Wissenschaftliche Arbeit

  1. Günther R*, Wurster C*,…, Hagenacker T. Long-term efficacy and safety of Nusinersen in adults with 5q spinal muscular atrophy: a prospective European  multinational observational study. The Lancet Regional Heath – Europe (accepted, in press) Lancet Reg Health Eur. 2024 Feb 6;39:100862. doi: 10.1016/j.lanepe.2024.100862. eCollection 2024 Apr.
  2. Weber C, Müller A, Freigang M, von der Hagen M, Günther R. 'Reading the palm' - A pilot study of grip and finger flexion strength as an outcome measure in 5q spinal muscular atrophy. Brain Dev. 2024 May;46(5):189-198. doi: 10.1016/j.braindev.2024.01.001. Epub 2024 Feb 7.
  3. Vidovic M, Menschikowski M, Freigang M, Lapp HS, Günther R. Macrophage inclusions in cerebrospinal fluid following treatment initiation with antisense oligonucleotide therapies in motor neuron diseases. Neurol Res Pract. 2024 Feb 22;6(1):11. https://doi.org/10.1186/s42466-023-00305-0
  4. Lapp HS, Freigang M, Friese J, Bernsen S, Tüngler V, von der Hagen M, Weydt P, Günther R. Troponin T is elevated in a relevant proportion of patients with 5q-associated spinal muscular atrophy. Sci Rep. 2024 Mar 19;14(1):6634. doi: 10.1038/s41598-024-57185-w.
  5. Linse K, Weber C, Reilich P, Schöberl F, Boentert M, Petri S, Rödiger A, Posa A, Otto M, Wolf J, Zeller D, Brunkhorst R, Koch J, Hermann A, Großkreutz J, Schröter C, Groß M, Lingor P, Machetanz G, Semmler L, Dorst J, Lulé D, Ludolph A, Meyer T, Maier A, Metelmann M, Regensburger M, Winkler J, Schrank B, Kohl Z, Hagenacker T, Brakemeier S, Weyen U, Weiler M, Lorenzl S, Bublitz S, Weydt P, Grehl T, Kotterba S, Lapp HS, Freigang M, Vidovic M, Aust E, Günther R. Patients' and caregivers' perception of multidimensional and palliative care in amyotrophic lateral sclerosis - protocol of a German multicentre study. Neurol Res Pract. 2024 Jul 4;6(1):34. https://doi.org/10.1186/s42466-024-00328-1
  6. Vidovic M, Lapp HS, Weber C, Plitzko L, Seifert M, Steinacker P, Otto M, Hermann A, Günther R. Comparative analysis of neurofilaments and biomarkers of muscular damage in amyotrophic lateral sclerosis. Brain Commun. 2024 Aug 26;6(5):fcae288. https://doi.org/10.1093/braincomms/fcae288
  7. Freigang M, Langner U, Hermann A, Günther R. Impaired diaphragmatic motility in treatment-naive adult patients with spinal muscular atrophy improved during nusinersen treatment. Muscle Nerve 2023 (in press) 
  8. Vidovic M, Freigang M, Aust E, Linse K, Petzold D, Günther R. Cognitive performance of adult patients with SMA before and after treatment initiation with nusinersen. BMC Neurol 2023 Jun 6;23(1):216. doi: 10.1186/s12883-023-03261-z. 
  9. 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 Sep;9(9):1437-1448. doi: 10.1002/acn3.51645. Epub 2022 Aug 11. *shared last 
  10. Günther R*, Pal A*, Williams C, Zimyanin VL, Liehr M, von Neubeck C, Krause M, Parab MG, Petri S, Kalmbach N, Marklund S, Sterneckert J, Andersen PM, Wegner F, Gilthorpe JD, Andreas Hermann. Alteration of Mitochondrial Integrity as Upstream Event in the Pathophysiology of SOD1-ALS. Cells. 2022 Apr 6;11(7):1246. doi: 10.3390/cells11071246. *shared first 
  11. Hermann W, Langner S, Freigang M, Fischer S, Storch A, Günther R*, Hermann A. Affection of Respiratory Muscles in ALS and SMA. J Clin Med. 2022 Feb 22;11(5):1163. doi: 10.3390/jcm11051163. *shared last 
  12. Jin M, Akgün K, Ziemssen T, Kipp M, Günther R*, Hermann A*. Interleukin-17 and Th17 Lymphocytes Directly Impair Motoneuron Survival of Wildtype and FUS-ALS Mutant Human iPSCs. Int J Mol Sci. 2021 Jul 27;22(15):8042. doi: 10.3390/ijms22158042. *shared last 
  13. 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 Chitotriosidase1 Concentration Following Nusinersen Treatment in Spinal Muscular Atrophy. Orphanet J Rare Dis. 2021 Jul 28;16(1):330. *shared last 
  14. 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 are promising biomarkers in adult spinal muscular atrophy. Ann Clin Transl Neurol. 2021 May;8(5):1049-1063.doi: 10.1002/acn3.51340. Epub 2021 Mar 31.* shared last
  15. Vidovic M, Aust E, Hermann A, Günther R. The palmomental reflex in amyotrophic lateral sclerosis – A clinical sign of executive or motor dysfunction? Amyotroph Lateral Scler Frontotemporal Degener. 2021Nov;22(7-8):588-591. doi: 10.1080/21678421.2021.1883667. Epub 2021 Mar 3.
  16. Beyer L*, Günther R*, Koch JC, Klebe S, Hagenacker T, Lingor P, Biesalski A, Hermann A, Nabers A, Gold R, Tönges L, Gerwert K. TDP-43 as Structure-Based Biomarker in Amyotrophic Lateral Sclerosis. Ann Clin Transl Neurol. 2021 Jan;8(1):271-277. doi: 10.1002/acn3.51256. Epub 2020 Dec 2. *shared first
  17. 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: non-interventional, multicentre, observational cohort study. Lancet Neurol. 2020 Apr;19(4):317-325.doi: 10.1016/S1474-4422(20)30037-5. Epub 2020 Mar 18. *shared first 
  18. Mengmeng J*, Günther R*, Akgün K, Hermann A, Ziemsse T. Peripheral proinflammatory Th1/Th17 immune cell shift is linked to disease severity in amyotrophic lateral sclerosis. Sci Rep. 2020 Apr3;10(1):5941. doi: 10.1038/s41598-020-62756-8. *shared first
  19. Wurster CD, Koch JC, Cordts I, Dreyhaupt J, Otto M, Uzelac Z, Witzel S, Winter B, Kocak B, 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 Nov7;10:1179. doi: 10.3389/fneur.2019.01179. eCollection 2019. 
  20. 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 Nov 1;10:1098. doi: 10.3389/fneur.2019.01098. eCollection 2019. 
  21. Günther R, Neuwirth C, Koch JC, Lingor P, Braun N, Untucht R, Petzold D, Weber M, Hermann Motor Unit Number Index (MUNIX) of hand muscles is a disease biomarker for adult spinal muscular atrophy. Clin Neurophysiol. 2019 Feb;130(2):315-319.doi:10.1016/j.clinph.2018.11.009. Epub 2018 Nov 24. 
  22. Günther R, Schrempf W, Hähner A, Hummel T, Wolz M, Storch A, Hermann A. Impairment in respiratory function contributes to olfactory impairment in Amyotrophic Lateral Sclerosis. Front Neurol. 2018 Feb 26;9:79. doi: 10.3389/fneur.2018.00079. eCollection 2018. 
  23. Günther R, Balck A, Koch JC, Nientiedt T, Sereda M, Bähr M, Lingor P, Tönges L. Rho Kinase Inhibition with Fasudil in the SOD1G93A Mouse Model of Amyotrophic Lateral Sclerosis-Symptomatic Treatment Potential after Disease Onset. Front Pharmacol. 2017 Jan 31;8:17. doi: 10.3389/fphar.2017.00017. eCollection 2017. 
  24. Günther R, Richter N, Sauerbier A, Chaudhuri KR, Martinez-Martin P, Storch A, Hermann A. Non-Motor Symptoms in Patients Suffering from Motor Neuron Diseases. Front Neurol. 2016 Jul 25;7:117.doi: 10.3389/fneur.2016.00117. eCollection 2016. 
  25. Günther R, Saal KA, Suhr M, Scheer D, Koch JC, Bähr M, Lingor P, Tönges L. The rho kinase inhibitor Y-27632 improves motor performance in male SOD1(G93A) mice. Front Neurosci. 2014 Oct 7;8:304. doi: 10.3389/fnins.2014.00304. eCollection 2014. 
  26. Günther R, Suhr M, Koch JC, Bähr M, Lingor P, Tönges L. Clinical testing and spinal cord removal in a mouse model for amyotrophic lateral sclerosis (ALS). J Vis Exp. 2012 Mar 17;(61):3936. doi: 10.3791/3936.
  27. Arteaga-Bracho E, Cosne G, Kanzler C, Karatsidis A, Mazzà C, Penalver-Andres J, Zhu C, Shen C, Erb M K, Freigang M, Lapp HS, Thiele S, Wenninger S, Jung E, Petri S, Weiler M, Kleinschnitz C, Walter MC, Günther R, Campbell N, Belachew S, Hagenacker T. Smartphone-Based Assessment of Mobility and Manual Dexterity in Adult People with Spinal Muscular Atrophy. J Neuromuscul Dis. 2024;11(5):1049-1065. doi: 10.3233/JND-240004.
  28. Aust E, Günther R, Hermann A, Linse K. [Psychologically guided group meetings for family caregivers of ALS patients]. Fortschr Neurol Psychiatr. 2024 Mar;92(3):81-89. doi: 10.1055/a-2156-9013. Epub 2023 Nov 6.
  29. Wiesenfarth M, Dorst J, Brenner D, Elmas Z, Parlak Ö, Uzelac Z, Kandler K, Mayer K, Weiland U, Herrmann C, Schuster J, Freischmidt A, Müller K, Siebert R, Bachhuber F, Simak T, Günther K, Fröhlich E, Knehr A, Regensburger M, German A, Petri S, Grosskreutz J, Klopstock T, Reilich P, Schöberl F, Hagenacker T, Weyen U, Günther R, Vidovic M, Jentsch M, Haarmeier T, Weydt P, Valkadinov I, Hesebeck-Brinckmann J, Conrad J, Weishaupt JH, Schumann P, Körtvélyessy P, Meyer T, Ruf WP, Witzel S, Senel M, Tumani H, Ludolph AC. Effects of tofersen treatment in patients with SOD1-ALS in a "real-world" setting - a 12-month multicenter cohort study from the German early access program. EClinicalMedicine. 2024 Feb 15;69:102495. doi: 10.1016/j.eclinm.2024.102495. eCollection 2024 Mar.
  30. Vill K, Tacke M, König A, Baumann M, Baumgartner M, Steinbach M, Bernert G, Blaschek A, Deschauer M, Flotats-Bastardas M, Friese J, Goldbach S, Gross M, Günther R, Hahn A, Hagenacker T, Hauser E, Horber V, Illsinger S, Johannsen J, Kamm C, Koch JC, Koelbel H, Koehler C, Kolzter K, Lochmüller H, Ludolph A, Mensch A, Meyer Zu Hoerste G, Mueller M, Mueller-Felber W, Neuwirth C, Petri S, Probst-Schendzielorz K, Pühringer M, Steinbach R, Schara-Schmidt U, Schimmel M, Schrank B, Schwartz O, Schlachter K, Schwerin-Nagel A, Schreiber G, Smitka M, Topakian R, Trollmann R, Tuerk M, Theophil M, Rauscher C, Vorgerd M, Walter MC, Weiler M, Weiss C, Wilichowski E, Wurster CD, Wunderlich G, Zeller D, Ziegler A, Kirschner J, Pechmann A; SMArtCARE study group. 5qSMA: standardised retrospective natural history assessment in 268 patients with four copies of SMN2. J Neurol. 2024 May;271(5):2787-2797. doi: 10.1007/s00415-024-12188-5. Epub 2024 Feb 27.
  31. Pal A, Grossmann D, Glaß H, Zimyanin V, Günther R, Catinozzi M, Boeckers TM, Sterneckert J, Storkebaum E, Petri S, Wegner F, Grill SW, Pan-Montojo F, Hermann A. Glycolic acid and D-lactate-putative products of DJ-1-restore neurodegeneration in FUS - and SOD1-ALS. Life Sci Alliance. 2024 May 17;7(8):e202302535. doi: 10.26508/lsa.202302535. Print 2024 Aug.
  32. Chatterjee M, Özdemir S, Fritz C, Möbius W, Kleineidam L, Mandelkow E, Biernat J, Doğdu C, Peters O, Cosma NC, Wang X, Schneider LS, Priller J, Spruth E, Kühn AA, Krause P, Klockgether T, Vogt IR, Kimmich O, Spottke A, Hoffmann DC, Fliessbach K, Miklitz C, McCormick C, Weydt P, Falkenburger B, Brandt M, Guenther R, Dinter E, Wiltfang J, Hansen N, Bähr M, Zerr I, Flöel A, Nestor PJ, Düzel E, Glanz W, Incesoy E, Bürger K, Janowitz D, Perneczky R, Rauchmann BS, Hopfner F, Wagemann O, Levin J, Teipel S, Kilimann I, Goerss D, Prudlo J, Gasser T, Brockmann K, Mengel D, Zimmermann M, Synofzik M, Wilke C, Selma-González J, Turon-Sans J, Santos-Santos MA, Alcolea D, Rubio-Guerra S, Fortea J, Carbayo Á, Lleó A, Rojas-García R, Illán-Gala I, Wagner M, Frommann I, Roeske S, Bertram L, Heneka MT, Brosseron F, Ramirez A, Schmid M, Beschorner R, Halle A, Herms J, Neumann M, Barthélemy NR, Bateman RJ, Rizzu P, Heutink P, Dols-Icardo O, Höglinger G, Hermann A, Schneider A. Plasma extracellular vesicle tau and TDP-43 as diagnostic biomarkers in FTD and ALS. Nat Med. 2024 Jun;30(6):1771-1783. doi: 10.1038/s41591-024-02937-4. Epub 2024 Jun 18.
  33. Meyer T, Dreger M, Grehl T, Weyen U, Kettemann D, Weydt P, Günther R, Lingor P, Petri S, Koch JC, Großkreutz J, Rödiger A, Baum P, Hermann A, Prudlo J, Boentert M, Weishaupt JH, Löscher WN, Dorst J, Koc Y, Bernsen S, Cordts I, Vidovic M, Steinbach R, Metelmann M, Kleinveld VE, Norden J, Ludolph A, Walter B, Schumann P, Münch C, Körtvélyessy P, Maier A. Serum neurofilament light chain in distinct phenotypes of amyotrophic lateral sclerosis: A longitudinal, multicenter study. Eur J Neurol. 2024 Sep;31(9):e16379. doi: 10.1111/ene.16379. Epub 2024 Jun 10.
  34. Schaub A, Erdmann H, Scholz V, Timmer M, Cordts I, Günther R, Reilich P, Abicht A, Schöberl F. J Neurol. Analysis and occurrence of biallelic pathogenic repeat expansions in RFC1 in a German cohort of patients with a main clinical phenotype of motor neuron disease. 2024 Sep;271(9):5804-5812. doi: 10.1007/s00415-024-12519-6. Epub 2024 Jun 25.
  35. Meyer T, Schumann P, Weydt P, Petri S, Weishaupt JH, Weyen U, Koch JC, Günther R, Regensburger M, Boentert M, Wiesenfarth M, Koc Y, Kolzarek F, Kettemann D, Norden J, Bernsen S, Elmas Z, Conrad J, Valkadinov I, Vidovic M, Dorst J, Ludolph AC, Hesebeck-Brinckmann J, Spittel S, Münch C, Maier A, Körtvélyessy P. Clinical and patient-reported outcomes and neurofilament response during tofersen treatment in SOD1-related ALS-A multicenter observational study over 18 months. Muscle Nerve. 2024 Sep;70(3):333-345. doi: 10.1002/mus.28182. Epub 2024 Jun 20.
  36. Meyer T, Schumann P, Grehl T, Weyen U, Petri S, Rödiger A, Steinbach R, Grosskreutz J, Bernsen S, Weydt P, Wolf J, Günther R, Vidovic M, Baum P, Metelmann M, Weishaupt JH, Streubel B, Kasper DC, Koc Y, Kettemann D, Norden J, Schmitt P, Walter B, Münch C, Spittel S, Maier A, Körtvélyessy P. SOD1 gene screening in ALS - frequency of mutations, patients' attitudes to genetic information and transition to tofersen treatment in a multi-center program. Amyotroph Lateral Scler Frontotemporal Degener. 2024 Sep 13:1-10. doi: 10.1080/21678421.2024.2401131. Online ahead of print.
  37. Koch JC, Leha A, Bidner H, Cordts I, Dorst J, Günther R, Zeller D, Braun N, Metelmann M, Corcia P, De La Cruz E, Weydt P, Meyer T, Großkreutz J, Soriani MH, Attarian S, Weishaupt JH, Weyen U, Kuttler J, Zurek G, Rogers ML, Feneberg E, Deschauer M, Neuwirth C, Wuu J, Ludolph AC, Schmidt J, Remane Y, Camu W, Friede T, Benatar M, Weber M, Lingor P; ROCK-ALS Study group. Safety, tolerability, and efficacy of fasudil in amyotrophic lateral sclerosis (ROCK-ALS): a phase 2, randomised, double-blind, placebo-controlled trial. Lancet Neurol. 2024 Nov;23(11):1133-1146. doi: 10.1016/S1474-4422(24)00373-9.
  38. Heinrich F, 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, Radelfahr F, Schönfelder E, Gardt P, Mohajer-Peseschkian T, Osmanovic A, Klopstock T, Dorst J, Ludolph AC, Schöffski O, Boentert M, Hagenacker T, Deschauer M, Lingor P, Petri S, Schreiber-Katz O. Economic evaluation of Motor Neuron Diseases: a nationwide cross-sectional analysis in Germany. J Neurol. 2023 Jun 25. doi: 10.1007/s00415-023-11811-1. Online ahead of print. 
  39. Ruf WP, Boros M, Freischmidt A, Brenner D, Grozdanov V, de Meirelles J, Meyer T, Grehl T, Petri S, Grosskreutz J, Weyen U, Guenther R, Regensburger M, Hagenacker T, Koch JC, Emmer A, Roediger A, Steinbach R, Wolf J, Weishaupt JH, Lingor P, Deschauer M, Cordts I, Klopstock T, Reilich P, Schoeberl F, Schrank B, Zeller D, Hermann A, Knehr A, Günther K, Dorst J, Schuster J, Siebert R, Ludolph AC, Müller K. Spectrum and frequency of genetic variants in sporadic amyotrophic lateral sclerosis. Brain Commun. 2023 May 9;5(3):fcad152. doi: 10.1093/braincomms/fcad152. eCollection 2023. 
  40. Meyer T, Schumann P, Weydt P, Petri S, Koc Y, Spittel S, Bernsen S, Günther R, Weishaupt JH, Dreger M, Kolzarek F, Kettemann D, Norden J, Boentert M, Vidovic M, Meisel C, Münch C, Maier A, Körtvélyessy P. Neurofilament light-chain response during therapy with antisense oligonucleotide tofersen in SOD1-related ALS: Treatment experience in clinical practice. Muscle Nerve. 2023 Jun;67(6):515-521. doi: 10.1002/mus.27818. Epub 2023 Apr 3. 
  41. Meyer T, Salkic E, Grehl T, Weyen U, Kettemann D, Weydt P, Günther R, Lingor P, Koch JC, Petri S, Hermann A, Prudlo J, Großkreutz J, Baum P, Boentert M, Metelmann M, Norden J, Cordts I, Weishaupt JH, Dorst J, Ludolph A, Koc Y, Walter B, Münch C, Spittel S, Dreger M, Maier A, Körtvélyessy P. Performance of serum neurofilament light chain in a wide spectrum of clinical courses of amyotrophic lateral sclerosis-a cross-sectional multicenter study. Eur J Neurol. 2023 Jun;30(6):1600-1610. doi: 10.1111/ene.15773. Epub 2023 Mar 26.
  42. Szewczyk B, Günther R, Japtok J, Frech MJ, Naumann M, Lee HO, Hermann A. FUS ALS neurons activate major stress pathways and reduce translation as an early protective mechanism against neurodegeneration. Cell Rep. 2023 Jan 24;42(2):112025. doi: 10.1016/j.celrep.2023.112025. Online ahead of print. 
  43. Pechmann A, Behrens M, Dörnbrack K, Tassoni A, Wenzel F, Stein S, Vogt S, Zöller D, Bernert G, Hagenacker T, Schara-Schmidt U, Walter MC, Steinbach M, Blaschek A, Baumann M, Baumgartner M, Becker B, Flotats-Bastardas M, Friese J, Günther R, Hahn A, Küpper H, Johannsen J, Kamm C, Koch JC, Köhler C, Kölbel H, Kolzter K, von Moers A, Naegel S, Neuwirth C, Petri S, Rödiger A, Schimmel M, Schrank B, Schreiber G, Smitka M, Stadler C, Steiner E, Stögmann E, Trollmann R, Türk M, Weiler M, Stoltenburg C, Willichowsky E, Zeller D, Ziegler A, Lochmüller H, Kirschner J; SMArtCARE study group. Improvements in Walking Distance during Nusinersen Treatment - A Prospective 3-year SMArtCARE Registry Study. J Neuromuscul Dis. 2023;10(1):29-40. doi: 10.3233/JND-221600. 
  44. Maier A, Boentert M, Reilich P, Witzel S, Petri S, Großkreutz J, Metelmann M, Lingor P, Cordts I, Dorst J, Zeller D, Günther R, Hagenacker T, Grehl T, Spittel S, Schuster J, Ludolph A, Meyer T; MND-NET consensus group. ALSFRS-R-SE: an adapted, annotated, and self-explanatory version of the revised amyotrophic lateral sclerosis functional rating scale. Neurol Res Pract. 2022 Dec 15;4(1):60. doi: 10.1186/s42466-022-00224-6. 
  45. Aust E, Linse K, Graupner ST, Joos M, Liebscher D, Grosskreutz J, Prudlo J, Meyer T, Günther R, Pannasch S, Hermann A. Quality of life and mental health in the locked-in-state-differences between patients with amyotrophic lateral sclerosis and their next of kin. J Neurol. 2022 Nov;269(11):5910-5925. doi: 10.1007/s00415-022-11238-0. Epub 2022 Jul 6. 
  46. Pechmann A, Behrens M, Dörnbrack K, Tassoni A, Wenzel F, Stein S, Vogt S, Zöller D, Bernert G, Hagenacker T, Schara-Schmidt U, Walter MC, Bertsche A, Vill K, Baumann M, Baumgartner M, Cordts I, Eisenkölbl A, Flotats-Bastardas M, Friese J, Günther R, Hahn A, Horber V, Husain RA, Illsinger S, Jahnel J, Johannsen J, Köhler C, Kölbel H, Müller M, von Moers A, Schwerin-Nagel A, Reihle C, Schlachter K, Schreiber G, Schwartz O, Smitka M, Steiner E, Trollmann R, Weiler M, Weiß C, Wiegand G, Wilichowski E, Ziegler A, Lochmüller H, Kirschner J; SMArtCARE study group. Improved upper limb function in non-ambulant children with SMA type 2 and 3 during nusinersen treatment: a prospective 3-years SMArtCARE registry study. Orphanet J Rare Dis. 2022 Oct 23;17(1):384. doi: 10.1186/s13023-022-02547-8. 
  47. Stolte B, Schreiber-Katz O, Günther R, Wurster CD, Petri S, Osmanovic A, Freigang M, Uzelac Z, Leo M, von Velsen O, Bayer W, Dittmer U, Kleinschnitz C, Hagenacker T. Prevalence of Anti-Adeno-Associated Virus Serotype 9 Antibodies in Adult Patients with Spinal Muscular Atrophy. Hum Gene Ther. 2022 Sep;33(17-18):968-976. doi:10.1089/hum.2022.054. Epub 2022 Sep 7. 
  48. Meyer T, Spittel S, Grehl T, Weyen U, Steinbach R, Kettemann D, Petri S, Weydt P, Günther R, Baum P, Schlapakow E, Koch JC, Boentert M, Wolf J, Grosskreutz J, Rödiger A, Ilse B, Metelmann M, Norden J, Koc RY, Körtvélyessy P, Riitano A, Walter B, Hildebrandt B, Schaudinn F, Münch C, Maier A. Remote digital assessment of amyotrophic lateral sclerosis functional rating scale - a multicenter observational study. Amyotroph Lateral Scler Frontotemporal Degener. 2022 Aug 1:1-10. doi: 10.1080/21678421.2022.2104649. Online ahead of print. 
  49. Hahn A, Günther R, Ludolph A, Schwartz O, Trollmann R, Weydt P, Weiler M, Neuland K, Schwaderer MS, HagenackerT. Short-term safety results from compassionate use of risdiplam in patients with spinal muscular atrophy in Germany. Orphanet J Rare Dis. 2022 Jul 19;17(1):276. doi: 10.1186/s13023-022-02420-8. 
  50. Pechmann A, Behrens M, Dörnbrack K, Tassoni A, Stein S, Vogt S, Zöller D, Bernert G, Hagenacker T, Schara-Schmidt U, Schwersenz I, Walter MC, Baumann M, Baumgartner M, Deschauer M, Eisenkölbl A, Flotats-Bastardas M, Hahn A, Horber V, Husain RA, Illsinger S, Johannsen J, Köhler C, Kölbel H, Müller M, von Moers A, Schlachter K, Schreiber G, Schwartz O, Smitka M, Steiner E, Stögmann E, Trollmann R, Vill K, Weiß C, Wiegand G, Ziegler A, Lochmüller H, Kirschner J; SMArtCARE study group. Effect of nusinersen on motor, respiratory and bulbar function in early-onset spinal muscular atrophy. Brain. 2022 Jul 20:awac252. doi: 10.1093/brain/awac252. Online ahead of print. 
  51. Schorling DC, Kölbel H, Hentschel A, Pechmann A, Meyer N, Wirth B, Rombo R; SMArtCARE consortium; Sickmann A, Kirschner J, Schara-Schmidt U, Lochmüller H, Roos A. Cathepsin D as biomarker in cerebrospinal fluid of nusinersen-treated patients with spinal muscular atrophy. Eur J Neurol. 2022 Jul;29(7):2084-2096. doi: 10.1111/ene.15331. Epub 2022 May 4 
  52. 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. 
  53. Binz C, Osmanovic A, Thomas N, 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. 
  54. 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 W, Kohl W, Hagenacker T, Ruckes C, Koch B, Spittel S, Günther K, Michels S, Dorst J, Meyer T*, Ludolph AC*, on behalf of the German Motor Neuron Disease Network (MND-Net). Neurology Safety and Efficacy of Long-Term Intravenous Edaravone Treatment in Amyotrophic Lateral Sclerosis — Results from a Multicenter Observational Study. JAMA Neurol. 2022 Feb 1;79(2):121-130. doi: 10.1001/jamaneurol.2021.4893. 
  55. Linse K, Aust E, Günther R, Hermann A. Caregivers’ View of Socio-Medical Care in the Terminal Phase of Amyotrophic Lateral Sclerosis—How Can We ImproveHolistic Care in ALS? J Clin Med. 2022 Jan 4;11(1):254. doi: 10.3390/jcm11010254. 
  56. Szewczyk B, Günther R, Sterneckert J, Petri S, Wegner F, Hermann A. FUS Is Not Mislocalized in Spinal Motor Neurons Derived From Human Induced Pluripotent Stem Cells of Main Non-FUS ALS Subtypes. J Neuropathol Exp Neurol. 2021 Aug 11;80(7):720-722. doi: 10.1093/jnen/nlaa154. 
  57. 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 Aug;28(8):2582-2595. doi: 10.1111/ene.14902. Epub 2021 Jun 16. 
  58. 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.
  59. Becker L, Weiss C, Günther R, Hermann A, Theophile M, Hübner A, Smitka M, Hagen M, Kaindl A. Evaluation of metabolic effects of nusinersen in patients with spinal muscular atrophy. Journal of Pediatric Neurology. Journal of Pediatric Neurology 2022; 20(04): 252-257. doi: 10.1055/s-0041-1731395 
  60. Pal A, Kretner B, Abo-Rady M, Glaβ H, Dash BP, Naumann M, Japtok J, Kreiter N, Dhingra A, Heutink P, Böckers T, Günther R, Sterneckert J, Hermann A. Concomitant gain and loss of function pathomechanisms in C9ORF72 amyotrophic lateral sclerosis. Life Sci Alliance. 2021 Feb 22;4(4):e202000764. doi: 10.26508/lsa.202000764. Print 2021 Apr. 
  61. Loewenbrück KF, Werner R, Günther R, Dittrich M, Klinkenberger R, Machetanz J, Reichmann H, Storch A, Hermann A. One nerve suffices: A clinically guided nerve ultrasound protocol for the differentiation of multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis (ALS). J Neurol. 2021 Apr;268(4):1495-1507. doi: 10.1007/s00415-020-10323-6. Epub 2020 Dec 23. 
  62. 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.
  63. Osmanovic A , RanxhaG; Kumpe M; Wurster CD, Stolte B, Cordts I, Günther R, Freigang M, Müschen L; 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. 
  64. Bräuer S, Günther R, Sterneckert J, Glaß H, Hermann A. Human Spinal Motor Neurons Are Particularly Vulnerable to Cerebrospinal Fluid of Amyotrophic Lateral Sclerosis Patients. Int J Mol Sci. 2020 May 18;21(10):3564. doi: 10.3390/ijms21103564. 
  65. Meyer T, Kettemann D, Maier A, Grehl T, Weyen U, Grosskreutz J, Steinbach J, Norden J, George A, Hermann A, Günther 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 Psychiatry. 2020 Jul;91(7):783-785. doi: 10.1136/jnnp-2020-322938. Epub 2020 Apr 21.
  66. 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. Neurofilament light chain in serum of adolescent and adult SMA patients under treatment with nusinersen. J Neurol. 2020 Jan;267(1):36-44. doi: 10.1007/s00415-019-09547-y. Epub 2019 Sep 24. 
  67. 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 Dec;6(12):2384-2394. doi: 10.1002/acn3.50930. Epub 2019 Nov 4. 
  68. Peikert K, Naumann M, Günther R, Wegner F, Hermann A. Off-Label Treatment of 4 Amyotrophic Lateral Sclerosis Patients With 4-Aminopyridine. J Clin Pharmacol. 2019 Oct;59(10):1400-1404. doi: 10.1002/jcph.1437. Epub 2019 Apr 30. 
  69. Wurster C, 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 under treatment with nusinersen. Ther Adv Neurol Disord. 2019 May 10;12:1756286419846058. doi: 10.1177/1756286419846058. eCollection 2019. 
  70. Winter B, Günther 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 Psychiatry. 2019 Sep;90(9):1068-1069. doi: 10.1136/jnnp-2018-320033. Epub 2019 Jan 10. 
  71. 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 Jan 23;9(1):335. doi: 10.1038/s41467-017-02299-1. 
  72. Tönges L, Günther R, Suhr M, Jansen J, Balck A, Saal KA, Barski E, Nientied T, Götz AA, Koch JC, Mueller BK, Weishaupt JH, Sereda MW, Hanisch UK, Bähr M, Lingor P. Rho kinase inhibition modulates microglia activation and improves survival in a model of amyotrophic lateral sclerosis. Glia. 2014 Feb;62(2):217-32. doi: 10.1002/glia.22601. Epub 2013 Dec 6. 
  73. Lapp HS, Freigang M, Hagenacker T, Weiler M, Wurster CD, Günther R. Biomarkers in 5q-associated spinal muscular atrophy-a narrative review. J Neurol. 2023 Jun 8. doi: 10.1007/s00415-023-11787-y. 
  74. Günther R.Gentherapien bei den Motoneuronerkrankungen ALS und SMA. Fortschr Neurol Psychiatr 2023;91:1–11, doi: 10.1055/a-2002-5215 
  75. Günther R, von der Hagen M, Weiler M, Winter B, Wurster CD, Ziegler A. Hrsg. Hagenacker T, Schara-Schmidt U. Spinale Muskelatrophie – Transition von der Neuropädiatrie in die Neurologie. Thieme Praxis Report, 2022;1-12. ISSN 1611-7891
  76. Lingor P, Koch JC, Statland JM, Hussain S, Hennecke C, Wuu J, Langbein T, Ahmed R, Günther R, Ilse B, Kassubek J, Kollewe K, Kuttler J, Leha A, Lengenfeld T, Meyer T, Neuwirth C, Tostmann R, Benatar M. Challenges and opportunities for Multi-National Investigator-Initiated clinical trials for ALS: European and United States collaborations. Amyotroph Lateral Scler Frontotemporal Degener. 2021 Aug;22(5-6):419-425. doi: 10.1080/21678421.2021.1879866. Epub 2021 Feb 3. 
  77. Wurster CD, Günther R. Innovative Therapiestrategien bei der Spinalen Muskelatrophie. Kompendium ZNS 2020; 7-15
  78. Wurster CD, Günther R. [New treatments for spinal muscular atrophy]. Der Nervenarzt. 2020 Apr;91(4):294-302 
  79. Hagenacker T, Hermann A, Kamm C, Walter MC, Weiler M, Günther R, Wurster CD, Kleinschnitz C. [Spinal Muscular Atrophy - expert recommendations for the use of nusinersen in adult patients]. Fortschr Neurol Psychiatr. 2019 Dec;87(12):703-710. doi: 10.1055/a-0996-0994. Epub 2019 Dec 17. 
  80. 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 A, Koch JC and the 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 Mar 27;10:293. doi: 10.3389/fneur.2019.00293. eCollection 2019. 
  81. 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]. 2019 Apr;90(4):343-351. doi: 10.1007/s00115-018-0653-7.Erfahrungen aus der klinischen Praxis. Der Nervenarzt. 90(4):343-351
  82. Günther R. Kapitel: Therapie der Amyotrophen Lateralsklerose in Amyotrophe Lateralsklerose und andere Motoneuronerkrankungen. Hermann A. Kohlhammerverlag, ISBN 978-3-17-039167-3