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Motor phenotypes of amyotrophic lateral sclerosis - a three-determinant anatomical classification based on the region of onset, propagation of motor symptoms, and the degree of upper and lower motor neuron dysfunction.
Meyer T, Boentert M, Großkreutz J, Weydt P, Bernsen S, Reilich P, Steinbach R, Rödiger A, Wolf J, Weyen U, Ludolph AC, Weishaupt J, Petri S, Lingor P, Günther R, Löscher W, Weber M, Münch C, Maier A, Grehl T. Meyer T, et al. Among authors: gunther r. Neurol Res Pract. 2025 Apr 28;7(1):27. doi: 10.1186/s42466-025-00389-w. Neurol Res Pract. 2025. PMID: 40289140 Free PMC article.
SOD1-ALS mimicking an inflammatory neuropathy: a case report.
Lapp HS, Günther R. Lapp HS, et al. Among authors: gunther r. Amyotroph Lateral Scler Frontotemporal Degener. 2025 Apr 12:1-4. doi: 10.1080/21678421.2025.2488296. Online ahead of print. Amyotroph Lateral Scler Frontotemporal Degener. 2025. PMID: 40219902 Free article.
Self-assessment of amyotrophic lateral sclerosis functional rating scale on the patient's smartphone proves to be non-inferior to clinic data capture.
Steinfurth L, Grehl T, Weyen U, Kettemann D, Steinbach R, Rödiger A, Grosskreutz J, Petri S, Boentert M, Weydt P, Bernsen S, Walter B, GüNTHER R, Lingor P, Koch JC, Baum P, Weishaupt JH, Dorst J, Koc Y, Cordts I, Vidovic M, Norden J, Schumann P, Körtvélyessy P, Spittel S, Münch C, Maier A, Meyer T. Steinfurth L, et al. Among authors: gunther r. Amyotroph Lateral Scler Frontotemporal Degener. 2025 Feb 22:1-12. doi: 10.1080/21678421.2025.2468404. Online ahead of print. Amyotroph Lateral Scler Frontotemporal Degener. 2025. PMID: 39985291 Free article.
Long-Term Dynamics of CSF and Serum Neurofilament Light Chain in Adult Patients With 5q Spinal Muscular Atrophy Treated With Nusinersen.
Cordts I, Fuetterer C, Wachinger A, von Heynitz R, Kessler T, Freigang M, Quinten AL, Bjelica B, Brakemeier S, Hobbiebrunken E, Hagenacker T, Petri S, Koch JC, Hahn A, Lingor P, Deschauer M, Günther R, Weiler M, Haller B, Feneberg E. Cordts I, et al. Among authors: gunther r. Neurology. 2025 Mar 11;104(5):e213371. doi: 10.1212/WNL.0000000000213371. Epub 2025 Feb 13. Neurology. 2025. PMID: 39946662 Free PMC article.
Shared prognostic information in amyotrophic lateral sclerosis - systematic assessment of the patients' perception of neurofilament light chain and the ALS functional rating scale.
Möhwald LM, Maier A, Grehl T, Weyen U, Weydt P, Günther R, Lingor P, Göricke B, Petri S, Grosskreutz J, Boentert M, Cordts I, Weishaupt JH, Dorst J, Münch C, Meyer T, Baum P. Möhwald LM, et al. Among authors: gunther r. Neurol Res Pract. 2025 Feb 6;7(1):6. doi: 10.1186/s42466-024-00363-y. Neurol Res Pract. 2025. PMID: 39910638 Free PMC article.
Efficacy and safety of gene therapy with onasemnogene abeparvovec in children with spinal muscular atrophy in the D-A-CH-region: a population-based observational study.
Weiß C, Becker LL, Friese J, Blaschek A, Hahn A, Illsinger S, Schwartz O, Bernert G, Hagen MV, Husain RA, Goldhahn K, Kirschner J, Pechmann A, Flotats-Bastardas M, Schreiber G, Schara U, Plecko B, Trollmann R, Horber V, Wilichowski E, Baumann M, Klein A, Eisenkölbl A, Köhler C, Stettner GM, Cirak S, Hasselmann O, Kaindl AM, Garbade SF, Johannsen J, Ziegler A; SMArtCARE and Swiss-Reg-NMD study group. Weiß C, et al. Lancet Reg Health Eur. 2024 Oct 7;47:101092. doi: 10.1016/j.lanepe.2024.101092. eCollection 2024 Dec. Lancet Reg Health Eur. 2024. PMID: 39434961 Free PMC article.
Safety, tolerability, and efficacy of fasudil in amyotrophic lateral sclerosis (ROCK-ALS): a phase 2, randomised, double-blind, placebo-controlled trial.
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. Koch JC, et al. Among authors: gunther r. Lancet Neurol. 2024 Nov;23(11):1133-1146. doi: 10.1016/S1474-4422(24)00373-9. Lancet Neurol. 2024. PMID: 39424560 Free article. Clinical Trial.
SOD1 gene screening in ALS - frequency of mutations, patients' attitudes to genetic information and transition to tofersen treatment in a multi-center program.
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. Meyer T, et al. Among authors: gunther r. Amyotroph Lateral Scler Frontotemporal Degener. 2025 Feb;26(1-2):162-171. doi: 10.1080/21678421.2024.2401131. Epub 2024 Sep 13. Amyotroph Lateral Scler Frontotemporal Degener. 2025. PMID: 39268612 Free article.
Clinical and patient-reported outcomes and neurofilament response during tofersen treatment in SOD1-related ALS-A multicenter observational study over 18 months.
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. Meyer T, et al. Among authors: gunther r. Muscle Nerve. 2024 Sep;70(3):333-345. doi: 10.1002/mus.28182. Epub 2024 Jun 20. Muscle Nerve. 2024. PMID: 39031772
Smartphone-Based Assessment of Mobility and Manual Dexterity in Adult People with Spinal Muscular Atrophy.
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. Arteaga-Bracho E, et al. Among authors: gunther r. J Neuromuscul Dis. 2024;11(5):1049-1065. doi: 10.3233/JND-240004. J Neuromuscul Dis. 2024. PMID: 38995798 Free PMC article.
Patients' and caregivers' perception of multidimensional and palliative care in amyotrophic lateral sclerosis - protocol of a German multicentre study.
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. Linse K, et al. Among authors: gunther r. Neurol Res Pract. 2024 Jul 4;6(1):34. doi: 10.1186/s42466-024-00328-1. Neurol Res Pract. 2024. PMID: 38961496 Free PMC article.
Serum neurofilament light chain in distinct phenotypes of amyotrophic lateral sclerosis: A longitudinal, multicenter study.
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. Meyer T, et al. Among authors: gunther r. Eur J Neurol. 2024 Sep;31(9):e16379. doi: 10.1111/ene.16379. Epub 2024 Jun 10. Eur J Neurol. 2024. PMID: 38859579 Free PMC article.
Glycolic acid and D-lactate-putative products of DJ-1-restore neurodegeneration in FUS - and SOD1-ALS.
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. Pal A, et al. Among authors: gunther r. Life Sci Alliance. 2024 May 17;7(8):e202302535. doi: 10.26508/lsa.202302535. Print 2024 Aug. Life Sci Alliance. 2024. PMID: 38760174 Free PMC article.
Clinical Effectiveness of Newborn Screening for Spinal Muscular Atrophy: A Nonrandomized Controlled Trial.
Schwartz O, Vill K, Pfaffenlehner M, Behrens M, Weiß C, Johannsen J, Friese J, Hahn A, Ziegler A, Illsinger S, Smitka M, von Moers A, Kölbel H, Schreiber G, Kaiser N, Wilichowski E, Flotats-Bastardas M, Husain RA, Baumann M, Köhler C, Trollmann R, Schwerin-Nagel A, Eisenkölbl A, Schimmel M, Fleger M, Kauffmann B, Wiegand G, Baumgartner M, Rauscher C, Cirak S, Gläser D, Bernert G, Hagenacker T, Goldbach S, Probst-Schendzielorz K, Lochmüller H, Müller-Felber W, Schara-Schmidt U, Walter MC, Kirschner J, Pechmann A; SMARTCARE study group. Schwartz O, et al. JAMA Pediatr. 2024 Jun 1;178(6):540-547. doi: 10.1001/jamapediatrics.2024.0492. JAMA Pediatr. 2024. PMID: 38587854 Free PMC article. Clinical Trial.
5qSMA: standardised retrospective natural history assessment in 268 patients with four copies of SMN2.
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. Vill K, et al. Among authors: gunther r. J Neurol. 2024 May;271(5):2787-2797. doi: 10.1007/s00415-024-12188-5. Epub 2024 Feb 27. J Neurol. 2024. PMID: 38409538 Free PMC article.
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.
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. Wiesenfarth M, et al. Among authors: gunther r. EClinicalMedicine. 2024 Feb 15;69:102495. doi: 10.1016/j.eclinm.2024.102495. eCollection 2024 Mar. EClinicalMedicine. 2024. PMID: 38384337 Free PMC article.
Long-term efficacy and safety of nusinersen in adults with 5q spinal muscular atrophy: a prospective European multinational observational study.
Günther R, Wurster CD, Brakemeier S, Osmanovic A, Schreiber-Katz O, Petri S, Uzelac Z, Hiebeler M, Thiele S, Walter MC, Weiler M, Kessler T, Freigang M, Lapp HS, Cordts I, Lingor P, Deschauer M, Hahn A, Martakis K, Steinbach R, Ilse B, Rödiger A, Bellut J, Nentwich J, Zeller D, Muhandes MT, Baum T, Christoph Koch J, Schrank B, Fischer S, Hermann A, Kamm C, Naegel S, Mensch A, Weber M, Neuwirth C, Lehmann HC, Wunderlich G, Stadler C, Tomforde M, George A, Groß M, Pechmann A, Kirschner J, Türk M, Schimmel M, Bernert G, Martin P, Rauscher C, Meyer Zu Hörste G, Baum P, Löscher W, Flotats-Bastardas M, Köhler C, Probst-Schendzielorz K, Goldbach S, Schara-Schmidt U, Müller-Felber W, Lochmüller H, von Velsen O; SMArtCARE Study Group; Kleinschnitz C, Ludolph AC, Hagenacker T. Günther R, et al. Lancet Reg Health Eur. 2024 Feb 6;39:100862. doi: 10.1016/j.lanepe.2024.100862. eCollection 2024 Apr. Lancet Reg Health Eur. 2024. PMID: 38361750 Free PMC article.
A randomized double-blind clinical trial on safety and efficacy of tauroursodeoxycholic acid (TUDCA) as add-on treatment in patients affected by amyotrophic lateral sclerosis (ALS): the statistical analysis plan of TUDCA-ALS trial.
Lombardo FL, Spila Alegiani S, Mayer F, Cipriani M, Lo Giudice M, Ludolph AC, McDermott CJ, Corcia P, Van Damme P, Van den Berg LH, Hardiman O, Nicolini G, Vanacore N, Dickie B, Albanese A, Puopolo M; TUDCA-ALS Study Group. Lombardo FL, et al. Trials. 2023 Dec 5;24(1):792. doi: 10.1186/s13063-023-07638-w. Trials. 2023. PMID: 38053196 Free PMC article. Clinical Trial.
Classifying flow cytometry data using Bayesian analysis helps to distinguish ALS patients from healthy controls.
Räuber S, Nelke C, Schroeter CB, Barman S, Pawlitzki M, Ingwersen J, Akgün K, Günther R, Garza AP, Marggraf M, Dunay IR, Schreiber S, Vielhaber S, Ziemssen T, Melzer N, Ruck T, Meuth SG, Herty M. Räuber S, et al. Among authors: gunther r. Front Immunol. 2023 Aug 1;14:1198860. doi: 10.3389/fimmu.2023.1198860. eCollection 2023. Front Immunol. 2023. PMID: 37600819 Free PMC article.
Economic evaluation of Motor Neuron Diseases: a nationwide cross-sectional analysis in Germany.
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. Heinrich F, et al. Among authors: gunther r. J Neurol. 2023 Oct;270(10):4922-4938. doi: 10.1007/s00415-023-11811-1. Epub 2023 Jun 25. J Neurol. 2023. PMID: 37356024 Free PMC article.
Biomarkers in 5q-associated spinal muscular atrophy-a narrative review.
Lapp HS, Freigang M, Hagenacker T, Weiler M, Wurster CD, Günther R. Lapp HS, et al. Among authors: gunther r. J Neurol. 2023 Sep;270(9):4157-4178. doi: 10.1007/s00415-023-11787-y. Epub 2023 Jun 8. J Neurol. 2023. PMID: 37289324 Free PMC article. Review.
Neurofilament light-chain response during therapy with antisense oligonucleotide tofersen in SOD1-related ALS: Treatment experience in clinical practice.
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. Meyer T, et al. Among authors: gunther r. Muscle Nerve. 2023 Jun;67(6):515-521. doi: 10.1002/mus.27818. Epub 2023 Apr 3. Muscle Nerve. 2023. PMID: 36928619
Performance of serum neurofilament light chain in a wide spectrum of clinical courses of amyotrophic lateral sclerosis-a cross-sectional multicenter study.
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. Meyer T, et al. Among authors: gunther r. Eur J Neurol. 2023 Jun;30(6):1600-1610. doi: 10.1111/ene.15773. Epub 2023 Mar 26. Eur J Neurol. 2023. PMID: 36899448
[Gene Therapies in Motor Neuron Diseases ALS and SMA].
Günther R. Günther R. Fortschr Neurol Psychiatr. 2023 Apr;91(4):153-163. doi: 10.1055/a-2002-5215. Epub 2023 Feb 23. Fortschr Neurol Psychiatr. 2023. PMID: 36822211 Review. German.
Improvements in Walking Distance during Nusinersen Treatment - A Prospective 3-year SMArtCARE Registry Study.
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. Pechmann A, et al. Among authors: gunther r. J Neuromuscul Dis. 2023;10(1):29-40. doi: 10.3233/JND-221600. J Neuromuscul Dis. 2023. PMID: 36565133 Free PMC article.
ALSFRS-R-SE: an adapted, annotated, and self-explanatory version of the revised amyotrophic lateral sclerosis functional rating scale.
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. Maier A, et al. Among authors: gunther r. Neurol Res Pract. 2022 Dec 15;4(1):60. doi: 10.1186/s42466-022-00224-6. Neurol Res Pract. 2022. PMID: 36522775 Free PMC article.
Correction to: Short-term safety results from compassionate use of risdiplam in patients with spinal muscular atrophy in Germany.
Hahn A, Günther R, Ludolph A, Schwartz O, Trollmann R, Weydt P, Weiler M, Neuland K, Schwaderer MS, Hagenacker T; Risdiplam Compassionate Use Program Group. Hahn A, et al. Among authors: gunther r. Orphanet J Rare Dis. 2022 Oct 25;17(1):387. doi: 10.1186/s13023-022-02548-7. Orphanet J Rare Dis. 2022. PMID: 36284315 Free PMC article. No abstract available.
Improved upper limb function in non-ambulant children with SMA type 2 and 3 during nusinersen treatment: a prospective 3-years SMArtCARE registry study.
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. Pechmann A, et al. Among authors: gunther r. Orphanet J Rare Dis. 2022 Oct 23;17(1):384. doi: 10.1186/s13023-022-02547-8. Orphanet J Rare Dis. 2022. PMID: 36274155 Free PMC article.
Glial fibrillary acidic protein in cerebrospinal fluid of patients with spinal muscular atrophy.
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. Freigang M, et al. Among authors: gunther r. Ann Clin Transl Neurol. 2022 Sep;9(9):1437-1448. doi: 10.1002/acn3.51645. Epub 2022 Aug 11. Ann Clin Transl Neurol. 2022. PMID: 35951535 Free PMC article.
Remote digital assessment of amyotrophic lateral sclerosis functional rating scale - a multicenter observational study.
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. Meyer T, et al. Among authors: gunther r. Amyotroph Lateral Scler Frontotemporal Degener. 2023 May;24(3-4):175-184. doi: 10.1080/21678421.2022.2104649. Epub 2022 Aug 1. Amyotroph Lateral Scler Frontotemporal Degener. 2023. PMID: 35912984 Free article.
Effect of nusinersen on motor, respiratory and bulbar function in early-onset spinal muscular atrophy.
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. Pechmann A, et al. Brain. 2023 Feb 13;146(2):668-677. doi: 10.1093/brain/awac252. Brain. 2023. PMID: 35857854
Use and subjective experience of the impact of motor-assisted movement exercisers in people with amyotrophic lateral sclerosis: a multicenter observational study.
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. Maier A, et al. Among authors: gunther r. Sci Rep. 2022 Jun 10;12(1):9657. doi: 10.1038/s41598-022-13761-6. Sci Rep. 2022. PMID: 35688956 Free PMC article.
Affection of Respiratory Muscles in ALS and SMA.
Hermann W, Langner S, Freigang M, Fischer S, Storch A, Günther R, Hermann A. Hermann W, et al. Among authors: gunther r. J Clin Med. 2022 Feb 22;11(5):1163. doi: 10.3390/jcm11051163. J Clin Med. 2022. PMID: 35268254 Free PMC article.
Validity and reliability of the German multidimensional fatigue inventory in spinal muscular atrophy.
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. Binz C, et al. Among authors: gunther r. Ann Clin Transl Neurol. 2022 Mar;9(3):351-362. doi: 10.1002/acn3.51520. Epub 2022 Feb 22. Ann Clin Transl Neurol. 2022. PMID: 35191616 Free PMC article.
Safety and Effectiveness of Long-term Intravenous Administration of Edaravone for Treatment of Patients With Amyotrophic Lateral Sclerosis.
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). Witzel S, et al. Among authors: gunther r. JAMA Neurol. 2022 Feb 1;79(2):121-130. doi: 10.1001/jamaneurol.2021.4893. JAMA Neurol. 2022. PMID: 35006266 Free PMC article. Clinical Trial.
Informal Caregiving in Amyotrophic Lateral Sclerosis (ALS): A High Caregiver Burden and Drastic Consequences on Caregivers' Lives.
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. Schischlevskij P, et al. Among authors: gunther r. Brain Sci. 2021 Jun 4;11(6):748. doi: 10.3390/brainsci11060748. Brain Sci. 2021. PMID: 34200087 Free PMC article.
Treatment expectations and perception of therapy in adult patients with spinal muscular atrophy receiving nusinersen.
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. Meyer T, et al. Among authors: gunther r. Eur J Neurol. 2021 Aug;28(8):2582-2595. doi: 10.1111/ene.14902. Epub 2021 Jun 16. Eur J Neurol. 2021. PMID: 33960080
A Nation-Wide, Multi-Center Study on the Quality of Life of ALS Patients in Germany.
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. Peseschkian T, et al. Among authors: gunther r. Brain Sci. 2021 Mar 14;11(3):372. doi: 10.3390/brainsci11030372. Brain Sci. 2021. PMID: 33799476 Free PMC article.
Serum creatine kinase and creatinine in adult spinal muscular atrophy under nusinersen treatment.
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. Freigang M, et al. Among authors: gunther r. Ann Clin Transl Neurol. 2021 May;8(5):1049-1063. doi: 10.1002/acn3.51340. Epub 2021 Mar 31. Ann Clin Transl Neurol. 2021. PMID: 33792208 Free PMC article.
Treatment satisfaction in 5q-spinal muscular atrophy under nusinersen therapy.
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. Osmanovic A, et al. Among authors: gunther r. Ther Adv Neurol Disord. 2021 Mar 5;14:1756286421998902. doi: 10.1177/1756286421998902. eCollection 2021. Ther Adv Neurol Disord. 2021. PMID: 33747131 Free PMC article.
Challenges and opportunities for Multi-National Investigator-Initiated clinical trials for ALS: European and United States collaborations.
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. Lingor P, et al. Among authors: gunther r. Amyotroph Lateral Scler Frontotemporal Degener. 2021 Aug;22(5-6):419-425. doi: 10.1080/21678421.2021.1879866. Epub 2021 Feb 3. Amyotroph Lateral Scler Frontotemporal Degener. 2021. PMID: 33533663 Free PMC article.
One nerve suffices: A clinically guided nerve ultrasound protocol for the differentiation of multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis (ALS).
Loewenbrück KF, Werner R, Günther R, Dittrich M, Klingenberger R, Reichmann H, Storch A, Hermann A. Loewenbrück KF, et al. Among authors: gunther r. J Neurol. 2021 Apr;268(4):1495-1507. doi: 10.1007/s00415-020-10323-6. Epub 2020 Dec 23. J Neurol. 2021. PMID: 33355881 Free PMC article.
TDP-43 as structure-based biomarker in amyotrophic lateral sclerosis.
Beyer L, Günther R, Koch JC, Klebe S, Hagenacker T, Lingor P, Biesalski AS, Hermann A, Nabers A, Gold R, Tönges L, Gerwert K. Beyer L, et al. Among authors: gunther r. Ann Clin Transl Neurol. 2021 Jan;8(1):271-277. doi: 10.1002/acn3.51256. Epub 2020 Dec 2. Ann Clin Transl Neurol. 2021. PMID: 33263951 Free PMC article.
Nusinersen in adults with 5q spinal muscular atrophy: a non-interventional, multicentre, observational cohort study.
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. Hagenacker T, et al. Among authors: gunther r. Lancet Neurol. 2020 Apr;19(4):317-325. doi: 10.1016/S1474-4422(20)30037-5. Epub 2020 Mar 18. Lancet Neurol. 2020. PMID: 32199097
Routine Cerebrospinal Fluid (CSF) Parameters in Patients With Spinal Muscular Atrophy (SMA) Treated With Nusinersen.
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. Wurster CD, et al. Among authors: gunther r. Front Neurol. 2019 Nov 7;10:1179. doi: 10.3389/fneur.2019.01179. eCollection 2019. Front Neurol. 2019. PMID: 31787927 Free PMC article.
Phenotypes and malignancy risk of different FUS mutations in genetic amyotrophic lateral sclerosis.
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. Naumann M, et al. Among authors: gunther r. Ann Clin Transl Neurol. 2019 Dec;6(12):2384-2394. doi: 10.1002/acn3.50930. Epub 2019 Nov 4. Ann Clin Transl Neurol. 2019. PMID: 31682085 Free PMC article.
Neurofilament light chain in serum of adolescent and adult SMA patients under treatment with nusinersen.
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. Wurster CD, et al. Among authors: gunther r. J Neurol. 2020 Jan;267(1):36-44. doi: 10.1007/s00415-019-09547-y. Epub 2019 Sep 24. J Neurol. 2020. PMID: 31552549
Neurochemical markers in CSF of adolescent and adult SMA patients undergoing nusinersen treatment.
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. Wurster CD, et al. Among authors: gunther r. Ther Adv Neurol Disord. 2019 May 10;12:1756286419846058. doi: 10.1177/1756286419846058. eCollection 2019. Ther Adv Neurol Disord. 2019. PMID: 31205491 Free PMC article.
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.
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; ROCK-ALS Investigators. Lingor P, et al. Among authors: gunther r. Front Neurol. 2019 Mar 27;10:293. doi: 10.3389/fneur.2019.00293. eCollection 2019. Front Neurol. 2019. PMID: 30972018 Free PMC article.
Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation.
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. Naumann M, et al. Among authors: gunther r. Nat Commun. 2018 Jan 23;9(1):335. doi: 10.1038/s41467-017-02299-1. Nat Commun. 2018. PMID: 29362359 Free PMC article.
Non-Motor Symptoms in Patients Suffering from Motor Neuron Diseases.
Günther R, Richter N, Sauerbier A, Chaudhuri KR, Martinez-Martin P, Storch A, Hermann A. Günther R, et al. Front Neurol. 2016 Jul 25;7:117. doi: 10.3389/fneur.2016.00117. eCollection 2016. Front Neurol. 2016. PMID: 27504105 Free PMC article.