurol 2016;23(1):11-2
• Marroquin N, Stranz S, Müller K, Wieland T, Ruf WP, Brockmann SJ, Danzer KM, Borck G, Hübers A, Weydt P, Meitinger T, Strom TM, Rosenbohm A, Ludolph AC, Weishaupt JH. Screening for CHCHD10 mutations in a large cohort of sporadic ALS patients: no evidence for pathogenicity of the p.P34S variant. Brain 2016;139(Pt 2):e8
• Müller HP, Turner MR, Grosskreutz J, Abrahams S, Bede P, Govind V, Prudlo J, Ludolph AC, Filippi M, Kassubek J; Neuroimaging Society in ALS (NiSALS) DTI Study Group. A large-scale multicentre cerebral diffusion tensor imaging study in amyotrophic lateral sclerosis. J Neurol Neurosurg Psych 2016;87(6):570-9
• Naujock M, Stanslowsky N, Bufler S, Naumann M, Reinhardt P, Sterneckert J, Kefalakes E, Kassebaum C, Bursch F, Lojewski X, Storch A, Frickenhaus M, Boeckers TM, Putz S, Demestre M, Liebau S, Klingenstein M, Ludolph AC, Dengler R, Kim KS, Hermann A, Wegner F, Petri S. 4-Aminopyridine Induced Activity Rescues Hypoexcitable Motor Neurons from Amyotrophic Lateral Sclerosis Patient-Derived Induced Pluripotent Stem Cells.  Stem Cells 2016;34(6):1563-75
• Neugebauer H, Lindner-Pfleghar B, Jüttler E, Ludolph AC, Riecker A. Prophylactic antibiotics to reduce pneumonia after acute stroke. Lancet 2016;387(10017):432-3
• Oeckl P, Metzger F, Nagl M, von Arnim CA, Halbgebauer S, Steinacker P, Ludolph AC, Otto M. Alpha-, Beta-, and Gamma-synuclein Quantification in Cerebrospinal Fluid by Multiple Reaction Monitoring Reveals Increased Concentrations in Alzheimer's and Creutzfeldt-Jakob Disease but No Alteration in Synucleinopathies. Mol Cell Proteomics 2016;15(10):3126-3138
• Pareyson D, Fratta P, Pradat PF, Sorarù G, Finsterer J, Vissing J, Jokela ME, Udd B, Ludolph AC, Sagnelli A, Weydt P. Towards a European Registry and Biorepository for Patients with Spinal and Bulbar Muscular Atrophy. J Mol Neurosci 2016;58(3):394-400
• Poletti B, Carelli L, Solca F, Lafronza A, Pedroli E, Faini A, Zago S, Ticozzi N, Meriggi P, Cipresso P, Lulé D, Ludolph AC, Riva G, Silani V. Cognitive assessment in Amyotrophic Lateral Sclerosis by means of P300-Brain Computer Interface: a preliminary study. Amyotroph Lateral Scler Frontotemp Degener 2016;17(7-8):473-481
• Rennebaum F, Kassubek J, Pinkhardt E, Hübers A, Ludolph AC, Schocke M, Fauser S. Status epilepticus: Clinical characteristics and EEG patterns associated with and without MRI diffusion restriction in 69 patients. Epilepsy Res 2016;120:55-64
• Rosenbohm A, Müller HP, Hübers A, Ludolph AC, Kassubek J. Corticoefferent pathways in pure lower motor neuron disease: a diffusion tensor imaging study. J Neurol 2016;263(12):2430-2437
• Scekic-Zahirovic J, Sendscheid O, El Oussini H, Jambeau M, Sun Y, Mersmann S, Wagner M, Dieterlé S, Sinniger J, Dirrig-Grosch S, Drenner K, Birling MC, Qiu J, Zhou Y, Li H, Fu XD, Rouaux C, Shelkovnikova T, Witting A, Ludolph AC, Kiefer F, Storkebaum E, Lagier-Tourenne C, Dupuis L. Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss. EMBO J 2016;35(10):1077-97
• Schoen M, Reichel JM, Demestre M, Putz S, Deshpande D, Proepper C, Liebau S, Schmeisser MJ, Ludolph AC, Michaelis J, Boeckers TM. Super-Resolution Microscopy Reveals Presynaptic Localization of the ALS/FTD Related Protein FUS in Hippocampal Neurons. Front Cell Neurosci 2016;9:496
• Schöpper M, Ludolph AC, Fauser S. Dental care in patients with epilepsy: a survey of 82 patients and their attending dentists and neurologists in southern Germany. Int Dent J 2016;66(6):366-374
• Schulthess I, Gorges M, Müller HP, Lulé D, Del Tredici K, Ludolph AC, Kassubek J. Functional connectivity changes resemble patterns of pTDP-43 pathology in amyotrophic lateral sclerosis. Sci Rep 2016;6:38391
• Schwenk BM, Hartmann H, Serdaroglu A, Schludi MH, Hornburg D, Meissner F, Orozco D, Colombo A, Tahirovic S, Michaelsen M, Schreiber F, Haupt S, Peitz M, Brüstle O, Küpper C, Klopstock T, Otto M, Ludolph AC, Arzberger T, Kuhn PH, Edbauer D. TDP-43 loss of function inhibits endosomal trafficking and alters trophic signaling in neurons. EMBO J 2016;35(21):2350-2370
• Steinacker P, Feneberg E, Weishaupt J, Brettschneider J, Tumani H, Andersen PM, von Arnim CA, Böhm S, Kassubek J, Kubisch C, Lulé D, Müller HP, Muche R, Pinkhardt E, Oeckl P, Rosenbohm A, Anderl-Straub S, Volk AE, Weydt P, Ludolph AC, Otto M. Neurofilaments in the diagnosis of motoneuron diseases: a prospective study on 455 patients. J Neurol Neurosurg Psych 2016;87(1):12-20
• Stösser S, Neugebauer H, Althaus K, Ludolph AC, Kassubek J, Schocke M. Perihematomal Diffusion Restriction in Intracerebral Hemorrhage Depends on Hematoma Volume, But Does Not Predict Outcome. Cerebrovasc Dis 2016;42(3-4):280-7
• Teipel S, Raiser T, Riedl L, Riederer I, Schroeter ML, Bisenius S, Schneider A, Kornhuber J, Fliessbach K, Spottke A, Grothe MJ, Prudlo J, Kassubek J, Ludolph AC, Landwehrmeyer B, Straub S, Otto M, Danek A; FTLDc study group. Atrophy and structural covariance of the cholinergic basal forebrain in primary progressive aphasia. Cortex 2016;83:124-35
• van Rheenen W, Shatunov A, Dekker AM, McLaughlin RL, Diekstra FP, Pulit SL, van der Spek RA, Võsa U, de Jong S, Robinson MR, Yang J, Fogh I, van Doormaal PT, Tazelaar GH, Koppers M, Blokhuis AM, Sproviero W, Jones AR, Kenna KP, van Eijk KR, Harschnitz O, Schellevis RD, Brands WJ, Medic J, Menelaou A, Vajda A, Ticozzi N, Lin K, Rogelj B, Vrabec K, Ravnik-Glavač M, Koritnik B, Zidar J, Leonardis L, Grošelj LD, Millecamps S, Salachas F, Meininger V, de Carvalho M, Pinto S, Mora JS, Rojas-García R, Polak M, Chandran S, Colville S, Swingler R, Morrison KE, Shaw PJ, Hardy J, Orrell RW, Pittman A, Sidle K, Fratta P, Malaspina A, Topp S, Petri S, Abdulla S, Drepper C, Sendtner M, Meyer T, Ophoff RA, Staats KA, Wiedau-Pazos M, Lomen-Hoerth C, Van Deerlin VM, Trojanowski JQ, Elman L, McCluskey L, Basak AN, Tunca C, Hamzeiy H, Parman Y, Meitinger T, Lichtner P, Radivojkov-Blagojevic M, Andres CR, Maurel C, Bensimon G, Landwehrmeyer B, Brice A, Payan CA, Saker-Delye S, Dürr A, Wood NW, Tittmann L, Lieb W, Franke A, Rietl M, Cichon S, Nöthen MM, Amouyel P, Tzourio C, Dartigues JF, Uitterlinden AG, Rivadeneira F, Estrada K, Hofman A, Curtis C, Blauw HM, van der Kooi AJ, de Visser M, Goris A, Weber M, Shaw CE, Smith BN, Pansarasa O, Cereda C, Del Bo R, Comi GP, D'Alfonso S, Bertolin C, Sorarù G, Mazzini L, Pensato V, Gellera C, Tiloca C, Ratti A, Calvo A, Moglia C, Brunetti M, Arcuti S, Capozzo R, Zecca C, Lunetta C, Penco S, Riva N, Padovani A, Filosto M, Muller B, Stuit RJ; PARALS Registry; SLALOM Group; SLAP Registry; FALS Sequencing Consortium; SLAGEN Consortium; NNIPPS Study Group, Blair I, Zhang K, McCann EP, Fifita JA, Nicholson GA, Rowe DB, Pamphlett R, Kiernan MC, Grosskreutz J, Witte OW, Ringer T, Prell T, Stubendorff B, Kurth I, Hübner CA, Leigh PN, Casale F, Chio A, Beghi E, Pupillo E, Tortelli R, Logroscino G, Powell J, Ludolph AC, Weishaupt JH, Robberecht W, Van Damme P, Franke L, Pers TH, Brown RH, Glass JD, Landers JE, Hardiman O, Andersen PM, Corcia P, Vourc'h P, Silani V, Wray NR, Visscher PM, de Bakker PI, van Es MA, Pasterkamp RJ, Lewis CM, Breen G, Al-Chalabi A, van den Berg LH, Veldink JH. Genome-wide association analyses identify new risk variants and the genetic architecture of amyotrophic lateral sclerosis. Nat Genet 2016;48(9):1043-8
• Vercruysse P, Sinniger J, El Oussini H, Scekic-Zahirovic J, Dieterlé S, Dengler R, Meyer T, Zierz S, Kassubek J, Fischer W, Dreyhaupt J, Grehl T, Hermann A, Grosskreutz J, Witting A, Van Den Bosch L, Spreux-Varoquaux O; GERP ALS Study Group, Ludolph AC, Dupuis L. Alterations in the hypothalamic melanocortin pathway in amyotrophic lateral sclerosis.  Brain 2016;139(Pt 4):1106-22
• Weydt P, Oeckl P, Huss A, Müller K, Volk AE, Kuhle J, Knehr A, Andersen PM, Prudlo J, Steinacker P, Weishaupt JH, Ludolph AC, Otto M. Neurofilament levels as biomarkers in asymptomatic and symptomatic familial amyotrophic lateral sclerosis. Ann Neurol 2016;79(1):152-8
• Weydt P, Sagnelli A, Rosenbohm A, Fratta P, Pradat PF, Ludolph AC, Pareyson D. Clinical Trials in Spinal and Bulbar Muscular Atrophy-Past, Present, and Future. J Mol Neurosci 2016;58(3):379-87
• Zondler L, Müller K, Khalaji S, Bliederhäuser C, Ruf WP, Grozdanov V, Thiemann M, Fundel-Clemes K, Freischmidt A, Holzmann K, Strobel B, Weydt P, Witting A, Thal DR, Helferich AM, Hengerer B, Gottschalk KE, Hill O, Kluge M, Ludolph AC, Danzer KM, Weishaupt JH.  Peripheral monocytes are functionally altered and invade the CNS in ALS patients. Acta Neuropathol 2016;132(3):391-411

• Abdelhak A, Junker A, Brettschneider J, Kassubek J, Ludolph AC, Otto M, Tumani H. Brain-Specific Cytoskeletal Damage Markers in Cerebrospinal Fluid: Is There a Common Pattern between Amyotrophic Lateral Sclerosis and Primary Progressive Multiple Sclerosis? Int J Mol Sci 2015;16(8):17565-88
• Agosta F, Al-Chalabi A, Filippi M, Hardiman O, Kaji R, Meininger V, Nakano I, Shaw P, Shefner J, van den Berg LH, Ludolph AC; WFN Research Group on ALS/MND. The El Escorial criteria: Strengths and weaknesses. Amyotroph Lateral Scler Frontotemporal Degener 2015;16(1-2):1-7
• Brettschneider J, Del Tredici K, Irwin DJ, Grossman M, Robinson JL, Toledo JB, Lee EB, Fang L, Van Deerlin VM, Ludolph AC, Lee VM, Braak H, Trojanowski JQ. Erratum to: Sequential distribution of pTDP-43 pathology in behavioral variant frontotemporal dementia (bvFTD). Acta Neuropathol 2015;129(6):929
• Demestre M, Orth M, Föhr KJ, Achberger K, Ludolph AC, Liebau S, Boeckers TM. Formation and characterisation of neuromuscular junctions between hiPSC derived motoneurons and myotubes. Stem Cell Res 2015;15(2):328-336
• Dorst J, Dupuis L, Petri S, Kollewe K, Abdulla S, Wolf J, Weber M, Czell D, Burkhardt C, Hanisch F, Vielhaber S, Meyer T, Frisch G, Kettemann D, Grehl T, Schrank B, Ludolph AC. Percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis: a prospective observational study. J Neurol 2015;262(4):849-58
• Eschbach J, von Einem B, Müller K, Bayer H, Scheffold A, Morrison BE, Rudolph KL, Thal DR, Witting A, Weydt P, Otto M, Fauler M, Liss B, McLean PJ, Spada AR, Ludolph AC, Weishaupt JH, Danzer KM. Mutual exacerbation of peroxisome proliferator-activated receptor γ coactivator 1α deregulation and α-synuclein oligomerization. Ann Neurol 2015;77(1):15-32
• Freischmidt A, Wieland T, Richter B, Ruf W, Schaeffer V, Müller K, Marroquin N, Nordin F, Hübers A, Weydt P, Pinto S, Press R, Millecamps S, Molko N, Bernard E, Desnuelle C, Soriani MH, Dorst J, Graf E, Nordström U, Feiler MS, Putz S, Boeckers TM, Meyer T, Winkler AS, Winkelman J, de Carvalho M, Thal DR, Otto M, Brännström T, Volk AE, Kursula P, Danzer KM, Lichtner P, Dikic I, Meitinger T, Ludolph AC, Strom TM, Andersen PM, Weishaupt JH. Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia. Nat Neurosci 2015;18(5):631-6
• Freischmidt A, Müller K, Zondler L, Weydt P, Mayer B, von Arnim CA, Hübers A, Dorst J, Otto M, Holzmann K, Ludolph AC, Danzer KM, Weishaupt JH. Serum microRNAs in sporadic amyotrophic lateral sclerosis. Neurobiol Aging 2015;36(9):2660.e15-20
• Gorges M, Müller HP, Lulé D; LANDSCAPE Consortium, Pinkhardt EH, Ludolph AC, Kassubek J. To rise and to fall: functional connectivity in cognitively normal and cognitively impaired patients with Parkinson's disease. Neurobiol Aging 2015;36:1727-1735
• Hermann A, Reuner U, Schaefer J, Fathinia P, Leimert T, Kassubek J, Leimert M, Ludolph AC, Storch A. The diagnostic value of midbrain hyperechogenicity in ALS is limited for discriminating key ALS differential diagnoses. BMC Neurol 2015;15:33
• Japtok J, Lojewksi X, Naumann M, Klingenstein M, Reinhardt P, Sterneckert J, Putz S, Demestre M, Boeckers TM, Ludolph AC, Liebau S, Storch A, Hermann A. Stepwise acquirement of hallmark neuropathology in FUS-ALS iPSC models depends on mutation type and neuronal aging. Neurobiol Dis 2015;82:420-429
• Keller J, Gorges M, Horn HT, Aho-Özhan HE, Pinkhardt EH, Uttner I, Kassubek J, Ludolph AC, Lulé D. Eye-tracking controlled cognitive function tests in patients with amyotrophic lateral sclerosis: a controlled proof-of-principle study. J Neurol 2015;262(8):1918-26
• Kunadt M, Eckermann K, Stuendl A, Gong J, Russo B, Strauss K, Rai S, Kügler S, Falomir Lockhart L, Schwalbe M, Krumova P, Oliveira LM, Bähr M, Möbius W, Levin J, Giese A, Kruse N, Mollenhauer B, Geiss-Friedlander R, Ludolph AC, Freischmidt A, Feiler MS, Danzer KM, Zweckstetter M, Jovin TM, Simons M, Weishaupt JH, Schneider A. Extracellular vesicle sorting of α-Synuclein is regulated by sumoylation. Acta Neuropathol 2015;129(5):695-713
• Ludolph AC, Brettschneider J. TDP-43 in amyotrophic lateral sclerosis - is it a prion disease? Eur J Neurol 2015;22(5):753-61
• Lulé D, Burkhardt C, Abdulla S, Böhm S, Kollewe K, Uttner I, Abrahams S, Bak TH, Petri S, Weber M, Ludolph AC. The Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen: A cross-sectional comparison of established screening tools in a German-Swiss population. Amyotroph Lateral Scler Frontotemporal Degener 2015;16(1-2):16-23
• Nagel G, Herbolsheimer F, Riepe M, Nikolaus T, Denkinger MD, Peter R, Weinmayr G, Rothenbacher D, Koenig W, Ludolph AC, von Arnim CA; ActiFE Study group. Serum Vitamin D Concentrations and Cognitive Function in a Population-Based Study among Older Adults in South Germany. J Alzheimers Dis 2015;45(4):1119-26
• Rosenbohm A, Buckert D, Gerischer N, Walcher T, Kassubek J, Rottbauer W, Ludolph AC, Bernhardt P. Early diagnosis of cardiac involvement in idiopathic inflammatory myopathy by cardiac magnetic resonance tomography. J Neurol 2015;262(4):949-56
• Rosskopf J, Müller HP, Dreyhaupt J, Gorges M, Ludolph AC, Kassubek J. Ex post facto assessment of diffusion tensor imaging metrics from different MRI protocols: Preparing for multicentre studies in ALS. Amyotroph Lateral Scler Frontotemporal Degener 2015;16(1-2):92-101
• Hawlik A, Wassner A, Ludolph AC, Lewerenz J, Rosenbohm A. Internalized capillaries in skeletal muscle biopsy. Neuromuscul Disord 2015;25(1):94-5
• Wiesner D, Sinniger J, Henriques A, Dieterlé S, Müller HP, Rasche V, Ferger B, Dirrig-Grosch S, Soylu-Kucharz R, Petersén A, Walther P, Linkus B, Kassubek J, Wong PC, Ludolph AC, Dupuis L. Low dietary protein content alleviates motor symptoms in mice with mutant dynactin/dynein-mediated neurodegeneration. Hum Mol Genet 2015;24(8):2228-40
• Zimmermann C, Kalepu R, Ponfick M, Reichel H, Cakir B, Zierz S, Gdynia HJ, Kassubek J, Ludolph AC, Rosenbohm A. Histological characterization and biochemical analysis of paraspinal muscles in neuromuscularly healthy subjects. Muscle Nerve 2015;52(1):45-54
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• Aulitzky A, Friedrich K, Gläser D, Gastl R, Kubisch C, Ludolph AC, Volk AE. A complex form of hereditary spastic paraplegia in three siblings due to somatic mosaicism for a novel SPAST mutation in the mother. J Neurol Sci 2014:pii: S0022-510X(14)00646-7
• Boxer AL, Lang AE, Grossman M, Knopman DS, Miller BL, Schneider LS, Doody RS, Lees A, Golbe LI, Williams DR, Corvol JC, Ludolph AC, Burn D, Lorenzl S, Litvan I, Roberson ED, Höglinger GU, Koestler M, Jack CR Jr, Van Deerlin V, Randolph C, Lobach IV, Heuer HW, Gozes I, Parker L, Whitaker S, Hirman J, Stewart AJ, Gold M, Morimoto BH; for the AL-108-231 Investigators. Davunetide in patients with progressive supranuclear palsy: a randomised, double-blind, placebo-controlled phase 2/3 trial. Lancet Neurol 2014;13(7):676-85
• Bozik ME, Mitsumoto H, Brooks BR, Rudnicki SA, Moore DH, Zhang B, Ludolph AC, Cudkowicz ME, van den Berg LH, Mather J, Petzinger T Jr, Archibald D. A post hoc analysis of subgroup outcomes and creatinine in the phase III clinical trial (EMPOWER) of dexpramipexole in ALS. Amyotroph Lateral Scler Frontotemporal Degener 2014;15(5-6):406-13
• Brettschneider J, Del Tredici K, Irwin DJ, Grossman M, Robinson JL, Toledo JB, Fang L, Van Deerlin VM, Ludolph AC, Lee VM, Braak H, Trojanowski JQ. Sequential distribution of pTDP-43 pathology in behavioral variant frontotemporal dementia (bvFTD). Acta Neuropathol 2014;127(3):423-39
• Brettschneider J, Arai K, Del Tredici K, Toledo JB, Robinson JL, Lee EB, Kuwabara S, Shibuya K, Irwin DJ, Fang L, Van Deerlin VM, Elman L, McCluskey L, Ludolph AC, Lee VM, Braak H, Trojanowski JQ. TDP-43 pathology and neuronal loss in amyotrophic lateral sclerosis spinal cord. Acta Neuropathol 2014;128(3):423-37
• Dengler J, Linke P, Gdynia HJ, Wolf S, Ludolph AC, Vajkoczy P, Meyer T. Differences in pain perception during open muscle biopsy and Bergstroem needle muscle biopsy. J Pain Res 2014;7:645-50
• Diekstra FP, Van Deerlin VM, van Swieten JC, Al-Chalabi A, Ludolph AC, Weishaupt JH, Hardiman O, Landers JE, Brown RH Jr, van Es MA, Pasterkamp RJ, Koppers M, Andersen PM, Estrada K, Rivadeneira F, Hofman A, Uitterlinden AG, van Damme P, Melki J, Meininger V, Shatunov A, Shaw CE, Leigh PN, Shaw PJ, Morrison KE, Fogh I, Chiò A, Traynor BJ, Czell D, Weber M, Heutink P, de Bakker PI, Silani V, Robberecht W, van den Berg LH, Veldink JH. C9orf72 and UNC13A are shared risk loci for amyotrophic lateral sclerosis and frontotemporal dementia: a genome-wide meta-analysis. Ann Neurol 2014;76(1):120-33
• Feneberg E, Steinacker P, Lehnert S, Schneider A, Walther P, Thal DR, Linsenmeier M, Ludolph AC, Otto M. Limited role of free TDP-43 as a diagnostic tool in neurodegenerative diseases. Amyotroph Lateral Scler Frontotemporal Degener 2014;15(5-6):351-6
• Freischmidt A, Müller K, Zondler L, Weydt P, Volk AE, Božič AL, Walter M, Bonin M, Mayer B, von Arnim CA, Otto M, Dieterich C, Holzmann K, Andersen PM, Ludolph AC, Danzer KM, Weishaupt JH. Serum microRNAs in patients with genetic amyotrophic lateral sclerosis and pre-manifest mutation carriers. Brain 2014;137(Pt 11):2938-50
• Gorges M, Müller HP, Ludolph AC, Rasche V, Kassubek J. Intrinsic functional connectivity networks in healthy elderly subjects: a multiparametric approach with structural connectivity analysis. Biomed Res Int 2014;2014:947252
• Grozdanov V, Bliederhaeuser C, Ruf WP, Roth V, Fundel-Clemens K, Zondler L, Brenner D, Martin-Villalba A, Hengerer B, Kassubek J, Ludolph AC, Weishaupt JH, Danzer KM. Inflammatory dysregulation of blood monocytes in Parkinson's disease patients. Acta Neuropathol 2014;128(5):651-63
• Grozdanov V, Bliederhaeuser C, Roth V, Fundel-Clemens K, Zondler L, Ruf W, Hengerer B, Kassubek J, Ludolph AC, Weishaupt JH, Danzer KM. Inflammatory dysregulation of blood monocytes in Parkinsons disease patients. J Neuroimmunol 2014;275(1-2): 84-85
• Heimrath J, Gorges M, Kassubek J, Müller HP, Birbaumer N, Ludolph AC, Lulé D. Additional resources and the default mode network: Evidence of increased connectivity and decreased white matter integrity in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2014;15:537-545
• Hübers A, Marroquin N, Schmoll B, Vielhaber S, Just M, Mayer B, Högel J, Dorst J, Mertens T, Just W, Aulitzky A, Wais V, Ludolph AC, Kubisch C, Weishaupt JH, Volk AE. Polymerase chain reaction and Southern blot-based analysis of the C9orf72 hexanucleotide repeat in different motor neuron diseases. Neurobiol Aging. 2014;35(5):1214.e1-6
• Kassubek J, Müller HP, Del Tredici K, Brettschneider J, Pinkhardt EH, Lulé D, Böhm S, Braak H, Ludolph AC. Diffusion tensor imaging analysis of sequential spreading of disease in amyotrophic lateral sclerosis confirms patterns of TDP-43 pathology. Brain 2014;137(Pt 6):1733-40
• Lehnert S, Costa J, de Carvalho M, Kirby J, Kuzma-Kozakiewicz M, Morelli C, Robberecht W, Shaw P, Silani V, Steinacker P, Tumani H, Van Damme P, Ludolph AC, Otto M. Multicentre quality control evaluation of different biomarker candidates for amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2014;15(5-6):344-50
• Lenglet T, Lacomblez L, Abitbol JL, Ludolph AC, Mora JS, Robberecht W, Shaw PJ, Pruss RM, Cuvier V, Meininger V; The Mitotarget study group. A phase II-III trial of olesoxime in subjects with amyotrophic lateral sclerosis. Eur J Neurol 2014;21(3):529-36
• Lewerenz J, Baxter P, Kassubek R, Albrecht P, Van Liefferinge J, Westhoff MA, Halatsch ME, Karpel-Massler G, Meakin PJ, Hayes JD, Aronica E, Smolders I, Ludolph AC, Methner A, Conrad M, Massie A, Hardingham GE, Maher P. Phosphoinositide 3-Kinases Upregulate System xc(-) via Eukaryotic Initiation Factor 2α and Activating Transcription Factor 4 - A Pathway Active in Glioblastomas and Epilepsy. Antioxid Redox Signal 2014;20(18):2907-22
• Lindenberg KS, Weydt P, Müller HP, Bornstedt A, Ludolph AC, Landwehrmeyer GB, Rottbauer W, Kassubek J, Rasche V. Two-point magnitude MRI for rapid mapping of brown adipose tissue and its application to the R6/2 mouse model of Huntington disease. PLoS One 2014;9(8):e105556
• Logroscino G, Ludolph AC. Amyotrophic lateral sclerosis: new ideas from cancer. Lancet Neurol 2014;13(11):1067-8
• Ludolph AC. Comment: Braak staging in clinical practice? Neurology 2014;82(10):862
• Ludolph AC. Frontotemporal degeneration in ALS - clinical variants and limits. Eur J Neurol 2014;21 1: 723-723
• Lulé D, Nonnenmacher S, Sorg S, Heimrath J, Hautzinger M, Meyer T, Kübler A, Birbaumer N, Ludolph AC. Live and let die: existential decision processes in a fatal disease. J Neurol 2014;261(3):518-25
• Mühling T, Duda J, Weishaupt JH, Ludolph AC, Liss B. Elevated mRNA-levels of distinct mitochondrial and plasma membrane Ca2+ transporters in individual hypoglossal motor neurons of endstage SOD1 transgenic mice. Front Cell Neurosci. 2014;8:353
• Müller HP, Kassubek J, Grön G, Sprengelmeyer R, Ludolph AC, Klöppel S, Hobbs NZ, Roos RA, Duerr A, Tabrizi SJ, Orth M, Süssmuth SD, Landwehrmeyer GB. Impact of the control for corrupted diffusion tensor imaging data in comparisons at the group level: an application in Huntington disease. Biomed Eng Online 2014;13:128
• Müller K, Andersen PM, Hübers A, Marroquin N, Volk AE, Danzer KM, Meitinger T, Ludolph AC, Strom TM, Weishaupt JH. Two novel mutations in conserved codons indicate that CHCHD10 is a gene associated with motor neuron disease. Brain 2014;137(Pt 12):e309
• Oeckl P, Steinacker P, von Arnim CA, Straub S, Nagl M, Feneberg E, Weishaupt JH, Ludolph AC, Otto M. Intact protein analysis of ubiquitin in cerebrospinal fluid by multiple reaction monitoring reveals differences in Alzheimer's disease and frontotemporal lobar degeneration. J Proteome Res 2014;13(11):4518-25
• Pinkhardt EH, Issa H, Gorges M, Jürgens R, Lulé D, Heimrath J, Müller HP, Ludolph AC, Becker W, Kassubek J. Do eye movement impairments in patients with small vessel cerebrovascular disease depend on lesion load or on cognitive deficits? A video-oculographic and MRI study. J Neurol. 2014;261(4):791-803
• Real RG, Dickhaus T, Ludolph AC, Hautzinger M, Kübler A. Well-being in amyotrophic lateral sclerosis: a pilot experience sampling study. Front Psychol 2014;5:704
• Rizik N, Freischmidt A, Ludolph AC, Weishaupt JH. FXN GAA repeat expansions in amyotrophic lateral sclerosis. J Clin Neurosci 2014;21(8):1319-22
• Rosenbohm A, Kassubek J, Weydt P, Marroquin N, Volk AE, Kubisch C, Huppertz HJ, Weber M, Andersen PM, Weishaupt JH, Ludolph AC, and the ALS Schwaben Register Group. Can lesions to the Motor Cortex induce Amyotrophic Lateral Sclerosis? J Neurol 2014; 261(2):283-290
• Rosenbohm A, Süssmuth SD, Kassubek J, Müller HP, Pontes C, Abicht A, Bulst S, Ludolph AC, Pinkhardt E. Novel ETFDH mutation and imaging findings in an adult with glutaric aciduria type II. Muscle and Nerve 2014;49 (3):446-450
• Rosskopf J, Müller HP, Huppertz HJ, Ludolph AC, Pinkhardt EH, Kassubek J. Frontal corpus callosum alterations in progressive supranuclear palsy but not in Parkinson's disease. Neurodegener Dis 2014;14(4):184-93
• Schneider M, Müller HP, Lauda F, Tumani H, Ludolph AC, Kassubek J, Pinkhardt EH. Retinal single-layer analysis in Parkinsonian syndromes: an optical coherence tomography study. J Neural Transm 2014;121(1):41-7
• Steinacker P, Berner C, Thal DR, Attems J, Ludolph AC, Otto M. Protease-resistant SOD1 aggregates in amyotrophic lateral sclerosis demonstrated by paraffin-embedded tissue (PET) blot. Acta Neuropathol Commun 2014;2:130
• Storch A, Kassubek J, Tumani H, Vladimirtsev VA, Hermann A, Osakovsky VL, Baranov VA, Krivoshapkin VG, Ludolph AC. Communicating hydrocephalus following eosinophilic meningitis is pathogenic for chronic Viliuisk encephalomyelitis in Northeastern Siberia. PLoS One. 2014 28;9(2):e84670
• Uenal H, Rosenbohm A, Kufeldt J, Weydt P, Goder K, Ludolph AC, Rothenbacher D, Nagel G; ALS registry Study Group. Incidence and Geographical Variation of Amyotrophic Lateral Sclerosis (ALS) in Southern Germany - Completeness of the ALS Registry Swabia. PLoS One 2014;9(4):e93932
• van Doormaal PT, Ticozzi N, Gellera C, Ratti A, Taroni F, Chiò A, Calvo A, Mora G, Restagno G, Traynor BJ, Birve A, Lemmens R, van Es MA, Saris CG, Blauw HM, van Vught PW, Groen EJ, Corrado L, Mazzini L, Del Bo R, Corti S, Waibel S, Meyer T, Ludolph AC, Goris A, van Damme P, Robberecht W, Shatunov A, Fogh I, Andersen PM, D'Alfonso S, Hardiman O, Cronin S, Rujescu D, Al-Chalabi A, Landers JE, Silani V, van den Berg LH, Veldink JH. Analysis of the KIFAP3 gene in amyotrophic lateral sclerosis: a multicenter survival study. Neurobiol Aging 2014;35(10):2420.e13-4

• Arning L, Epplen JT, Rahikkala E, Hendrich C, Ludolph AC, Sperfeld AD. The SETX missense variation spectrum as evaluated in patients with ALS4-like motor neuron diseases. Neurogenetics 2013;14(1):53-61
• Brettschneider J, Del Tredici K, Toledo JB, Robinson JL, Irwin DJ, Grossman M, Suh E, Van Deerlin VM, Wood EM, Baek Y, Kwong L, Lee EB, Elman L, McCluskey L, Fang L, Feldengut S, Ludolph AC, Lee VM, Braak H, Trojanowski JQ. Stages of pTDP-43 pathology in amyotrophic lateral sclerosis. Ann Neurol 2013;74:20-38
• Brettschneider J, Kurent J, Ludolph A. Drug therapy for pain in amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database Syst Rev 2013;6:CD005226
• Chad DA, Rowland LP, Armon C, Bedlack R, Durham H, Factor-Litvak P, Heiman-Patterson T, Heitzman D, Lacomis D, Ludolph A, Maragakis N, Miller R, Pattee G, Shoesmith C, Sorenson E, Turner MR. Peer recommendations on how to improve clinical research, and Conference wrap-up. Amyotroph Lateral Scler Frontotemporal Degener 2013;14 Suppl 1:67-73
• Cudkowicz ME, van den Berg LH, Shefner JM, Mitsumoto H, Mora JS, Ludolph AC, Hardiman O, Bozik ME, Ingersoll EW, Archibald D, Meyers AL, Dong Y, Farwell WR, Kerr DA; EMPOWER investigators. Dexpramipexole versus placebo for patients with amyotrophic lateral sclerosis (EMPOWER): a randomised, double-blind, phase 3 trial. Lancet Neurol 2013;12(11):1059-67
• Dorst J, Cypionka J, Ludolph AC. High-caloric food supplements in the treatment of amyotrophic lateral sclerosis: A prospective interventional study. Amyotroph Lateral Scler Frontotemporal Degener 2013;14(7-8):533-6
• Eschbach J, Sinniger J, Bouitbir J, Fergani A, Schlagowski AI, Zoll J, Geny B, René F, Larmet Y, Marion V, Baloh RH, Harms MB, Shy ME, Messadeq N, Weydt P, Loeffler JP, Ludolph AC, Dupuis L. Dynein mutations associated with hereditary motor neuropathies impair mitochondrial morphology and function with age. Neurobiol Dis 2013;58C:220-230
• Eschbach J, Schwalenstöcker B, Soyal SM, Bayer H, Wiesner D, Akimoto C, Nilsson AC, Birve A, Meyer T, Dupuis L, Danzer KM, Andersen PM, Witting A, Ludolph AC, Patsch W, Weydt P. PGC-1α is a male-specific disease modifier of human and experimental amyotrophic lateral sclerosis. Hum Mol Genet 2013;22(17):3477-84
• Fathinia P, Hermann A, Reuner U, Kassubek J, Storch A, Ludolph AC.
• Parkinson's disease-like midbrain hyperechogenicity is frequent in 
• amyotrophic lateral sclerosis. J Neurol 2013;260(2):454-457
• Gorges M, Müller HP, Lulé D, Ludolph AC, Pinkhardt EH, Kassubek J. Functional connectivity within the default mode network is associated with saccadic accuracy in Parkinson's disease: a resting-state FMRI and videooculographic study. Brain Connect 2013;3(3):265-72
• Hübers A, Weishaupt JH, Ludolph AC. Genetik der amyotrophen Lateralsklerose. Nervenarzt 2013;84(10):1213-9
• Ingre C, Landers JE, Rizik N, Volk AE, Akimoto C, Birve A, Hübers A, Keagle PJ, Piotrowska K, Press R, Andersen PM, Ludolph AC, Weishaupt JH. A novel phosphorylation site mutation in profilin 1 revealed in a large screen of US, Nordic, and German amyotrophic lateral sclerosis/frontotemporal dementia cohorts. Neurobiol Aging 2013;34(6):1708.e1-6
• Karcher HS, Holzwarth R, Mueller HP, Ludolph AC, Huber R, Kassubek J, Pinkhardt EH. Body Fat Distribution as a Risk Factor for Cerebrovascular Disease: An MRI-Based Body Fat Quantification Study. Cerebrovasc Dis 2013;35(4):341-8
• Lindauer E, Dupuis L, Müller HP, Neumann H, Ludolph AC, Kassubek J. Adipose Tissue Distribution Predicts Survival in Amyotrophic Lateral Sclerosis. PLoS One 2013;8(6):e67783
• Ludolph AC. Oligodendroglia: new players in amyotrophic lateral sclerosis. Brain 2013;136(Pt 2):370-1
• Lulé D, Ehlich B, Lang D, Sorg S, Heimrath J, Kübler A, Birbaumer N, Ludolph AC. Quality of life in fatal disease: the flawed judgement of the social environment. J Neurol 2013;260(11):2836-43
• Müller HP, Kassubek J, Vernikouskaya I, Ludolph AC, Stiller D, Rasche V. Diffusion Tensor Magnetic Resonance Imaging of the Brain in APP Transgenic Mice: A Cohort Study. PLoS One 2013;8(6):e67630
• Müller HP, Grön G, Sprengelmeyer R, Kassubek J, Ludolph AC, Hobbs N, Cole J, Roos RAC, Duerr A, Tabrizi SJ, Landwehrmeyer GB, Süssmuth SD. Evaluating multicenter DTI data in Huntington´s disease on site specific effects: An ex post facto approach. Neuroimage 2013;2:161-167
• Müller HP, Niessen HG, Kaulisch T, Ludolph AC, Kassubek J, Stiller D. MRI allows for longitudinal quantitative analysis of body fat composition in rats: An analysis of sibutramine-associated changes at the group level. Magn Reson Imaging 2013 ;31(7):1150-5
• Nagel G, Unal H, Rosenbohm A, Ludolph AC, Rothenbacher D; S Registry Study Group. Implementation of a population-based epidemiological rare disease registry: study protocol of the amyotrophic lateral sclerosis (ALS) - registry Swabia. BMC Neurol 2013;13:22
• Roth NM, Saidha S, Zimmermann H, Brandt AU, Oberwahrenbrock T, Maragakis NJ, Tumani H, Ludolph AC, Meyer T, Calabresi PA, Paul F. Optical coherence tomography does not support optic nerve involvement in amyotrophic lateral sclerosis. Eur J Neurol 2013;20(8):1170-6
• Stockmann M, Linta L, Föhr KJ, Boeckers A, Ludolph AC, Kuh GF, Udvardi PT, Proepper C, Storch A, Kleger A, Liebau S, Boeckers TM. Developmental and Functional Nature of Human iPSC Derived Motoneurons. Stem Cell Rev. 2013;9(4):475-92
• Stockmann M, Meyer-Ohlendorf M, Achberger K, Putz S, Demestre M, Yin H, Hendrich C, Linta L, Heinrich J, Brunner C, Proepper C, Kuh GF, Baumann B, Langer T, Schwalenstöcker B, Braunstein KE, von Arnim C, Schneuwly S, Meyer T, Wong PC, Boeckers TM, Ludolph AC, Liebau S. The dynactin p150 subunit: cell biology studies of sequence changes found in ALS/MND and Parkinsonian Syndromes. J Neural Transm 2013;120(5):785-98
• Szalardy L, Zadori D, Plangar I, Vecsei L, Weydt P, Ludolph AC, Klivenyi P, Kovacs GG. Neuropathology of Partial PGC-1α Deficiency Recapitulates Features of Mitochondrial Encephalopathies but Not of Neurodegenerative Diseases. Neurodegener Dis 2013;12(4):177-188
• Turner MR, Bowser R, Bruijn L, Dupuis L, Ludolph A, McGrath M, Manfredi G, Maragakis N, Miller RG, Pullman SL, Rutkove SB, Shaw PJ, Shefner J, Fischbeck KH. Mechanisms, models and biomarkers in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2013;14 Suppl 1:19-32
• van Rheenen W, Diekstra FP, van Doormaal PT, Seelen M, Kenna K, McLaughlin R, Shatunov A, Czell D, van Es MA, van Vught PW, van Damme P, Smith BN, Waibel S, Schelhaas HJ, van der Kooi AJ, de Visser M, Weber M, Robberecht W, Hardiman O, Shaw PJ, Shaw CE, Morrison KE, Al-Chalabi A, Andersen PM, Ludolph AC, Veldink JH, van den Berg LH. H63D polymorphism in HFE is not associated with amyotrophic lateral sclerosis. Neurobiol Aging 2013;34(5):1517.e5-7
• Waibel S, Neumann M, Rosenbohm A, Birve A, Volk AE, Weishaupt JH, Meyer T, Müller U, Andersen PM, Ludolph AC. Truncating mutations in FUS/TLS give rise to a more aggressive ALS-phenotype than missense mutations: a clinico-genetic study in Germany. Eur J Neurol 2013;20(3):540-6
• Weishaupt JH, Waibel S, Birve A, Volk AE, Mayer B, Meyer T, Ludolph AC, Andersen PM. A novel optineurin truncating mutation and three glaucoma-associated missense variants in patients with familial amyotrophic lateral sclerosis in Germany. Neurobiol Aging 2013;34(5):1516.e9-1516.e15
• Wiesner D, Merdian I, Lewerenz J, Ludolph AC, Dupuis L, Witting A.NeurFumaric Acid Esters Stimulate Astrocytic VEGF Expression through HIF-1α and Nrf2.PLoS One. 2013;8(10):e76670

• Uttner I, Weber S, Freund W, Bengel D, Schmitz B, Ludolph AC, Huber R. Long-term outcome in TGA patients with and without focal hyperintensities in the CA1 region of the hippocampus. Eur Neurol 2012;67:155-160
• Jesse S, Kassubek J, Müller H-P, Ludolph AC, Unrath A. Signal alterations of the basal ganglia in the differential diagnosis of Parkinson's disease: a retrospective case-controlled MRI data bank analysis BMC Neurology 2012; 12 (1): 163(1-8)
• Müller HP, Vernikouskaya I, Ludolph AC, Kassubek J, Rasche V. Fast diffusion tensor magnetic resonance imaging of the mouse brain at ultrahigh-field: aiming at cohort studies. PLoS One 2012;7(12):e53389
• Kuh GF, Stockmann M, Meyer-Ohlendorf M, Linta L, Proepper C, Ludolph AC, Bockmann J, Boeckers TM, Liebau S. Tubulin-binding cofactor B is a direct interaction partner of the dynactin subunit p150(Glued). Cell Tissue Res 2012;350(1):13-26
• Cipresso P, Carelli L, Solca F, Meazzi D, Meriggi P, Poletti B, Lule D, Ludolph AC, Silani V, Riva G. The use of P300-based BCIs in amyotrophic lateral sclerosis: from augmentative and alternative communication to cognitive assessment. Brain Behav 2012;2(4):479-498
• Ludolph AC, Brettschneider J, Weishaupt JH. Amyotrophic lateral sclerosis. Curr Opin Neurol 2012;25(5):530-5
• Unrath A, Müller HP, Ludolph AC, Kassubek J. Reversible cortical diffusion restriction, hyperperfusion and t2-hyperintensity caused by two different types of epileptic seizure. Clin Neuroradiol 2012;22(3):239-243
• von Arnim CA, Herbolsheimer F, Nikolaus T, Peter R, Biesalski HK, Ludolph AC, Riepe M, Nagel G an the ActiFE Ulm study group. Dietary antioxidants and dementia in a population-based case-control study among older people in South Germany. J Alzheimers Dis 2012;31(4):717-724
• Dupuis L, Dengler R, Heneka MT, Meyer T, Zierz S, Kassubek J, Fischer W, Steiner F, Lindauer E, Otto M, Dreyhaupt J, Grehl T, Hermann A, Winkler AS, Bogdahn U, Benecke R, Schrank B, Wessig C, Grosskreutz J, Ludolph AC; the GERP ALS Study Group. A Randomized, Double Blind, Placebo-Controlled Trial of Pioglitazone in Combination with Riluzole in Amyotrophic Lateral Sclerosis. PLoS One 2012;7(6):e37885
• Ponfick M, Ludolph AC, Dekomien G, Uttner I, Kassubek J, Gdynia HJ. Inclusion body myositis mit Morbus Paget und frontotemporaler Demenz: frühe Beteiligung des Herzens und der Atemmuskulatur. Fortschr Neurol Psych 2012;80(6):344-7
• Blauw HM, van Rheenen W, Koppers M, Van Damme P, Waibel S, Lemmens R, van Vught PW, Meyer T, Schulte C, Gasser T, Cuppen E, Pasterkamp RJ, Robberecht W, Ludolph AC, Veldink JH, van den Berg LH. NIPA1 polyalanine repeat expansions are associated with amyotrophic lateral sclerosis. Hum Mol Genet. 2012;21(11):2497-502
• Müller HP, Unrath A, Huppertz HJ, Ludolph AC, Kassubek J. Neuroanatomical patterns of cerebral white matter involvement in different motor neuron diseases as studied by diffusion tensor imaging analysis. Amyotroph Lateral Scler 2012;13(3):254-64
• Jesse S, Ludolph AC. [Thiamine, pyridoxine and cobalamine. From myths to pharmacology and clinical practice]. Nervenarzt 2012;83(4):521-32
• Kassubek J, Ludolph AC, Müller HP. Neuroimaging of motor neuron diseases. Ther Adv neurol Disord 2012;5(2):119-127
• Oeckl P, Steinacker P, Lehnert S, Jesse S, Kretzschmar HA, Ludolph AC, Otto M, Ferger B. CSF Concentrations of cAMP and cGMP Are Lower in Patients with Creutzfeldt-Jakob Disease but Not Parkinson's Disease and Amyotrophic Lateral Sclerosis. PLoS One 2012;7(3):e32664
• Knauer C, Knauer K, Muller S, Ludolph AC, Bengel D, Muller HP, Huber R. A biochemical marker panel in MRI-proven hyperacute ischemic stroke - a prospective study. BMC Neurol 2012;12(1):14
• Yilmaz A, Gdynia HJ, Ponfick M, Rösch S, Lindner A, Ludolph AC, Sechtem U. Cardiovascular magnetic resonance imaging (CMR) reveals characteristic pattern of myocardial damage in patients with mitochondrial myopathy. Clin Res Cardiol 2012;101(4):255-61
• Nagel L, Rosenbohm A, Ünal1 H, Rothenbacher D, Ludolph AC. Möglichkeiten register-basierter Studien zur Erforschung seltener Erkrankungen. Beispiel des ersten deutschen bevölkerungsbezogenen Registers für die Amyotrophe Lateralsklerose. Akt Neurol 2012: 39:12-17
• Pinkhardt EH, Jurgens R, Lule D, Heimrath J, Ludolph AC, Becker W, Kassubek J. Eye movement impairments in Parkinson's disease: possible role of extradopaminergic mechanisms. BMC Neurol 2012 Feb 29;12(1):5
• Kassubek J, Sperfeld AD, Pinkhardt EH, Unrath A, Müller HP, Scharffetter-Kochanek K, Ludolph AC, Berneburg M. The cerebro-morphological fingerprint of a progeroid syndrome: white matter changes correlate with neurological symptoms in xeroderma pigmentosum. PLoS One 2012;7(2):e30926
• Lulé D, Pauli S, Altintas E, Singer U, Merk T, Uttner I, Birbaumer N, Ludolph AC. Emotional adjustment in amyotrophic lateral sclerosis (ALS). J Neurol 2012;259(2):334-41
• Meyer C, Landwehrmeyer B, Schwenke C, Doble A, Orth M, Ludolph AC; on behalf of the EHDI Study Group. Rate of change in early Huntington's disease: A clinicometric analysis. Mov Disord 2012;27(1):118-124
• Lebedeva E, Stingl JC, Thal DR, Ghebremedhin E, Strauss J, Ozer E, Bertram L, von Einem B, Tumani H, Otto M, Riepe MW, Högel J, Ludolph AC, von Arnim CA. Genetic variants in PSEN2 and correlation to CSF β-amyloid42 levels in AD. Neurobiol Aging 2012;33(1):201.e9-201.e18
• Gispert S, Kurz A, Waibel S, Bauer P, Liepelt I, Geisen C, Gitler AD, Becker T, Weber M, Berg D, Andersen PM, Krüger R, Riess O, Ludolph AC, Auburger G. The modulation of Amyotrophic Lateral Sclerosis risk by Ataxin-2 intermediate polyglutamine expansions is a specific effect. Neurobiol Dis 2012;45(1):356-61
• Lulé D, Heimrath J, Pinkhardt EH, Ludolph AC, Uttner I, Kassubek J. Deep brain stimulation and behavioural changes: is comedication the most important factor? Neurodegener Dis 2012;9(1):18-24
• Otto M, Bowser R, Turner M, Berry J, Brettschneider J, Connor J, Costa J, Cudkowicz M, Glass J, Jahn O, Lehnert S, Malaspina A, Parnetti L, Petzold A, Shaw P, Sherman A, Steinacker P, Süßmuth S, Teunissen C, Tumani H, Wuolikainen A, Ludolph A. Roadmap and standard operating procedures for biobanking and discovery of neurochemical markers in ALS. Amyotroph Lateral Sc 2012;13(1):1-10

• Müller HP, Süssmuth SD, Landwehrmeyer GB, Ludolph AC, Tabrizi SJ, Kloppel S, Kassubek J. Stability effects on results of diffusion tensor imaging analysis by reduction of the number of gradient directions due to motion artifacts: an application to presymptomatic Huntington's disease. PLoS Curr 2011;3:RRN1292
• van Es MA, Schelhaas HJ, van Vught PW, Ticozzi N, Andersen PM, Groen EJ, Schulte C, Blauw HM, Koppers M, Diekstra FP, Fumoto K, Leclerc AL, Keagle P, Bloem BR, Scheffer H, van Nuenen BF, van Blitterswijk M, van Rheenen W, Wills AM, Lowe PP, Hu GF, Yu W, Kishikawa H, Wu D, Folkerth RD, Mariani C, Goldwurm S, Pezzoli G, Van Damme P, Lemmens R, Dahlberg C, Birve A, Fernández-Santiago R, Waibel S, Klein C, Weber M, van der Kooi AJ, de Visser M, Verbaan D, van Hilten JJ, Heutink P, Hennekam EA, Cuppen E, Berg D, Brown RH Jr, Silani V, Gasser T, Ludolph AC, Robberecht W, Ophoff RA, Veldink JH, Pasterkamp RJ, de Bakker PI, Landers JE, van de Warrenburg BP, van den Berg LH.Angiogenin variants in Parkinson disease and amyotrophic lateral sclerosis. Ann Neurol 2011;70(6):964-73
• Couthouis J, Hart MP, Shorter J, Dejesus-Hernandez M, Erion R, Oristano R, Liu AX, Ramos D, Jethava N, Hosangadi D, Epstein J, Chiang A, Diaz Z, Nakaya T, Ibrahim F, Kim HJ, Solski JA, Williams KL,  Mojsilovic-Petrovic J, Ingre C, Boylan K, Graff-Radford NR, Dickson DW, Clay-Falcone D, Elman L, McCluskey L,  Greene R, Kalb RG, Lee VM, Trojanowski JQ, Ludolph AC, Robberecht W, Andersen PM, Nicholson GA, Blair IP, King OD, Bonini NM, Van Deerlin V, Rademakers R, Mourelatos Z, Gitler AD. Feature Article: A yeast functional screen predicts new candidate ALS disease genes. Proc Natl Acad Sci U S A 2011;108(52):20881-90
• McCrone P, Payan CA, Knapp M, Ludolph AC, Agid Y, Leigh PN, Bensimon G; NNIPPS Study Group. The economic costs of progressive supranuclear palsy and multiple system atrophy in France, Germany and the United Kingdom. PLoS One 2011;6(9):e24369
• Thal DR, Del Tredici K, Ludolph AC, Hoozemans JJ, Rozemuller AJ, Braak H, Knippschild U. Stages of granulovacuolar degeneration: their relation to Alzheimer's disease and chronic stress response. Acta Neuropathol. 2011;122(5):577-589
• Steinacker P, Fang L, Kuhle J, Petzold A, Tumani H, Ludolph AC, Otto M, Brettschneider J. Soluble beta-amyloid precursor protein is related to disease progression in amyotrophic lateral sclerosis. PLoS One 2011;6(8):e23600
• Weber YG, Kamm C, Suls A, Kempfle J, Kotschet K, Schüle R, Wuttke TV, Maljevic S, Liebrich J, Gasser T, Ludolph AC, Van Paesschen W, Schöls L, De Jonghe P, Auburger G, Lerche H. Paroxysmal choreoathetosis/spasticity (DYT9) is caused by a GLUT1 defect. Neurology 2011;77(10):959-64
• Ponfick M, Gdynia HJ, Kassubek J, Ludolph AC, Petscher K, Walcher T, Walcher D, Bernhardt P. Cardiac involvement in juvenile overlap-myositis detected by cardiac magnetic resonance imaging. Int J Cardiol 2011;6;152(1):e25-6
• Otto M, Ludolph AC, Landwehrmeyer B, Förstl H, Diehl-Schmid J, Neumann M, Kretzschmar HA, Schroeter M, Kornhuber J, Danek A; FTLD consortium. Konsortium zur Erforschung der frontotemporalen Lobärdegeneration. Nervenarzt 2011;82(8):1002-5
• Ludolph AC, Kassubek J. Erweiterte therapeutische Strategien bei neurologischen Erkrankungen. Nervenarzt 2011;82(8):955-6
• Schulz JB, Gerlach M, Gille G, Kuhn W, Müngersdorf M, Riederer P, Südmeyer M, Ludolph AC. Basic science in Parkinson's disease: its impact on clinical practice. J Neurol. 2011;258(Suppl 2):S299-306
• Ludolph AC, Kassubek J. Neuen Erkenntnissen Rechnung tragen. MedReport 2011;27:1
• Berger R, Jennewein C, Marschall V, Karl S, Cristofanon S, Wagner L, Vellanki SH, Hehlgans S, Rödel F, Debatin KM, Ludolph AC, Fulda S. NF-{kappa}B Is Required for Smac Mimetic-Mediated Sensitization of Glioblastoma Cells for {gamma}-Irradiation-Induced Apoptosis. Mol Cancer Ther 2011;10(10):1867-75
• Rolland Y, Vérin M, Payan CA, Duchesne S, Kraft E, Hauser TK, Jarosz J, Deasy N, Defevbre L, Delmaire C, Dormont D, Ludolph AC, Bensimon G, Leigh PN; on behalf of the NNIPPS Study Group. A new MRI rating scale for progressive supranuclear palsy and multiple system atrophy: validity and reliability. J Neurol Neurosurg Psychiatry 2011;82(9):1025-1032
• Kassubek J, Pinkhardt EH, Dietmaier A, Ludolph AC, Landwehrmeyer GB, Huppertz HJ. Fully Automated Atlas-Based MR Imaging Volumetry in Huntington Disease, Compared with Manual Volumetry. AJNR Am J Neuroradiol 2011;32(7):1328-32
• Payan CA, Viallet F, Landwehrmeyer BG, Bonnet AM, Borg M, Durif F, Lacomblez L, Bloch F, Verny M, Fermanian J, Agid Y, Ludolph AC, Leigh PN, Bensimon G; on behalf of the NNIPPS Study Group. Disease Severity and Progression in Progressive Supranuclear Palsy and Multiple System Atrophy: Validation of the NNIPPS - PARKINSON PLUS SCALE. PLoS One 2011;6(8):e22293
• Ludolph AC. Erweiterte therapeutische Strategien bei neurologischen Erkrankungen. Nervenarzt 2011;82:955-956
• Schulz JB, Gerlach M, Gille G, Kuhn W, Müngersdorf M, Riederer P, Südmeyer M, Ludolph A. Basic science in Parkinson's disease: its impact on clinical practice. J Neurol. 2011;258(Suppl 2):S299-306
• Brainin M, Berardelli A, Boon P, Gilhus NE, Leys D, Ludolph AC, Schapira AH, Skvortskova V; for the EFNS Scientific Committee. Comment from the EFNS Scientific Committee on the letter from Gunther Haag concerning Bendsten L et al. EFNS guideline on the treatment of tension-type headache - report of an EFNS task force. Eur J Neurol 2010; 17: 1318-1325. Eur J Neurol 2011;18(7):e83-e84
• Yilmaz A, Gdynia HJ, Ponfick M, Ludolph AC, Rösch S, Sechtem U. The proteoglycan-dystrophin complex in genetic cardiomyopathies-lessons from three siblings with limb-girdle muscular dystrophy-2I (LGMD-2I). Clin Res Cardiol 2011;100(7):611-5
• Weis J, Katona I, Müller-Newen G, Sommer C, Necula G, Hendrich C, Ludolph AC, Sperfeld AD. Small-fiber neuropathy in patients with ALS. Neurology 2011;76(23):2024-9
• Ponfick M, Gdynia H.-J, Ludolph AC, Kassubek J. Camptocormia in Parkinson´s disease - A review of the literature. Neurodegen Diseases 2011;8(5): 283-288
• Jesse S, Brettschneider J, Süssmuth SD, Landwehrmeyer BG, von Arnim CA, Ludolph AC, Tumani H, Otto M. Summary of cerebrospinal fluid routine parameters in neurodegenerative diseases. J Neurol 2011;258(6):1034-41
• Unrath A, Ludolph AC, Kassubek J. Alterations of the Corpus Callosum as an MR Imaging Based Hallmark of Motor Neuron Diseases. AJNR Am J Neuroradiol 2011;32(5):E90
• Uttner I, Weber S, Freund W, Bengel D, Schmitz B, Ludolph AC, Huber R. Hippocampal cavities are not associated with cognitive impairment in transient global amnesia. Eur J Neurol 2011;18(6):882-7
• Kuźma-Kozakiewicz M, Usarek E, Ludolph AC, Barańczyk-Kuźma A. Mice with Mutation in Dynein Heavy Chain 1 Do Not Share the Same Tau Expression Pattern with Mice with SOD1-Related Motor Neuron Disease. Neurochem Res 2011;36(6):978-85
• Fergani A, Eschbach J, Oudart H, Larmet Y, Schwalenstocker B, Ludolph AC, Loeffler JP, Dupuis L. A mutation in the dynein heavy chain gene compensates for energy deficit of mutant SOD1 mice and increases potentially neuroprotective IGF-1. Mol Neurodegener 2011;6(1):26
• Fernández-Santiago R, Sharma M, Berg D, Illig T, Anneser J, Meyer T, Ludolph AC, Gasser T. No evidence of association of FLJ10986 and ITPR2 with ALS in a large German cohort. Neurobiol Aging 2011;32(3):551.e1-4
• Dorst J, Kühnlein P, Hendrich C, Kassubek J, Sperfeld AD, Ludolph AC. Patients with elevated triglyceride and cholesterol serum levels have a prolonged survival in amyotrophic lateral sclerosis. J Neurol 2011;258(4):613-7
• Treiber N, Maity P, Singh K, Kohn M, Keist AF, Ferchiu F, Sante L, Frese S, Bloch W, Kochanek S, Sindrilaru A, Iben S, Högel J, Ohnmacht M, Claes LE, Ignatius A, Chung JH, Lee MJ, Kamenisch Y, Berneburg M, Nikolaus T, Braunstein K, Sperfeld AD, Ludolph AC, Briviba K, Wlaschek M, Scharffetter-Kochanek K. Accelerated aging phenotype in mice with conditional deficiency for mitochondrial superoxide dismutase in the connective tissue. Aging Cell 2011;10:239-254
• Rolfs A, Martus P, Heuschmann PU, Grittner U, Holzhausen M, Tatlisumak T, Böttcher T, Fazekas F, Enzinger C, Ropele S, Schmidt R, Riess O, Norrving B. Protocol and Methodology of the Stroke in Young Fabry Patients (sifap1) Study [including Ludolph AC, Huber R, Knauer K, Hendrich C, Raubold S]. A Prospective Multicenter European Study of 5,024 Young Stroke Patients Aged 18-55 Years. Cerebrovasc Dis 2011;31(3):253-62
• Kühnlein P, Jung H, Farkas M, Keskitalo S, Ineichen B, Jelcic I, Petersen J, Semmler A, Weller M, Ludolph AC, Linnebank M. The thermolabile variant of 5,10-methylenetetrahydrofolate reductase is a possible risk factor for amyotrophic lateral sclerosis. Amyotroph Lateral Scler 2011;12(2):136-9
• Müller HP, Raudies F, Unrath A, Neumann H, Ludolph AC, Kassubek J. Quantification of human body fat tissue percentage by MRI. NMR Biomed 2011;24(1):17-24
• Brettschneider J, Hartmann N, Lehmensiek V, Mogel H, Ludolph AC, Tumani H. Cerebrospinal fluid markers of idiopathic intracranial hypertension: Is the renin-angiotensinogen system involved? Cephalalgia 2011 Jan;31(1):116-21
• Ludolph AC. Motor neuron disease: Urgently needed-biomarkers for amyotrophic lateral sclerosis. Nat Rev Neurol 2011;7(1):13-4
• Müller HP, Lulé D, Unrath A, Ludolph AC, Riecker A, Kassubek J. Complementary image analysis of diffusion tensor imaging and 3-dimensional t1-weighted imaging: white matter analysis in amyotrophic lateral sclerosis. J Neuroimaging. 2011;21(1):24-33
• Dupuis L, Pradat PF, Ludolph AC, Loeffler JP. Energy metabolism in amyotrophic lateral sclerosis. Lancet Neurol 2011;10(1):75-82
• Eschbach J, Fergani A, Oudart H, Robin JP, Rene F, Gonzalez de Aguilar JL, Larmet Y, Zoll J, Hafezparast M, Schwalenstocker B, Loeffler JP, Ludolph AC, Dupuis L. Mutations in cytoplasmic dynein lead to a Huntington's disease-like defect in energy metabolism of brown and white adipose tissues. Biochim Biophys Acta 2011;1812(1):59-69

• Süssmuth SD, Uttner I, Landwehrmeyer B, Pinkhardt EH, Brettschneider J, Petzold A, Kramer B, Schulz JB, Palm C, Otto M, Ludolph AC, Kassubek J, Tumani H. Differential pattern of brain-specific CSF proteins tau and amyloid-beta in Parkinsonian syndromes. Mov Disord 2010;25(9):1284-8
• Braunstein KE, Eschbach J, Ròna-Vörös K, Soylu R, Mikrouli E, Larmet Y, Rene F, Gonzalez de Aguilar JL, Loeffler JP, Müller HP, Bucher S, Kaulisch T, Niessen HG, Tillmanns J, Fischer K, Schwalenstocker B, Kassubek J, Pichler B, Stiller D, Petersen A, Ludolph AC, Dupuis L. A point mutation in the dynein heavy chain gene leads to striatal atrophy and compromises neurite outgrowth of striatal neurons. Hum Mol Genet 2010;19:4385-98
• Unrath A, Müller HP, Riecker A, Ludolph AC, Sperfeld AD, Kassubek J. Whole brain-based analysis of regional white matter tract alterations in rare motor neuron diseases by diffusion tenson imaging. Human Brain Mapp 2010;31:1727-1740
• Blauw HM, Al-Chalabi A, Andersen PM, van Vught PW, Diekstra FP, van Es MA, Saris CG, Groen EJ, van Rheenen W, Koppers M, Van't Slot R, Strengman E, Estrada K, Rivadeneira F, Hofman A, Uitterlinden AG, Kiemeney LA, Vermeulen SH, Birve A, Waibel S, Meyer T, Cronin S, McLaughlin RL, Hardiman O, Sapp PC, Tobin MD, Wain LV, Tomik B, Slowik A, Lemmens R, Rujescu D, Schulte C, Gasser T, Brown RH Jr, Landers JE, Robberecht W, Ludolph AC, Ophoff RA, Veldink JH, van den Berg LH. A large genome scan for rare CNVs in amyotrophic lateral sclerosis. Hum Mol Genet 2010;19:4091-9
• Ludolph AC. Zum Gedenken an Professor Hans Helmut Kornhuber. Akt Neurol 2010;37:304
• Brown RG, Lacomblez L, Landwehrmeyer BG, Bak T, Uttner I, Dubois B, Agid Y, Ludolph AC, Bensimon G, Payan C, Leigh NP; NNIPPS Study Group. Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy. Brain 2010;133(Pt 8):2382-93
• Waibel S, Neumann M, Rabe M, Meyer T, Ludolph AC. Novel missense and truncating mutations in FUS/TLS in familial ALS. Neurology 2010;75(9):815-7
• Brettschneider J, Czerwoniak A, Senel M, Fang L, Kassubek J, Pinkhardt E, Lauda F, Kapfer T, Jesse S, Lehmensiek V, Ludolph AC, Otto M, Tumani H. The chemokine CXCL13 is a prognostic marker in clinically isolated syndrome (CIS). PLoS One 2010;5(8):e11986
• Ferger AI, Campanelli L, Reimer V, Muth KN, Merdian I, Ludolph AC, Witting A. Effects of mitochondrial dysfunction on the immunological properties of microglia. J Neuroinflammation. 2010;7:45
• Senel M, Rupprecht TA, Tumani H, Pfister HW, Ludolph AC, Brettschneider J. The chemokine CXCL13 in acute neuroborreliosis. J Neurol Neurosurg Psych 2010;81(8):929-33
• Fang L, Teuchert M, Huber-Abel F, Schattauer D, Hendrich C, Dorst J, Zettlmeissel H, Wlaschek M, Scharffetter-Kochanek K, Kapfer T, Tumani H, Ludolph AC, Brettschneider J. MMP-2 and MMP-9 are elevated in spinal cord and skin in a mouse model of ALS. J Neurol Sci 2010;294(1-2):51-6
• Lulé D, Diekmann V, Müller HP, Kassubek J, Ludolph AC, Birbaumer N. Neuroimaging of multimodal sensory stimulation in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 2010;81(8):899-906
• Pinkhardt EH, Sperfeld AD, Uttner I, Ludolph AC, Kassubek J. Amygdala size reduction is associated with memory deficits in complicated hereditary spastic paraparesis: An MRI Study. Eur Neurol 2010;64(2):117-123
• Habisch HJ, Liebau S, Lenk T, Ludolph AC, Brenner R, Storch A. Neuroectodermally converted human mesenchymal stromal cells provide cytoprotective effects on neural stem cells and inhibit their glial differentiation. Cytotherapy 2010;12:491-504
• Braak H, Ludolph AC, Thal DR, Del Tredici K. Amyotrophic lateral sclerosis: dash-like accumulation of phosphorylated TDP-43 in somatodendritic and axonal compartments of somatomotor neurons of the lower brainstem and spinal cord. Acta Neuropathol 2010;120(1):67-74
• Rabe M, Felbecker A, Waibel S, Steinbach P, Winter P, Ludolph AC. The epidemiology of CuZn-SOD mutations in Germany: a study of 217 families. J Neurol 2010;257:1298-1302
• Habisch HJ, Schmid B, von Arnim CA, Ludolph AC, Brenner R, Storch A. Efficient processing of Alzheimer's disease amyloid-Beta peptides by neuroectodermally converted mesenchymal stem cells. Stem Cells Dev 2010;19(5):629-33
• Felbecker A, Camu W, Valdmanis PN, Sperfeld AD, Waibel S, Steinbach P, Rouleau GA, Ludolph AC, Andersen PM. Four familial ALS pedigrees discordant for two SOD1 mutations: are all SOD1 mutations pathogenic? J Neurol Neurosurg Psych 2010;81(5):572-7
• Süssmuth SD, Sperfeld AD, Ludolph AC, Tumani H. Hypercapnia is a possible determinant of the function of the blood-cerebrospinal fluid barrier in amyotrophic lateral sclerosis. Neurochem Res 2010;35(7):1071-4
• Uttner I, Kirchheiner J, Tumani H, Mottaghy FM, Lebedeva E, Ozer E, Ludolph AC, Huber R, von Arnim CA. A novel presenilin1 mutation (Q223R) associated with early onset Alzheimer's disease, dysarthria and spastic paraparesis and decreased Abeta levels in CSF. Eur J Neurol 2010;17(4):631-3
• Steinacker P, Hawlik A, Lehnert S, Jahn O, Meier S, Görz E, Braunstein KE, Krzovska M, Schwalenstöcker B, Jesse S, Pröpper C, Böckers T, Ludolph AC, Otto M. Neuroprotective function of cellular prion protein in a mouse model of amyotrophic lateral sclerosis. Am J Pathol 2010;176(3):1409-20
• Yilmaz A, Gdynia HJ, Ludolph AC, Klingel K, Kandolf R, Sechtem U. Images in cardiovascular medicine. Cardiomyopathy in a Duchenne muscular dystrophy carrier and her diseased son: similar pattern revealed by cardiovascular MRI. Circulation 2010;121(10):e237-9
• Süssmuth SD, Sperfeld AD, Hinz A, Brettschneider J, Endruhn S, Ludolph AC, Tumani H. CSF glial markers correlate with survival in amyotrophic lateral sclerosis. Neurology 2010;74(12):982-7
• Gdynia HJ, Ludolph AC. Polyneuropathy in type 1 and type 2 diabetes: do we need different examinations? Diabetes Metab Res Rev. 2010;26(2):107-8
• Ludolph AC, Bendotti C, Blaugrund E, Chio A, Greensmith L, Loeffler JP, Mead R, Niessen HG, Petri S, Pradat PF, Robberecht W, Ruegg M, Schwalenstöcker B, Stiller D, van den Berg L, Vieira F, von Horsten S. Guidelines for preclinical animal research in ALS/MND: A consensus meeting. Amyotroph Lateral Scler 2010;11(1-2):38-45
• Brettschneider J, Lehmensiek V, Mogel H, Pfeifle M, Dorst J, Hendrich C, Ludolph AC, Tumani H. Proteome analysis reveals candidate markers of disease progression in amyotrophic lateral sclerosis (ALS). Neurosci Lett 2010;468:23-27

• Meininger V, Drory VE, Leigh PN, Ludolph AC, Robberecht W, Silani V. Glatiramer acetate has no impact on disease progression in ALS at 40 mg/day: a double- blind, randomized, multicentre, placebo-controlled trial. Amyotroph Lateral Scler 2009;10(5-6):378-83
• Süssmuth SD, Landfester K, Tumani H, Ludolph AC, Brettschneider J. Blood-brain barrier in neurodegenerative diseases: perspectives for Nanomedicine. Eur J Nanomed 2009;2:39-47
• Lambrechts D, Poesen K, Fernandez-Santiago R, Al-Chalabi A, Del Bo R, Khan S, Van Vught PWJ, Marklund SL, Brockington A, von Marion I, Anneser J, Shaw Ch, Ludolph AC, Leigh NP, Comi GP, Gasser T, Shaw PJ, Morrison KE, Andersen PM, Van den Berg L, Thijs V, Siddique D, Robberecht W. Meta-analysis of vascular endothelial growth factor variations in amyotrophic lateral sclerosis: increased susceptibility in male carriers or the – 2578AA genotype. J Med Genet 2009;46:840-846
• Lulé D, Ludolph AC, Kassubek J. MRI-based functional neuroimaging in ALS: An update. Amyotrophic Lateral Scler 2009;10(5-6):258-268
• Brettschneider J, Tumani H, Kiechle U, Muche R, Richards G, Lehmensiek V, Ludolph AC, Otto M. IgG antibodies against measles, rubella, and varicella zoster virus predict conversion to multiple sclerosis in clinically isolated syndrome. PLoS One 2009;4(11):e7638
• Olanow CW, Rascol O, Hauser R, Feigin PD, Jankovic J, Lang A, Langston W, Melamed E, Poewe W, Stocchi F, Tolosa E, for the ADAGIO Study Investigators [including Ludolph AC]. A double-blind, delayed-start trial of rasagiline in Parkinson´s disease. N Engl J Med 2009;361:1268-1278
• Al Chalabi A, Dürr A, Wood NW, Parkinson MH, Camuzat A, Hulot JS, Morrison KE, Renton A, Süssmuth SD, Landwehrmeyer BG, Ludolph AC, Agid Y, Brice A, Leigh N, Bensimon G. Genetic variants of the α-synuclein gene SNCA are associated with multiple system atrophy. PloS ONE 2009;4(9):e7114
• Van Es MA, Veldink JH, Saris CGJ, Blauw HM, van Vught PWJ, Birve A, Lemmens R, Schelhaas HJ, Groen E, Huisman MHB, can der Kooi AJ, de Visser M, Rivadeneira F, Hofman A, Rujescu D, Strengman E, Goldstein D, Muglia P, Slowik A, Uitterlinden AG, Hendrich C, Waibel S, Meyer T, Ludolph AC, Glass JD, Purcell S, Landers J, Vermeulen SHM, Kiemeney LA, Wokke JHJ, Cronin S, Hardiman O, Fumoto K, Pasterkamp RJ, Meininger V, Melki J, Leigh PN, Shaw CE, Al-Chalabi A, Brown RH, Robberecht W, Andersen PM, Ophoff  RA, van den Berg LH. Genome-wide association study identifies UNC13A and 9p21.2 as susceptibility loci for sporadic amyotrophic lateral sclerosis. Nature Genetics 2009,41:1083-1087
• Soukup GR, Sperfeld AD, Uttner I, Karitzky J, Ludolph AC, Kassubek J, Schreiber H. Frontotemporal cognitive function in X-linked spinal and bulbar muscular atrophy (SBMA): a controlled neuropsychological study of 20 patients. J Neurol 2009;256(11):1869-75
• Gdynia HJ, Sperfeld AD, Unrath A, Ludolph AC, Sabolek M, Storch A, Kassubek J. Histopathological analysis of skeletal muscle in patients with Parkinson's disease and 'dropped head'/'bent spine' syndrome. Parkinsonism Relat Disord 2009;15(9):633-9
• Gdynia HJ, Müller HP, Ludolph AC, Köninger H, Huber R. Quantitative muscle ultrasound in neuromuscular disorders using the parameters 'intensity', 'entropy', and 'fractal dimension. Eur J Neurol 2009;16:1151-58
• Fernández-Santiago R, Hoenig S, Lichtner P, Sperfeld AD, Sharma M, Berg D, Weichenrieder O, Illig T, Eger K, Meyer T, Anneser J, Münch C, Zierz S, Gasser T, Ludolph AC. Identification of novel Angiogenin (ANG) gene missense variants in German patients with amyotrophic lateral sclerosis. J Neurol 2009;256(8):1337-42
• Steinacker P, Hendrich C, Sperfeld AD, Jesse S, Lehnert S, Pabst A, von Arnim CA, Mottaghy FM, Uttner I, Tumani H, Ludolph AC, Otto M. Concentrations of beta-amyloid precursor protein processing products in cerebrospinal fluid of patients with amyotrophic lateral sclerosis and frontotemporal lobar degeneration. J Neural Transm 2009;116(9):1169-78
• Fang L, Huber-Abel F, Teuchert M, Hendrich C, Dorst J, Schattauer D, Zettlmeissel H, Wlaschek M, Scharffetter-Kochanek K, Tumani H, Ludolph AC, Brettschneider J. Linking neuron and skin: Matrix metalloproteinases in amyotrophic lateral sclerosis (ALS). J Neurol Sci 2009;285(1-2):62-6
• Tumani H, Pfeifle M, Lehmensiek V, Rau D, Mogel H, Ludolph AC, Brettschneider J. Candidate biomarkers of chronic inflammatory demyelinating polyneuropathy (CIDP): Proteome analysis of cerebrospinal fluid. J Neuroimmunol 2009;214(1-2):109-112
• Ludolph AC, Jesse S. Evidence-based drug treatment in amyotrophic lateral sclerosis and upcoming clinical trials. Ther Adv Neurol Dis 2009;2:319-326
• Pinkhardt E, Kassubek J, Süßmuth S, Ludolph AC, Becker W, Jürgens R. Comparison of smooth pursuit eye movement deficits in multiple system atrophy and Parkinson´s disease. J Neurol 2009;256:1438-1446
• Dupuis L, Fergani A, Braunstein KE, Eschbach J, Holl N, Rene F, Gonzalez De Aguilar JL, Zoerner B, Schwalenstoecker B, Ludolph AC, Loeffler JP. Mice with a mutation in the dynein heavy chain 1 gene display sensory neuropathy but lack motor neuron disease. Exp Neurol 2009;215:146-52
• Ludolph AC. Neuromuscular diseases: new hopes for alleviation and elimination. Lancet Neurol 2009;8:16-7
• Bensimon G, Ludolph AC, Agid Y, Vidailhet M, Payan C, Leigh PN, NNIPPS Study Group. Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: The NNIPPS Study. Brain 2009;132:156-171
• Tumani H, Lehmensiek V, Rau D, Guttmann I, Tauscher G, Mogel H, Palm C, Hirt V, Suessmuth S, Sapunova-Meyer I, Ludolph AC, Brettschneider J. CSF proteome analysis in clinically isolated syndrome (CIS): Candidate markers for conversion to definite multiple sclerosis. Neurosci Lett 2009:452:214-217
• Diener HC, Hartung HP, Bien CG, Hacke W, Ringelstein EB, Ludolph AC, Deutschl G, Wiltfang J, Weller M, Steinhausen K, Stemper B, Drepper J, Griewing B, Meier U, Herschel M, Hummel K, Paar WD, Dintslos CM, Schade-Brittinger C, Weber R, Weinar C. Klinische Studien in der Neurologie in Deutschland. Akt Neurol 2009;36:8-18
• Pinkhardt EH, Kassubek J, Brummer D, Kölch M, Ludolph AC, Fegert M,  Ludolph AG. Intensified testing for attention-deficit hyperactivity disorder (ADHD) in girls should reduce depression and smoking in adult females and the prevalence of ADHD in the longterm. Medical Hypotheses 2009; 72:409-412
• Ludolph AC, Kassubek J, Landwehrmeyer BG, Burn DJ, Caparros-Lefebvre D, Garcia de Yebenes J, Gasser T, Heutink P, Höglinger G, Jamrozik Z, Jellinger KA, Kazantsev A, Kretzschmar H, Lang AE, Litvan I, Lucas J, Mandelkow E, Mandelkow EM, McGeer PL, Melquist S, Oertel W, Otto M, Paviour D, Reum T, Saint-Raymond A, Schellenberg GD, Steele JC, Tolnay M, Tumani H, van Swieten JC, Vanier MT, Vonsattel JP, Wagner S, Wszolek ZK. Tauopathies with parkinsonism: clinical spectrum, neuropathological basis, biological markers and treatment options. Eur J Neurol 2009;16:297-309
• Müller HP, Unrath A, Riecker A, Pinkhardt EH, Ludolph AC, Kassubek J. Intersubject variability in the analysis of diffusion tensor images at the group level: fractional anisotropy mapping and fiber tracking techniques. Magnetic Resonance Imaging 2009;27:324-334
• Pinkhardt EH, Sperfeld AD, Gdynia HJ, Ludolph AC, Kassubek J. The combination of dopa-responsive parkinsonian syndrome and motor neuron disease. Neurodegener Dis 2009,6:95-101
• Shoichet A, Waibel S, , Endruhn S, Sperfeld AD, Vorwerk B, Müller I, Erdogan F, Ludolph AC, Ropers HH, Ullmann R. Identification of candidate genes for sporadic amyotrophic lateral sclerosis by array comparative genomic hybridisation. Amyotroph Lateral Scler 2009; 10:162-169
• Kraft E, Loichinger W, Diepers M, Lule D, Schwarz J, Ludolph AC, Storch A. Levodopa-induced strial activation in Parkinson´s disease: A functional MRI study. Parkinsonism Relat Disord 2009;15:558-563

• Janowski M, Kuzma-Kozakiewicz M, Binder D, Habisch HJ, Habich A, Lukomska B, Domanska-Janik K, Ludolph AC, Storch A. Neurotransplantation in mice: The concorde-like position ensures minimal cell leakage and widespread distribution of cells transplanted into cisterna magna. Neurosci Lett 2008;430:169-174
• Gdynia HJ, Müller T, Sperfeld AD, Kühnlein P, Otto M, Kassubek J, Ludolph AC. Severe sensorimotor neuropathy after intake of highest dosages of vitamin B6. Neuromuscular Disorders 2008;18:156-158
• Unrath A, Klose U, Grodd W, Ludolph AC, Kassubek J. Directional colour encoding of the human thalamus by diffusion tensor imaging. Neurosci Lett 2008,434:322-327
• Storch A, Vladimirtsev VA, Tumani H, Wellinghausen N, Haas A, Krivoshapkin VG, Ludolph AC. Viliuisk Encephalomyelitis in Northeastern Siberia is not caused by Borrelia burgdorferi infection. Neurol Sci 2008;29:11-14
• Gdynia HJ, Kühnlein P, Ludolph AC, Huber R. Connective tissue disorders in dissections of the carotid or vertebral arteries. J Clin Neurosci 2008;15:489-494
• Kühnlein P, Kübler A, Raubold S, Worrell M, Kurt A, Gdynia HJ, Sperfeld AD, Ludolph AC. Palliative care and circumstances of dying in German ALS patients using non-invasive ventilation. Amyotroph Lateral Sc 2008;9:91-98
• Pinkhardt EH, Sperfeld AD, Bretschneider V, Unrath A, Ludolph AC, Kassubek J. Is spinal epidural lipomatosis an MRI-based diagnosis with clinical implications? A retrospective analysis. Acta Neurol Scand 2008;117:409-414
• Pinkhardt EH, Jürgens R, Becker W, Mölle M, Born J, Ludolph AC, Schreiber H. Signs of impaired selective attention in patients with amyotrophic lateral sclerosis. J Neurol 2008;255:532-538
• Lule D, Ludolph AC, Ludolph AG. Neurodevelopmental and neurodegenerative diseases – Is there a pathophysiological link? Attention-deficit/hyperactivity disorder and amyotrophic lateral sclerosis as examples. Med Hypotheses 2008;70:1133-1138
• Kühnlein P, Sperfeld AD, Endruhn S, Varon R, Ludolph AC, Hübner C. Sporadic ALS with early-onset respiratory failure is not associated with IGHMBP2 gene mutations. J Neurol Neurosurg Psych 2008;79:737-738
• Münch C, Zhu B, Mink A, Seefried U, Riepe MW, Ludolph AC, Meyer T. Chemical hypoxia facilitates alternative splicing of EAAT2 in presymptomatic APP23 transgenic mice. Neurochem Res 2008;33:1005-1010
• Kühnlein P, Gdynia HJ, Sperfeld AD, Lindner-Pfleghar B, Ludolph AC, Prosiegel M, Riecker A. Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis. Nat Clin Pract Neurol 2008;4:366-74
• Lule D, Häcker S, Ludolph AC, Birbaumer N, Kübler A. Depression und Lebensqualität bei Patienten mit amyotropher Lateralsklerose. Dtsch Ärzteblatt 2008,105:397-403
• Ludolph A, Pinkhardt EH, van Elst LT, Libal G, Ludolph AC, Fegert JM, Kassubek J. Are amygdalar volume alterations in children with Tourette syndrome due to ADHD comorbidity? Dev Med Child Neurol 2008;50:524-529
• Tumani H, Teunissen C, Süssmuth S, Otto M, Ludolph AC, Brettschneider J. Cerebrospinal fluid biomarkers of neurodegeneration in chronic neurological diseases. Expert Rev Mol Diagn 2008;8:479-494
• Unrath A, Müller HP, Ludolph AC, Riecker A, Kassubek J. Cerebral white mater alterations in idiopathic restless legs syndrome, as measured by diffusion tensor imaging. Mov Disord 2008;23:1250-1255
• Süssmuth S, Brettschneider J, Ludolph AC, Tumani H. Biochemical markers in CSF of ALS patients. Curr Med Chem 2008;15:1788-1801
• Brettschneider J, Sperfeld A, Ludolph AC, Kassubek J. Intracerebral hemorrhage in a patient with glycogenesis type II (pompe disease): is there a pathophysiological relationship? Muscle Nerve 2008;38:1211-1212
• Grosskreutz J, Peschel T, Unrath A, Dengler R, Ludolph AC, Kassubek J. Whole brain-based computerized neuroimaging in ALS and other motor neuron disorders. Amyotroph Lateral Sc 2008;9:238-248
• Kühnlein P, Sperfeld AD, Vanmassenhove B, Van Deerlin V, Lee VM, Trojanowski JQ, Kretzschmar HA, Ludolph AC, Neumann M. Two German kindreds with familial amyotrophic lateral sclerosis due to TARDBP mutations. Arch Neurol 2008;65:1185-89
• Gdynia HJ, Osterfeld N, Dorst J, Ludolph AC, von Arnim CA, Sperfeld AD. Granulomatous myositis mimicking slowly progressive lower motor neuron disease. Eur J Med Res 2008;13:330-331
• Brettschneider J, Mogel H, Lehmensiek V, Ahlert T, Süssmuth S, Ludolph AC, Tumani H. Proteome analysis of cerebrospinal fluid in amyotrophic lateral sclerosis (ALS). Neurochem Res 2008;33:2358-63
• Brettschneider J, Kurent J, Ludolph AC, Mitchell JD. Drug therapy for pain in amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database Syst Rev. 2008;(3):CD005226
• Steinacker P, Hendrich C, Sperfeld AD, Jesse S, Lehnert S, Pabst A, von Arnim C, Uttner I, Tumani H, Lee VM, Trojanowski JQ, Kretzschmar HA, Ludolph AC, Neumann M, Otto M. TDP-43 in cerebrospinal fluid of patients with frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Arch Neurol 2008;65:1481-1487
• Yilmaz A, Gdynia HJ, Baccouche H, Mahrholdt H, Meinhardt G, Basso C, Thiene G, Sperfeld AD, Ludolph AC, Sechtem U. Cardiac involvement in patients with Becker muscular dystrophy: new diagnostic and pathophysiological insights by a CMR approach. J Cardiovasc Magn Reson 2008;10:50
• Habisch HJ, Fiedler J, Ludolph AC, Storch A, Brenner RE. Altered migration and adhesion potential of pro-neurally converted human bone marrow stromal cells. Cytotherapy 2008;10:824-33
• Pinkhardt EH, Jürgens R, Becker W, Valdarno F, Ludolph AC, Kassubek J. Differential diagnostic value of eye movement recording in PSP-Parkinsonism, Richardson s syndrome, and idiopathic Parkinson s disease. J Neurol 2008; 255(12):1916-1925

2007

• Gdynia HJ, Sperfeld AD, Ludolph AC. Neuronale Zeroidlipofuszinosen des Erwachsenenalters. Nervenarzt 2007;78:139-144
• Niessen HG, Debska-Vielhaber G, Sander K, Angenstein F, Ludolph AC, Hilfert L, Willker W, Leibfritz D, Heinze HJ, Kunz WS, Vielhaber S. Metabolic progression markers of neurodegeneration in the transgenic G93A-SOD1 mouse model of amyotrophic lateral sclerosis. Eur J Neurosci 2007; 25:1669-1677
• Müller HP, Unrath A, Ludolph AC, Kassubek J. Preservation of diffusion tensor properties during spatial normalization by use of tensor imaging and fibre tracking on a normal brain database. Phys Med Biol 2007;52:N99-N109
• Brettschneider J, Wild K, Schattauer D, Ludolph AC, Tumani H. Cerebrospinal fluid erythropoietin (EPO) in amyotrophic lateral sclerosis. Neurosci Lett 2007;416:257-260
• Lulé D, Diekmann V, Anders S, Kassubek J, Kuebler A, Ludolph AC, Birbaumer N. Brain responses to emotional stimuli in patients with amyotrophic lateral sclerosis. J Neurol 2007;254:519-527
• Dorst J, Kuehnlein P, Sperfeld AD, Ludolph AC. Therapieoptionen bei der Amyotrophen Lateralsklerose (ALS). Nervenheilkunde 2007; 5:362-367
• Hirschfeld G, Berneburg M, Arnim von C, Iben S, Ludolph AC, Scharffetter-Kochanek K. Progeroid-Syndrome. Klinik und Molekularbiologie der vorzeitigen Alterung. Dtsch. Ärzteblatt 2007;6,246-353
• Gdynia HJ, Sperfeld AD, Flaith L, Kuehnlein P, Unrath A, Ludolph AC, Kassubek J. Classification of phenotype characteristics in adult-onset spinal muscular atrophy. Eur Neurol 2007;58:170-176
• Hendrich C, Gastl R, Ludolph AC. Neurotoxizität des Alkohols. Welche klinisch wichtigen Effekte sind gesichert? InFo NP 2007;9:28-32
• Bengel D, Susa M, Schreiber H, Ludolph AC, Tumani H. Early diagnosis of rhinocerebral mucormycosis by cerebrospinal fluid analysis and determination of 16s rRNA gene sequence. Eur J Neurol 2007;14:1067-1070
• Ludolph AC, Bendotti C, Blaugrund E, Hengerer B, Löffler JP, Martin J, Meininger V, Meyer T, Moussaoui S, Robberecht W, Scott S, Silani V, van d. Berg L, (ENMC Group for the establishment of guidelines for the conduct of preclinical and proof of concept studies in ALS/MND models). Guidelines for the preclinical in vivo evaluation of pharmacological active drugs for ALS/MND: Report on the 142^nd ENMC international workshop. Amyotroph Lateral Sc 2007;8(4):217-23
• Münch C, Meyer R, Linke P, Meyer T, Ludolph AC, Haas J, Hemmer B, The p 150 subunit of dynactin (DCTN1) gene in multiple sclerosis. Acta Neurol Scand 2007;116(4):231-4
• Unrath A, Ludolph AC, Kassubek J. Brain metabolites in definite amyotrophic lateral sclerosis. A longitudinal proton magnetic resonance spectroscopy study. J Neurol. 2007;254(8):1099-106
• Gastl R, Ludolph AC. Genußgift Alkohol. Folgen für das Nervensystem. Der Allgemeinarzt 2007; 1(29):42-44
• Baranczyk-Kuzma A, Usarek E, Kuzma-Kozakiewicz M, Kazmierczak B, Gajewska B, Schwalenstoecker B, Ludolph AC. Age-related changes in tau expression in transgenic mouse model of amyotrophic lateral sclerosis. Neurochem Res 2007;32(3):415-21
• Habisch HJ, Schwalenstocker B, Danzeisen R, Neuhaus O, Hartung HP, Ludolph AC. Limited effects of glatiramer acetate in the high-copy number hSOD1-G93A mouse model of ALS. Exp Neurol. 2007;206(2):288-95
• Widl K, Brettschneider J, Schattauer D, Süßmuth S, Huber R, Ludolph AC, Tumani H. Erythropoietin in cerebrospinal fluid: age-related reference values and relevance in neurological disease. Neurochem Res 2007;32:1163-1168
• Lule D, Diekmann V, Kassubek J, Kurt A, Birbaumer N, Ludolph AC, Kraft E. Cortical plasticity in amyotrophic lateral sclerosis: Motor imagery and function. Neurorehabil Neural Repair 2007;21:518-526
• Bucher S, Braunstein KE, Niessen HG, Kaulisch T, Neumaier M, Stiller D, Boeckers TM, Ludolph AC. Vacuolisation correlates with spin-spin relaxation time in motor brainstem nuclei and behavioural test in the transgenic G93A-SOD1 mouse model of ALS. Eur J Neurosci 2007;26:1895-1901
• Landwehrmeyer B, Dubois B, Garcia de Yebenes J, Kremer B, Gaus W, Kraus PH, Przuntek H, Dib M, Doble A, Fischer W, Ludolph AC, EHDI Study Group. Riluzole in Huntington´s disease: a three year, randomised controlled study. Ann Neurol 2007;62:262-72
• Kassubek J, Jüngling FD, Baumgartner A, Unrath A, Ludolph AC, Sperfeld AD. Different regional brain volume loss in pure and complicated hereditary spastic paraparesis: A voxel-based morphometric study. Amyotroph Lateral Sc 2007;8:328-336
• Groeneveld GJ, Graf M, Van Der Tweel I, van den Berg LH, Ludolph AC. Alternative trial design in amyotrophic lateral sclerosis saves time and patients. Amyotroph Lateral Sc 2007;8:266-269
• Habisch HJ, Janowski M, Binder D, Kuzma-Kozakiewicz A, Widmann A, Habich A, Schwalenstöcker B, Hermann A, Brenner R, Lukomska B, Domanska-Janik K, Ludolph AC, Storch A. Intrathecal application of neuroectodermally converted stem cells into a mouse model of ALS: Limited intraparenchymal migration and survival narrows therapeutic effects. J Neural Transm 2007;114:1395-406
• Gastl R, Ludolph AC. Amyotrophe Lateralsklerose. Nervenarzt 2007;12:1449-1457
• Mueller HP, Unrath A, Sperfeld AD, Ludolph AC, Riecker A, Kassubek J. Diffusion tensor imaging and tractwise fractional anisotropy statistics: quantitative analysis in white matter pathology. Biomed Eng Online 2007; 6(1):42

 2006

• Danzeisen R, Schwalenstoecker B, Gillardon F, Buerger E, Krzykalla V, Klinder K, Schild L, Hengerer B, Ludolph AC, Dorner-Ciossek C, Kussmaul L. Targeted antioxidative and neuroprotective properties of the dopamine agonist pramipexole and its non-dopaminergic enantiomer SND919CL2x. J Pharmacol Exp Ther 2006;316:189-199
• Meininger V, Asselain B, Guillet P, Leigh PN, Ludolph AC, Lacomblez L, Robberecht W. Pentoxifylline in ALS. A double-blind, randomised, multicenter, placebo-controlled trial. Neurology 2006;66:88-92
• Gizatullina ZZ, Lindenberg KS, Harjes P, Chen Y, Kosinski CM, Landwehrmeyer BG, Ludolph AC, Striggow F, Zierz S, Gellerich FN. Low stability of huntington muscle mitochondria against Ca (2+) in R6/2 mice. Ann Neurol 2006;59:407-411
• Teuchert M, Fischer D, Schwalenstoecker B, Habisch HJ, Boeckers T, Ludolph AC. A dynein mutation attenuates motor neuron degeneration in SOD1^(G93A) mice. Exp Neurol 2006;198:271-274
• Brettschneider J, Petzold A, Süßmuth SD, Ludolph AC, Tumani H. Axonal damage markers in cerebrospinal fluid are increased in ALS. Neurology 2006;66:852-856
• Uttner I, Mottaghy FM, Schreiber H, Riecker A, Ludolph AC, Kassubek J. Primary progressive aphasia accompanied by environmental sound agnosia: A neuropsychological, MRI and PET study. NeuroImaging 2006;146:191-197
• Ludolph AG, Juengling FD, Libal G, Ludolph AC, Fegert JM, Kassubek J. Grey-matter abnormalities in boys with Tourette syndrome: magnetic resonance imaging study using optimised voxel-based morphometry. British J Psychiatry 2006; 188:484-485
• Roettig D, Leplow B, Eger K, Ludolph AC, Graf M, Zierz S. Only subtle cognitive deficits in non-bulbar amyotrophic lateral sclerosis patients. J Neurol 2006; 253:333-339
• Danzeisen R, Achsel T, Bederke U, Cozzolino M, Crosio C, Ferri A, Frenzel M, Gralla EB, Huber L, Ludolph AC, Nencini M, Rotilio G, Valentie JS, Carri MT. Superoxide dismutase 1 modulates expression of transferrin receptor. J Biol Inorg Chem 2006;11:489-498
• Odin P, Oehlwein C, Storch A, Polzer U, Werner G, Renner R, Shing M, Ludolph AC, Schüler P. Efficacy and safety of high-dose cabergoline in Parkinson´s disease. Acta Neurol Scand 2006;113:18-24
• Niessen HG, Angenstein F, Sander K, Kunz WS, Teuchert M, Ludolph AC, Heinze H-J, Scheich H, Vielhaber S. In vivo quantification of spinal and bulbar motor neuron degeneration in the G93A-SOD1 transgenic mouse model of ALS by T₂ relaxation time and apparent diffusion coefficient. Exp Neurol 2006;201:293-300
• Usarek E, Kuzma-Kozakiewcz M, Schwalenstöcker B, Münch Ch, Kazmierczak B, Ludolph AC, Baranczyk-Kuzma A. Tau isoforms expression in transgenic mouse model of amyotrophic lateral sclerosis. Neurochem Res 2006; 31:597-602
• Gdynia HJ, Kurt A, Endruhn S, Ludolph AC, Sperfeld AD. Cardiomyopathy in motor neuron diseases. J Neurol Neurosurg Psychiatry 2006;188:484-5
• Ludolph AC. Workshop Report. 135^th ENMC interantional Workshop: Nutrition in Amyotrophic Lateral Sclerosis. 18-20 of March 2005, Naarden, The Netherlands. Neuromuscular Disord 2006;16:530-538
• Ludolph AC. Matrix metalloproteinases – A conceptional alternative for disease-modifying strategies in ALS/MND? Exp Neurol 2006;201:277-280
• Kühnlein P, Sperfeld AD, Ludolph AC, Huber R. Respiratorische Insuffizienz bei neuromuskulären Erkrankungen. Extracta psychiatrica/neurologica 2006;1 / 2:15-18
• Fernández-Santiago R, Sharma M, Mueller JC, Gohlke H, Illig T, Anneser J, Münch C, Ludolph AC, Kamm C, Gasser T. Possible genderdependent association of vascular endothelial growth factor (VEGF) gene and ALS. Neurology 2006;66:1929-1931
• Gdynia H-J, Kühnlein P, Ludolph AC, Sperfeld AD. Hyperexzitabilitätssyndrome peripherer Nerven: Varianten, Differenzialdiagnosen und Therapie anhand eines Fallbeispieles. Akt Neurol 2006;33:560-563
• Brettschneider J, Petzold A, Süßmuth S. Landwehrmeyer B, Ludolph AC, Kassubek J, Tumani H: The neurofilament heavy chain NfH^SMI35 in cerebrospinal fluid supports the differential diagnosis of Parkinsonian syndromes. Mov Disord 2006;21:2224-2227
• Pinkhardt EH, van Elst LT, Ludolph AC, Kassubek J. Amygdala size in amyotrophic lateral sclerosis without dementia: an in vivo study using MRI volumetry. BMC Neurology 2006;6:1-6
• Gdynia HJ, Sperfeld AD, Knirsch U, Homberg V, Rosenbohm A, Müller-Hocker J, Ludolph AC, Hanemann CO. Benign symmetric lipomatosis with axonal neuropathy and abnormalities in specific mitochondrial tRNA regions. Eur J Med Res 2006;11:545-6

2005

• Graf M, Ecker D, Horowski R, Kramer B, Riederer P, Gerlach M, Hager C, Ludolph AC, Becker G, Osterhage J, Jost WH, Schrank B, Stein C, Kostopulos P, Lubik S, Wekwerth K, Dengler R, Troeger M, Wuerz A, Hoge A, Schrader C, Schimke N, Krampfl K, Petri S, Zierz S, Eger K, Neudecker S, Traufeller K, Sievert M, Neundoerfer B, Hecht M High dose vitamin E therapy in amyotrophic lateral sclerosis as add-on therapy to riluzole: results of a placebo-controlled double-blind study. J Neural Transm 2005;112(5):649-660
• Ecker D, Geisthoff UW, Juchems M, Schneider G, Ludolph AC, Kassubek J, Huber R. Hereditary hemorrhagic telangiectasia A rare treatable cause of stroke. Nervenarzt 2005;76(8):980-983
• Schreiber H, Gaigalat T, Wiedemuth-Catrinescu U, Graf M, Uttner I, Muche R, Ludolph AC. Cognitive function in bulbar- and spinal-onset amyotrophic lateral sclerosis. A longitudinal study in 52 patients. J Neurol 2005;252(7):772-81
• Sperfeld AD, Hanemann CO, Ludolph AC, Kassubek J. Laryngospasm: an underdiagnosed symptom of X-linked spinobulbar muscular atrophy. Neurology 2005; 64(4):753-754
• Sperfeld AD, Bretschneider , Flaith L, Unrath A, Hanemann OC, Ludolph AC, Kassubek J. MR-pathologic comparison of the upper spinal cord in different motor neuron diseases. Eur Neurol 2005; 53(2):74-77
• Kuner R, Groom A, Bresink I, Müller G, Kornau H-C, Hartmann B, Stefovska V, Ikonomidou C, Tschauner C, Waibel S, Ludolph AC, Seeburg PH, Turski L. Late-onset motoneuron disease caused by a functionally modified AMPA receptor subunit. Proc Natl Acad Sci USA 2005;102(16):5826-31
• Spencer PS, Palmer VS, Ludolph AC. On the decline and etiology of high-incidence motor system disease in West Papua (Southwest New Guinea). Mov Disord 2005;20 (12):119-126
• Münch C, Rosenbohm A, Sperfeld AD, Uttner I, Reske S, Krause BJ, Sedlmeier R, Meyer T, Hanemann CO, Stumm G, Ludolph AC. Heterozygous R1101K mutation of the DCTNI gene in a family with ALS and FTD. Ann Neurol 2005;58:777-780
• Lule D, Kurt A, Jürgens R, Kassubek J, Diekmann V, Kraft E, Neumann N, Ludolph AC, Birbaumer N, Anders S. Emotional responding in amyotrophic lateral sclerosis. J Neurol 2005;252:1517-1524
• Alber B, Pernauer M, Schwan A, Rothmund G, Brummer D, Sperfeld AD, Uttner I, Binder H, Epplen JT, Ludolph AC, Meyer T, Dullinger J. Spastin related hereditary spastic paraplegia with dysplastic corpus callosum. J Neurol Sci 2005;236:9-12
• Kress JA, Kühnlein P, Winter P, Ludolph AC, Kassubek J, Müller U, Sperfeld AD. Novel mutation in the ALS2 gene in juvenile amyotrophic lateral sclerosis. Ann Neurol 2005;58:800-803
• Muench C, Precher F, Renshi X, Linke P, Prudlo J, Kuzma M, Kwiecinski H, Ludolph AC, Meyer T. Frequency of a tau genotype in amyotrophic lateral sclerosis. J Neurol Sci 2005;236(1-2):13-16
• Kuebler A, Winter S, Ludolph AC, Hautzinger M, Birbaumer N. Severity of Depressive Symptoms and Quality of Life in Patients with Amyotrophic Lateral Sclerosis. Neurorehabilitation and Neural Repair 2005;19:182-193
• Müller HP, Decesaris I, Demelis M, Marzetti L, Pasquarelli A, Erné SN, Ludolph AC, Kassubek J. Open Magnetic and Electric Graphic Analysis. IEEE Eng Med Biol 2005,24 (3):109-16
• Kuner R, Groom AJ, Müller G, Kornau H-CH, Stefovska V, Bresink I, Hartmann B, Tschauner K, Waibel S, Ludolph AC, Ikonomidou Ch, Seeburg PH, Turski L. Mechanisms of Disease: Motoneuron Disease Aggravated by Transgenic Expression of a Funktionally Modified AMPA Receptor Subunit. Ann N Y Acad Sci 2005;1053:269-286
• Hanemann CO, Ludolph AC. Motor protein diseases of the nervous system. Amyotrophic Lateral Sclerosis 2005;6:197-201
• Kassubek J, Unrath A, Huppertz HJ, Lule D, Ethofer T, Sperfeld AD, Ludolph AC. Global brain atrophy and corticospinal tract alterations in ALS, as investigated by voxel-based morphometry of 3-D MRI. Amyotrophic Lateral Sclerosis 2005;6:213-220
• Henkel K, Ludolph AC, Huber R. Teil X: Akuter Thiaminmangel. Extracta psychiatrica/neurologica 2005;12:5-6
• Huber R, Ludolph AC. Teil XI: Zentrale pontine Myelinolyse. Extracta psychiatrica/neurologica 2005 12: 8-9
• Ludolph AC, Sperfeld AD. Preclinical Trials – An Update on Translational Research in ALS. Neurodegen Dis 2005;2:215-219

2004

• Völkel H, Selzle M, Walk T, Jung G, Link J, Ludolph AC, Reuter A. Reduced reactivation rate in mutant CuZnSOD and progression rate of amyotrophic lateral sclerosis. Eur J Neurol 2004;11:397-404
• Kurt A, Ludolph AC. Warum eine gute Betreuung so wichtig ist. DNP 4: 48-50
• Leigh N, Swash M, Iwasaki Y, Ludolph AC, Meininger V, Miller RG, Mitsumoto H, Shaw P, Tashiro K, Van Den Berg L (2004): Amyotrophic lateral sclerosis – a consensus viewpoint on designing and implementing clinical trials. Amyotroph Lateral Sc 2004;3: 84-98
• Kassubek J, Jüngling FD, Kioschies T, Henkel K, Karitzky J, Kramer B, Ecker D, Andrich J, Saft, C, Kraus P, Aschoff AJ, Ludolph AC, Landwehrmeyer GB. Topography of cerebral atrophy in early Huntington’s disease: a voxel-based morphometric MRI-study. J Neurol Neurosurg Psych 2004;75 (2):213-220
• Prudlo J, Alber B, Kalscheuer V, Roemer K, Martin T, Dullinger J, Sittinger H, Niemann S, Heutink P, Ludolph AC, Ropers HH, Zang K, Meyer T. Chromosomal translocation t (18;21) (q23; q22.1) indicates novel susceptibility loci for frontotemporal dementia with ALS. Ann Neurol 2004;55:134-138
• Sperfeld AD, Kassubek J, Ludolph AC. Current aspects in diagnostics and therapy of amyotrophic lateral sclerosis. Aktuelle Neurologie 2004;31:209-215
• Münch C, Sedlmeier R, Meyer T, Homberg V, Sperfeld AD, Kurt A, Prudlo J, Peraus G, Hanemann CO, Stumm G, Ludolph AC. Point mutations of the p150 subunit of dynactin (DCTN1) gene in amyotrophic lateral sclerosis, Neurology 2004; 63(4):724-726
• Henkel K, Karitzky J, Schmid M, Mader I, Glatting G, Unger JW, Neumaier B, Ludolph AC, Reske SN, Landwehrmeyer GB. Imaging of activated microglia with PET and [11C] PK 11195 in corticobasal degeneration. Mov Disord 2004;19(7):817-821
• Waibel S, Reuter A, Malessa S, Blaugrund E, Ludolph AC. Rasagiline alone and in combination with riluzole prolongs survival in an ALS mouse model. J Neurol 2004;251(9):1080-1084
• Angenstein F, Niessen HG, Goldschmidt J, Vielhaber S, Ludolph AC, Scheich H. Age-dependent changes in MRI of motor brain stem nuclei in a mouse model of ALS. Neuroreport 2004;15(14):2271-2274
• Storch A, Ludolph AC, Schwarz J. Dopamine transporter: involvement in selective dopaminergic neurotoxicity and degeneration. J Neural Transm 2004;111(10-11):1267-1286
• Sperfeld AD, Kassubek J, Crosby AH, Winner B, Ludolph AC, Uttner I, Hanemann CO. Complicated hereditary spastic paraplegia with thin corpus callosum: Variation of phenotypic expression over time. J Neurol 2004;251(10):1285-1287
• Brettschneider J, Riepe MW, Petereit HF, Ludolph AC, Tumani H. Meningeal derived cerebrospinal fluid proteins in different forms of dementia: is a meningopathy involved in normal pressure hydrocephalus? J Neurol Neurosurg Psych 2004;75(11):1614-1616
• Kaufmann E, Boehm B, Süssmuth SD, Kientsch-Engel R, Sperfeld A, Ludolph AC, Tumani H. The advanced glycation end-product N epsilon-(carboxymethyl)lysine level is elevated in cerebrospinal fluid of patients with amyotrophic lateral sclerosis. Neurosci Lett 2004;371(2-3):226-229

2003

• Meyer T, Alber B, Roemer K, Martin T, Kalscheuer VM, Göttert E, Zang KD, Ludolph AC, Ropers HH, Prudlo J. High rate of constitutional chromosomal rearrangements in apparently sporadic ALS. Neurology 2003;60(8):1348-1350
• Kassubek J, Jüngling FD, Hoffmann S, Rosenbohm A, Kurt A, Jurkat-Rott K, Steinbach P, Wolf M, Ludolph AC, Lehmann-Horn F, Lerche H, Weber YG. Quantification of brain atrophy in patients with myotonic dystrophy and proximal myotonic myopathy: a controlled 3-dimensional magnetic resonance imaging study. Neurosci Lett 2003;348:73-76
• Butz M, Wollinsky KH, Wiedemuth-Catrinescu U, Sperfeld A, Winter S, Mehrkens HH, Ludolph AC, Schreiber H. Longitudinal effects of noninvasive positive pressure ventilation in patients with ALS. J Phys Med Rehabil 2003;82 (8):597-604
• Bachus R, Neubert K, Brockmöller J, Ludolph AC, Roots I. Polymorphisms of toxifying and detoxifying hepatic enzymes in amyotrophic lateral sclerosis. J Neurol Neurosurgery Psychiatry 2003;74 (8): 1161
• Angelov DN, Waibel S, Guntinas-Lichius O, Lenzen M, Neiss WF, Tomov TL, Yoles E, Kipnis J, Reuter A, Ludolph AC, Schwartz M. Therapeutic vaccine for acute and chronic motor neuron diseases : Implications for ALS. PNAS 2003;100 (8): 4790-4795
• Hanemann CO, Ludolph AC. Meeting Report of the ALS/MND Meeting of the European ALS/MND group on Reisensburg Castle, January 30 to February 1, 2003. Amyotrophic Lateral Sclerosis and other Motor Neuron Disorders 2003;4 (2):121-122
• Kassubek J, Tumani H, Ecker D, Kurt A, Ludolph AC, Jüngling F. Age-related brain parenchymal fraction is significantly decrease in young multiple sclerosis patients: A quantitative MRI study. Neuroreport 2003;14 (3):427-430
• Ludolph AC. Kommentar zur Arbeit von Veldink JH,Wokke JHJ, van der Wal G, Vianney de Jong JM, van den Berg LH: Euthanasie bei amyotropher Lateralsklerose – Wie dem Todeswunsch begegnen? InFo Neurologie 2003; 4 (5):16
• Storch A, Ludolph AC. Neuroprotektion: Mechanismen und derzeitige klinische Therapiestategien. Die Medizinische Welt 2003;54:300-305
• Ethofer T, Mader I, Seger U, Helms G, Erb M, Grodd W, Ludolph AC, Klose U. Comparison of longitudinal metabolite relaxation times in different regions of the human brain at 1.5 and 3 Tesla. Magnet Reson Med 2003;50:1296-1301
• Süssmuth SD, Tumani H, Ecker D, Ludolph AC. Amyotrophic lateral sclerosis: disease stage related changes of tau protein and S100 beta in cerebrospinal fluid and creatine kinase in serum. Neurosci Lett 2003;353:57-60
• Münch C, Zhu BG, Leven A, Stamm S, Einkörn H, Schwalenstöcker B, Ludolph AC, Riepe MW, Meyer T. Differential regulation of 5´ splice variants of the glutamate transporter EAAT2 in an in vivo model of chemical hypoxia induced by 3-Nitropropionic acid. J Neurosci Res 2003;71:819-825
• Hartung HP, Ludolph AC. Neuroprotektion bei der MS – Utopie oder Wirklichkeit? Nervenarzt 2003;74 (11):1-6

2002

• Sperfeld AD, Hein C, Schröder JM, Ludolph AC, Hanemann CO. I und characterization of peripheral nerve involvement in neurofibromatosis type 2. Brain 2002;125: 996-1004
• Hanemann CO, Ludolph AC. Hereditary motor neuropathies and motor neuron diseases: Which is which? Amyotrophic Lateral Sclerosis and Other Motor Neuron Disord 2002;4:186-189
• Tumani H, Windl O, Kretzschmar HA, Ludolph AC. Die klinisch atypische CJK – Diagnostische Bedeutung von Liquormarkern und molekulargenetischen Untersuchungen? Deutsche Medizinische Wochenschrift 2002;127:318-320
• Münch C, Zhu BG, Schwalenstöcker B, Seefried U, Ebstein M, Leven M, Landwehrmeyer GB, Ludolph AC, Meyer T. Alternative splicing of the 5’sequences of the mouse EAAT2 glutamate transporter and expression in a transgenic model for amyotrophic lateral sclerosis. J Neurochem 2002;82:594-603
• Huber R, Aschoff AJ, Ludolph AC, Riepe MW. Transient global amnesia – evidence against vascular ischemic etiology from diffusion weighted imaging. J Neurol 2002;249(11):1520-1524
• Ludolph AC. Measures of Quality of Life: Pro. ALS and other motor neuron disord 2002;Suppl 1,S17
• Jonsson PA, Bäckstrand A, Andersen PM, Jakobsson J, Parton M, Shaw CE, Swingler R, Shaw PJ, Robberecht W, Ludolph AC, Siddique T, Skvortsova V, Marklund SL. CuZn-superoxide dismutase in D90A heterozygotes from recessive and dominant ALS pedigrees. Neurobiol Dis 2002;10 (3):327-333
• Meyer T, Ludolph AC, Münch C. Ifosfamide encephalopathy presenting with asterixis. J Neurol Sci 2002;199: 85-88
• Mader I, Karitzky J, Klose U, Seeger U, Sperfeld A, Naegele T, Schick F, Ludolph AC, Grodd W. Proton MRS in Kennedy disease: Absolute metabolite and macromolecular concentrations. J Mag Res Imaging 2002;16 (2):160-167

2001

• Münch C, Ludolph AC. Pharmacological treatment of ALS. Neurol Neurochir Pol 2001;1:41-50
• Reuter A, Mi J, Sehrsam I, Ludolph AC, Völkel H. A novel calcineurin splice variant that modifies calcineurin activity. Eur J Biochem 2001;268: 5955-5960
• Karitzky, J, Ludolph AC. Imaging and Neurochemical Markers for Diagnosis and Disease Progression in ALS. J Neurol Sci 2001;191: 35-41
• Münch C, Penndorf A, Schwalenstöcker B, Troost D, Ludolph AC, Ince P, Meyer T. Impaired RNA splicing of 5’-regulatory sequences of the astroglial glutmate transporter EAAT2 in human astrocytoma. J Neurol Neurosurg Psychiatry 2001;71 (5):675-8.
• Lerche H, Weber YG, Baier H, Jurkat-Rott K, Kraus de Camargo O, Ludolph AC, Bode H, Lehmann-Horn F. Generalized epilepsy with febrile seizures plus: further eterogeneity in a large family. Neurology 2001;57:1191-1198
• Völkel H, Scholz M, Link J, Selzle M, Werner P, Tunnemann R, Jung G, Sehrsam, I, Ludolph AC, Reuter A. Superoxide dismutase mutations of amyotrophic lateral sclerosis and the oxidative inactivation of calcineurin. FEBS Letters 2001;503 (2-3):201-205
• Arnim von CAF, Ludolph AC. Normal and pathological aging of the brain. Fortschr Med Orig 2001;118 (4): 165-168
• Kasischke K, Büchner M, Ludolph AC, Riepe MW. Nuclear shrinkage in live mouse hippocampal slices. Acta Neuropathol 2001;101, 483-490
• Ludolph AC, Storch A, Sperfeld A, Collatz M. Tauopathien – eine neue Kategorie degenerativer Hirnerkrankungen. Nervenarzt 2001;72:78-85
• Arnim von CAF, Timmler M, Ludolph AC, Riepe MW. Chemical preconditioning in mice is not mediated by upregulation of nitrite oxide synthase isoforms. Neurosci Lett 2001;299: 130-134
• Knirsch U, Sturm S, Reuter A, Bachus R, Gosztonyi G, Voelkel H, Ludolph AC. Calcineurin A- and calbindin immunoreactivity in the spinal cord of G93A superoxide dismutase transgenic mice. Brain Res 2001;889: 234-238

2000

• Ludolph AC, Meyer T, Riepe MW. The role of excitotoxicity in ALS: what is the evidence? J Neurol 2000;247: 7-16
• Ludolph AC. Treatment of amyotrophic lateral sclerosis – what is the next step? J Neurol 2000;247 (Suppl. 6), 13-18
• Knirsch U, Bachus R, Zschenderlein R, Gosztonyi G, Ludolph AC. Clinicopathological study of atypical motor neuron disease with vertical gaze palsy and ballism. Acta Neuropathol 2000;100: 342-346.
• Arnim von CAF, Timmler M, Ludolph AC, Riepe M. Adenosine receptor up-regulation: initiated upon preconditioning but not upheld. Neuroreport 2000;11: 1223-1226.
• Münch C, Schwalenstöcker B, Hermann C, Cirovic S, Stamm S,Ludolph AC, Meyer T. Differential RNA cleavage and polyadenylation of the glutamate transporter EAAT2 in the human brain. Mol Brain Res 2000;80:244-251
• Winter SM, Claus A, Oberwittler C, Völkel H, Wetzler S, Ludolph AC. Recessively inherited amyotrophic lateral sclerosis: A German f amily with the D90A Cu/Zn SOD mutation. J Neurol 2000;247: 783-6
• Gerhard A, Neumaier B, Elitok E, Glatting G, Ries V, Tomczak R, Ludolph AC, Reske SN. In vivo imaging of activated microglia using (11C) Pk 11195 and positron emission tomography after ischemic stroke. Neuroreport 2000;11: 2957-2960
• Storch A, Burkhardt K, Ludolph AC, Schwarz J. Protective effects of riluzole on dopamine neurons: involvement of oxidative stress and cellular energy metabolism. J Neurochem 2000;75:2259-2269

1999

• Röricht S, Meyer BU, Irlbacher K, Ludolph AC. Impairment of callosal and corticospinal system function in adolescents with early-treated phenylketonuria: a transcranial magnetic stimulation study. J Neurol 1999;246: 21-30
• Ullrich K, Flott-Rahmel B, Schluff P, Musshoff U, Das A, Lücke T, Steinfeld R, Christensen E, Jakobs C, Ludolph AC, Neue A, Röper R. Glutaric aciduria type I: Pathomechanisms of neurodegeneration. J Inher Metab Dis 1999;2: 392-403
• Huber R, Kasischke K, Ludolph AC, Riepe MW. Increase of cellular hypoxic tolerance by erythromycin and other antibiotics. NeuroReport 1999;10:1543-1546
• Ludolph AC. Defining the concept and consequences of neuroprotektive therapy in neurodegenerative diseases. Eur J Neurol 1999; 6 (Suppl. 6), S1-S6
• Ludolph AC, Meyer T, Riepe MW. Antiglutamate therapy of ALS – which is the next step? J Neural Transm 1999;55 (Suppl.): 79-96
• Storch A, Ludolph AC, Schwarz J. HEK-293 cells expressing the human dopamine transporter are susceptible to low concentrations of 1-methyl-4-phenylpyridine (MPP+) via impairment of energy metabolism. Neurochem Intern 1999;35: 393-403
• Ludolph AC, Münch C. Neurotoxic mechanisms of degeneration in motor neuron diseases. Drug Metab Rev 1999;31:619-634
• Ludolph AC, Knirsch U. Problems and Pitfalls in the diagnosis of ALS. J Neurol Sci 1999;165:S14 – S20
• Vielhaber S, Winkler K, Kirches E, Kunz D, Büchner M, Feistner H, Elger CE, Ludolph AC, Riepe MW, Kunz WS. Visualization of defective mitochondria in skeletal muscle fibers of patients with sporadic amyotrophic lateral sclerosis. J Neurol Sci 1999;169: 133-139
• Büchner M, Li H, Huber R, Timmler M, Sehrsam I, Kasischke K, Völkel H, Ludolph AC, Riepe MW. Impaired tolerance to repetitive hypoxia in hippocampal slices of Cu, Zn superoxide dismutase transgenic mice. Neurosci Lett 1999; 276: 131-134
• Meyer T, Fromm A, Münch C, Schwalenstöcker B, Fray AE, Ince PG, Stamm S, Grön G, Ludolph AC, Shaw PJ. The RNA of the glutamate transporter EAAT2 is variably spliced in amyotrophic lateral sclerosis and normal individuals. J Neurol Sci 1999;170: 45-50
• Sperfeld AD, Collatz MB, Baier H, Palmbach M, Storch A, Schwarz J, Tatsch K, Reske S, Joosse M, Heutink P, Ludolph AC. FTDP-17: An early-onset phenotype with parkinsonism and epileptic seizures caused by a novel mutation. Ann Neurol 1999;46: 708-715
• Karitzky J, Block W, Mellies JK, Träber F, Sperfeld A, Schild HH, Haller P, Ludolph AC. Proton magnetic resonance spectroscopy in Kennedy´s syndrome. Arch Neurol 1999;56:1465-1471

1998

• Meyer T, Münch C, Knappenberger B, Liebau S, Völkel H, Ludolph AC. Alternative splicing of the glutamate transporter EAAT2 (GLT-1). Neurosci Lett 1998;241; 1-3
• Meyer T, Münch C, Völkel H, Booms C, Ludolph AC. The EAAT2 (GLT-I) gene in motor neuron disease: Absence of mutations in amyotrophic lateral sclerosis and a point mutation in patients with hereditary spastic paraplegia. J Neurol Neurosurg Psychiatry 1998;65; 594-597
• Gasser T, Windgassen K, Bereznai B, Kabus C, Ludolph AC. Phenotypic expression of the DYT1-mutation: a family with writer´s cramp of juvenile-onset. Ann Neurol 1998;44: 126-128
• Münch C, Schwalenstöcker B, Liebau S, Völkel H, Ludolph AC, Meyer T. 5`- heterogeneity of the human glutamate transporter cDNA EAAT2 (GLT-1). Neuro Report 1998;9: 1295-1297
• Ludolph AC, Meyer T, Riepe MW, Völkel H. Amyotrophic lateral sclerosis and glutamate. Rest. Neurol Neurosci 1998;13 (1-2): 59-67

1997

• Flott-Rahmel B, Falter C, Schluff P, Fingerhut R, Christensen E, Jakobs C, Mußhoff U, Fautek JD, Deufel T, Ludolph AC, Ullrich K. Nerve cell lesions caused by 3-hydroxyglutaric acid: A possible mechanism for neurodegeneration in glutaric riluzole I. J Inher Metab Dis 1997;20: 387-390
• Harms L, Meierkord H, Timm G, Pfeiffer L, Ludolph AC. Decreased n-acetyl-aspartate and increased lactate in the frontal lobe of patients suffering from Huntington´s disease – a proton magnetic resonance study.  J Neurol Neurosurg Psychiatry 1997;62:27-31
• Bachus R, Bader S, Geßne, R, Ludolph AC. Lack of association of apolipoprotein E e4 allele with bulbar-onset motor neuron disease. Ann Neurol 1997;41, 417-418
• Riepe M, Klappenbach G, Ludolph AC. Increase of flexor reflex latency in patients with amyotrophic lateral sclerosis treated with riluzole. J Neurol Neurosurg Psychiatry 1997;427
• Riepe MW, Esclaire F, Kasischke K, Schreiber S, Nakase H, Kempski O, Ludolph AC, Dirnagl U, Hugon J. Increased hypoxic tolerance by chemical inhibition of oxidative phosphorylation: “chemical preconditioning”. J Cereb Blood Flow Metab 1997;17:257-264
• Meyer T, Ludolph AC, Morkel M, Hagemeier C, Speer A. Genomic organization of the human excitatory amino acid transporter gene GLT-1. Neuroreport 1997;8:775-777
• Riepe M, Ludolph AC. Chemical preconditioning: a cytoprotective strategy. Mol Cell Biochem 1997;174:249-254
• Bachus R, Claus A, Meyer T, Riepe M, Ludolph AC. Molekularbiologische Befunde bei der ALS. Nervenarzt 1997;68:785-791

1996

• Riepe M, Schmalzigaug K, Fink F, Oexle K, Ludolph AC. NADH in the pyramidal cell layer of hippocampal regions CA1 and CA3 upon selective inhibition and uncoupling of oxidative phosphorylation. Brain Res 1996;710:21-27
• Riepe M, Niemi WN, Megow D, Ludolph AC, Carpenter DO. Mitochondrial oxidation in rat hippocampus can be preconditioned be selective chemical inhibition of succinic dehydrogenase. Exp Neurol 1996;138, 15-21.
• Purucker M, Schröder R, Harms L, Timm G, Zierz S, Ludolph AC. Reversible focal brain edema in a patient with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) syndrome. J Epilepsy 1996;9:79-82
• Kolde G, Bachus R, Ludolph AC. Skin involvement in amyotrophic lateral sclerosis. Lancet 1996;347:1226-1227
• Ludolph AC, Vetter U, Ullrich K. Studies of multimodal evoked potentials in treated phenylketonuria – the pattern of vulnerability. Eur J Ped 1996;155 (suppl. 1):S64 – S68
• Kasischke K, Ludolph AC, Riepe MW. NMDA-antagonists reverse increased hypoxic tolerance by preceding chemical hypoxia. Neurosci Lett 1996;214:175-8
• Terro F, Lasort M, Vlader F, Ludolph AC, Hugon J. Antioxidant drugs block in vitro the neurotoxicity of CSF from patients with amyotrophic lateral sclerosis.  Neuroreport 1996;7:1970-1972
• Ludolph AC, König M, Roszinsky-Köcher G. Riluzol – ein neuer therapeutischer Ansatz bei der amyotrophen Lateralsklerose. Nervenheilkunde 1996;15:364-369
• Ludolph AC, Spencer PS. Toxic models of upper motor neuron disease.  J Neurol Sci 1996;139 (Suppl.):53-59
• Meyer T, Speer A, Meyer B, Münch C, Sitte W, Küther G, Ludolph AC. The glial glutamate transporter cDNA in patients with amyotrophic lateral sclerosis. Ann Neurol 1996;40:456-459
• Ludolph AC. Animal models for motor neuron disease – research directions. Neurology 1996;47(Suppl 6): S228-S232

1995

• Riepe MW, Hori N, Ludolph AC, Carpenter DO. Failure of neuronal ion exchange, not potentiated excitation causes excitotoxicity after inhibition of oxidative phosphorylation. Neuroscience 1995;64:91-97
• Riepe M, Spencer PS, Lambein F, Ludolph AC, Allen CA. Toxicological and pharmacological activity investigations of components of Lathyrus sativus. Natural Toxins 1995;3:58-64
• Ullrich K, Möller H, Weglage J, Schuierer G, Bick U, Ludolph AC, Hahn-Ullrich H, Fünders B, Koch HG. White matter abnormalities in phenylketonuria: results of magnetic resonance measurements. Acta Ped Scand 1995;(suppl.) 407:78-82.
• Bachus R, Claus A, Brockmöller J, Riepe M, Gericke C, Roots J, Ludolph AC. Cu/Zn SOD in German families with ALS.  J Neurol Sci 1995;129 (suppl): 93-95
• Gericke C, Zschenderlein R, Ludolph AC. Amyotrophic lateral sclerosis associated with multiple myeloma, endocrinopathy and skin changes suggestive of a POEMS syndrome variant.  J Neurol Sci 1995;129 (suppl), 58-60
• Enayat ZE, Orrell RW, Claus A, Ludolph AC, Bachus R, Brockmöller J, Ray-Chaudhuri K, Radunovic A, Shaw C, Wilkinson J, King A, Swash M, Leigh PN, de Belleroche J, Powell J. Two novel mutations in the gene for copper zinc superoxide dismutase in UK families with amyotrophic lateral sclerosis. Hum Mol Gen 1995;4:1239-1240
• Meyer T, Lenk U, Küther G, Weindl A, Speer A, Ludolph AC. Studies of the coding region of the neuronal glutamate transporter (EAAC1) gene in ALS. Ann Neurol 1995;37:817-819

1994 and earlier 

• Spencer PS, Allen CN, Kisby GE, Ludolph AC. On the Etiology and Pathogenesis of Chemically Induced Neurodegenerative Disorders. Neurobiol Aging 1994;15; 265-267
• Meierkord H, Pfeiffer L, Ludolph AC. Neue Erkenntnisse zu Ätiologie und Pathogenese der Chorea Huntington. Nervenarzt 1994;65;519-526
• Riepe M, Ludolph A, Seelig M, Spencer PS, Ludolph AC. Increase of ATP levels by glutamate antagonists is unrelated to neuroprotection. NeuroReport 1994;5:2130-2132
• Gericke CA, Ludolph AC. Chronic abuse of zolpidem. JAMA 1994;272:1721-1722
• Spencer PS, Ludolph AC, Kisby GE. Neurologic Diseases Associated with Use of Plant Components with Toxic Potential. Environmental Research 1994;62:106-113
• Ludolph AC, Riepe M, Ullrich K. Excitotoxicity, Energy Metabolism and Neuronal Degeneration. J Inher Metab Dis 1993;16:716-723
• Osterhage J, Ludolph AC, Masur H. Evozierte Potentiale in der Diagnostik der erektilen Dysfunktion. Kontinenz 1993;2 (4):175-182
• Ludolph AC, Masur H, Oberwittler C, Koch H, Ullrich K. Sensory Neuropathies in Homocystinuria – caused by vitamine B6. Eur J Pediatr 1993;152:271-272
• Spencer PS, Kisby GE, Ross SM, Roy DN, Hugon J, Ludolph AC, Nunn PB. Guam ALS/PDC – possibiles causes. Science 1993;262:825-826
• Hugon J, Ludolph AC, Spencer PS, Gimenez-Roldan S, Dumas JL.  Studies on the Etiology and Pathogenesis of Motor Neuron Diseases. III. Magnetic Cortical Stimulation in Patients with Lathyrism. Acta Neurol Scand 1993;88:412-416
• Bick U, Stöber U, Möller H, Schuierer G, Ludolph AC, Oberwittler C, Weglage J, Wendel U, Ullrich K. White Matter Abnormalities in Patients with Treated Hyperphenylalaninemia: Magnetic Resonance Relaxometry and Proton Spectroscopy Findings. Eur J Ped 1993;152:1012-1020
• Riepe M, Ludolph AC. Die Untersuchung kortikobulbärer Bahnen und peripherer Hirnnerven bei Normalpersonen und Patienten mit Multipler Sklerose: Ergebnisse nach nichtinvasiver elektromagnetischer Reizung. Z EEG EMG 1993;24:269-273
• Ludolph AC, Ullrich K, Nedjat S, Masur H, Bick U. Neurological Outcome in 22 Patients with Hyperphenylalaninemia – a Clinical and Electrophysiological Study. Acta Neurol Scand 1992;85:235-241
• Ludolph AC, Windgassen K. Der Schreibkrampf – eine Untersuchung bei 30 Patienten. Nervenarzt 1992;63:462-466
• Ludolph AC, Seelig M, Sabri M, Spencer PS. 3-Nitropropionic Acid Decreases Cellular Energy Levels and Causes Neutral Degeneration in Cortical Explants. Neurodegeneration 1992;1:155-161
• Riepe M, Hori N, Ludolph AC, Carpenter DO, Spencer PS, Allen CA. Inhibition of Energy Metabolism by 3-Nitropropionic Acid Activates ATP-Sensitive Potassium Channels. Brain Res 1992;586 (1):61-66
• Oberwittler C, Masur H, Ludolph AC, Fahrendorf G (1992): MR-Imaging of the Pyramidal Tract in Motor Neuron Disease. Neurology 42; 1550-1551
• Ludolph AC, Langen KJ, Regard M, Herzog H, Kemper B, Kuwert T, Böttger IG, Feinendegen L. Frontal Lobe Function in Amyotrophic Lateral Sclerosis – a Neuropsychologic and Positron Emission Tomography Study. Acta Neurol Scand 1992;85:81-89
• Masur H, Oberwittler C, Fahrendorf G, Heyen P, Reuther G, Nedjat S, Ludolph AC, Brune GG. The relation of functional deficits, motor and sensory conduction times and MRI findings in syringomyelia. Electroencephalogr Clin Neurophysiol 1992;85:321-330
• Masur H, Ludolph AC, Hilker E, Hengst K, Knuth U, Rolf LH, Bals-Pratsch M. Transcranial magnetic stimulation: Influence on plasma levels of hormones of the anterior pituitary gland and of cortisol? Functional Neurology 1991;6:1-5
• Spencer PS, Kisby GE, Ludolph AC. Slow Toxins, Biologic Markers, and Long-Latency Neurodegenerative Disease in the Western Pacific. Neurology 1991;41 (suppl. 2):61-66
• Bick U, Fahrendorf G, Ludolph AC, Vassallo P, Weglage J, Ullrich K. Disturbed Myelination in Patients with Treated Hyperphenylalaninaemia: Evaluation with Magnetic Resonance Imaging. Eur J Pediatr 1991;150:185-189
• Bartenstein P, Ludolph AC, Schober O, Lottes G, Scheidhauer K, Sciuk J, Beer HF.Benzodiazepine receptors and cerebral blood flow in partial epilepsy. Eur J Nucl Med 1991;18:111-118
• Ludolph AC, Ullrich K, Bick U, Fahrendorf G, Przyrembel H. Functional and Morphological Deficits in Late-Treated Patients with Homocystinuria: A Clinical, Electrophysiologic and MRI study. Acta Neurol Scand 1991;83:161-165
• Spencer PS, Kisby GE, Ludolph AC. Long-Latency Neurodegenerative Disease in the Western Pacific. Geriatrics 1991;46 (suppl. 1):37-42
• Ludolph AC, He F, Spencer PS, Hammerstad J, Sabri M. 3-Nitropropionic Acid – Exogenous Animal Neurotoxin and Possible Human Striatal Toxin. Canad J Neurol Sci 1991;18:492-498
• Windgassen K, Ludolph AC. Psychiatric Aspects of Writer’s Cramp. Eur Arch Psych Clin Neurosci 1991;240:171-176
• Spencer PS, Allen RG, Kisby GE, Ludolph AC. Excitotoxic Disorders. Science 1991;248:144
• Ludolph AC, Masur H, Füratsch N, Güth V. Normal Corticomotoneuronal Latencies in Idiopathic Scoliosis. Dev Med Child Neurol 1990;32:554-556
• Masur H, Zierz St, Ludolph AC, Elger CE, Palm DG. Corticomotoneuronal Latencies after Non-invasive Stimulation of the Motor Cortex in Adrenomyeloneuropathy and Adrenoleukodystrophy: Results in Patients and their Relatives.  Functional Neurology 1990;5:10-12
• Masur H, Elger CE, Ludolph AC, Galanski M. Cerebellar Atrophy Following Acute Intoxication with Phenytoin. Neurology 1989;39:432-433
• Deuschl G, Ludolph AC, Schenk E, Lücking CH. The Relations between Long-latency Reflexes in Hand Muscles, Somatosensory Evoked Potentials, and Transcranial Stimulation of Motor Tracts.  Electroencephalogr Clin Neurophysiol 1989; 74:425-430
• Masur H, Elger CE, Render K, Fahrendorf G, Ludolph AC. Functional Deficits of Central Sensory and Motor Pathways in Patients with Cervical Spinal Stenosis: A Study of SEPs and EMG Responses to Non-invasive Brain Stimulation. Electroencephalogr Clin Neurophysiol 1989;74:450-457
• Ludolph AC, Füratsch N, Elger CE, Masur H. Die elektromagnetische Stimulation des Nervensystems. I. Normalwerte im zentralen Nervensystem und Vergleich mit elektrischen Stimulationsmethoden.  Z EEG EMG 1989;20:153-158
• Ludolph AC, Dikämper S, Elger CE, Masur H. Die elektromagnetische Stimulation des Nervensystems. II. Normalwerte im peripheren Nervensystem und Vergleich mit elektrischen Stimulationsmethoden.  Z EEG EMG 1989;20:159-164
• Ludolph AC, Elger CE, Böttger IG, Kuttig AG, Lottes G, Brune GG. N-Isopropyl-p-123I-Amphetamine Single Photon Emission Computer Tomography in Motor Neuron Disease. Eur Neurol 1989;29:255-260
• Bartenstein P, Ludolph AC, Schober O, Lottes G, Böttger I. Vergleich von Blutfluß und Benzodiazepinrezeptorverteilung bei fokaler Epilepsie: vorläufige Ergebnisse einer SPECT Studie. Nuklearmedizin 1989;28:181-186
• Ludolph AC, Hugon J, Roman GC, Spencer PS, Schoenberg BS. A Clinical Neurophysiologic Study of Tropical Spastic Paraparesis. Muscle Nerve 1988;11:392-397
• Hugon J, Ludolph AC, Roy DN, Schaumburg HH, Spencer PS. Studies on the Etiology and Pathogenesis of Motor Neuron Disease. II. Clinical and Electrophysiologic Features of Pyramidal Dysfunction in Macaques fed Lathyrus sativus and IDPN. Neurology 1988;38:435-442
• Ludolph AC, Spille M, Masur H, Elger CE. Methodik und Normalwerte für die Ableitung von evozierten Muskelpotentialen nach nicht-invasiver Stimulation über den motorischen Wurzeln.  Z EEG EMG 1988;19:71-74
• Masur H, Elger CE, Render K, Fahrendorf G, Ludolph AC. Funktion der langen Rückenmarksbahnen bei engem Zervikalkanal – eine elektrophysiologische Untersuchung.  Z EEG EMG 1988;19:264-266
• Ludolph AC, Spille M, Masur H, Elger CE. Befunde im peripher-motorischen System nach Stimulation der motorischen Wurzeln: Polyradikulitis, ALS und Polyneuropathien.  Z EEG EMG 1988;19:255-259
• Ludolph AC, Husstedt IW, Schlake HP, Grotemeyer KH, Brune GG (1988): Chronic Ergotamine Abuse: Evidence of Functional Impairment of Long Ascending Spinal Tracts. Eur Neurol 28; 311-316
• Roman GC, Spencer PS, Schoenberg BS, Madden DL, Sever JL, Hugon, J, Ludolph AC.Tropical Spastic Paraparesis: HTLV-I Antibodies in Patients From the Seychelles.  New Engl J Med 1987;316:51
• Ludolph AC, Elger CE, Gößling JH, Hugon J. Methodik und Normalwerte für die Ableitung motorisch evozierter Potentiale nach transkranieller Stimulation beim Menschen.  Z EEG EMG 1987;18:33-35
• Ludolph AC, Hugon J, Dwivedi MP, Spencer PS, Schaumburg HH. Studies on the Aetiology and Pathogenesis of Motor Neuron Diseases. I. Lathyrism: Clinical Findings in Established Cases. Brain 1987;110:149-165
• Hirschberg M, Ludolph AC, Grotemeyer KH, Gullotta, F. Development of a Subacute Tetraparesis after Disulfiram Intoxication. Eur Neurol 1987;26:222-228
• Roman GC, Schoenberg BS, Madden DL, Sever JL, Hugon J, Ludolph AC, Spencer PS. Human T-Lymphotropic Virus Type I Antibodies in the Serum of Patients with Tropical Spastic Paraparesis in the Seychelles. Arch Neurol 1987;44:605-607
• Spencer PS, Nunn PB, Hugon J,Ludolph AC, Robertson RC, Ross SM, Roy DN. Guam amyotrophic lateral sclerosis-parkinsonism-dementia linked to a plant excitant neurotoxin.  Science 1987;237:517-521
• Roman CG, Spencer PS, Schoenberg BS, Hugon J, Ludolph AC, Rodgers-Johnson P, Osuntokon BO, Shamlaye C. Tropical Spastic Paraparesis in the Seychelles Islands: A Clinical and Case-control Neuroepidemiology Study. Neurology 1987;37:1323-1328
• Masur H, Kuchelmeister K, Ludolph AC, Elger CE. Hydrocephalus internus bei cerebralem M. Whipple. Psycho 1987;13:402-403
• Ludolph AC, Hugon J, Spencer PS. Non-invasive Assessment of Pyramidal Tract and Motor Pathway in Primates. Electroencephalogr. Clin. Neurophysiol 1987;67:63-67
• Ludolph AC, Elger CE, Gößling JH, Brune GG. Die Untersuchung der langen spinalen Bahnen bei der amyotrophen Lateralsklerose – elektrophysiologische und klinische Befunde.  Nervenarzt 1987;58:543-548
• Niemeyer K, Ludolph AC. Zur Bedeutung der subfaszialen Aeste des Plexus cervicalis für die motorische Innervation des M. trapezius. Ergebnisse elektromyographischer Untersuchungen des M. trapezius nach radikaler Neck dissection. Dtsch Z Mund Kiefer GesichtsChir 1987;11:356-360
• Knop J, Bonsmann G, Happle R, Ludolph AC, Matz DR, Mifsud EJ, Macher E. Thalidomide in the Treatment of Sixty Cases of Chronic Discoid Lupus Erythematodes. Br J Dermatol 1986;108; 461-466
• Van der Veen M, Ludolph AC, Telger K. Wirkungen und Nebenwirkungen von Haloperidoldecanoat und Fluphenazin-decanoat. Vorläufige Mitteilung über eine Vergleichsuntersuchung. Psycho 1986;11:471-472
• Ludolph AC, Elger C. Injektionsschäden rein sensibler Nerven im Bereich der oberen Extremität – eine nicht immer bekannte Komplikation. Med Welt 1986;37:713-715
• Ludolph AC, Elger CE, Hugon J. Die Untersuchung der Pyramidenbahnfunktion durch nicht-invasive Stimulation des motorischen Cortex. Psycho 1986;12:331
• Spencer PS, Nunn PB, Hugon J, Ludolph AC, Roy DN. Motorneurone Disease on Guam: Possible Role of a Food Neurotoxin, Lancet 1986;I:965
• Ludolph AC, Elger C, Sennhenn R, Bertram HP. Chronic Thallium Exposure in Workers of a Cement Plant: Clinical and Electrophysiological Data.  Trace Elem Med 1986;3:121-125
• Spencer PS, Roy DN, Ludolph AC, Hugon J, Dwivedi MP, Schaumburg HH. Lathyrism: Evidence for Role of the Neuroexcitatory Amino Acid BOAA. Lancet II 1986;1066-1067
•  

• Spencer PS, Schaumburg HH (eds.), Ludolph AC (Assoc. ed.) (2000): Spencer´s and Schaumburg´s Experimental and Clinical Neurotoxicology. Oxford University Press.

• Ludolph AC, Doble A (1997): Amyotrophic Lateral Sclerosis. Its Diagnosis and Treatment.

• Dengler R, Zierz S, Ludolph AC (1999): Die amyotrophe Lateralsklerose. Thieme Verlag, Stuttgart.
• Lehmann-Horn F. Ludolph AC (2000): Therapieschemata Neurologie. 3. Auflage. Urban & Schwarzenberg, München, Wien, Baltimore.
• Ludolph AC, Schwarz, J (2001): Grundlagen der klinischen Neuro-protektion. Unimed Verlag
• Ludolph AC, Winkler J (2004): Neurodegenerative Erkrankungen, Thieme-Verlag, Stuttgart
• Beal MF, Lang AT, Ludolph AC (2005): Neurodegenerative Diseases. Cambridge University Press