Post #33326 - Ataxia UK

Current and recently completed clinical trials in ataxias

 

Here you will find a summary of recently published and ongoing trials. This summary is not exhaustive, but illustrates that a number of types of drug trials have been completed, or are currently taking place. There are also many more trials in the pipeline, either to confirm the results of pilot studies listed or exploring new potential treatments (see www.clinical trials.gov for clinical trials information). 

This table was updated in June 2026. 

 

Treatment  Ataxia type  Investigator or company  Clinical stage and clinical research references 
Omaveloxolone   Friedreich’s ataxia  Biogen  Phase 3 published [1, 2, 3] 
Etravirine (drug repurposing)  Friedreich’s ataxia  Scientific Institute IRCCS, Italy  Phase 2 published [4] 
Vatiquinone  Friedreich’s ataxia  PTC Therapeutics  FDA did not approve in 2026. PTC plans to conduct another trial. 
Nomlabofusp  Friedreich’s ataxia  Larimar  Open label study ongoing in US 
LX2006 (gene therapy)  Friedreich’s ataxia  Lexeo Therapeutics  Phase 1/2 ongoing in US 
SGT-212 (gene therapy)  Friedreich’s ataxia  Solid Biosciences  Phase 1b ongoing in US 
DT219P2  Friedreich’s ataxia  Design Therapeutics  Phase 1/2 ongoing in Australia 
Transcranial stimulation (multiple types)  Mixed ataxias  Multiple  Multiple studies with mixed results  [5, 6, 7, 8, 9, 10, 11, 12] 
4-aminopyridine (4-AP)  SCA27B  Solaxa and an investigator-led study at Assistance Publique - Hôpitaux de Paris, France  Phase 3 ongoing, other clinical results and observations published [13, 14, 15] 
ARO-ATXN2 (gene silencing)  SCA2  Arrowhead Therapeutics  Phase 1 ongoing worldwide, not yet including sites in UK 
VO659 (antisense oligonucleotide)  SCA1 and SCA3  VICO Therapeutics  Phase 1/2a ongoing including site in UK 
Troriluzole  SCAs  Biohaven  Phase 3 trial complete, discussions with FDA ongoing. 

 

References

 

[1]   D. R. Lynch, M. P. Chin, S. Boesch, M. B. Delatycki, P. Giunti, A. Goldsberry, J. C. Hoyle, C. Mariotti, K. D. Mathews, W. Nachbauer, M. O'Grady, S. Perlman, S. H. Subramony, G. Wilmot, T. Zesiewicz and C. J. Meyer, “Efficacy of Omaveloxolone in Friedreich's Ataxia: Delayed-Start Analysis of the MOXIe Extension,” Movement Disorders, vol. 38, no. 2, pp. 313-320, 2023.  
[2]   D. R. Lynch, M. P. Chin, M. B. Delatycki, S. H. Subramony, M. Corti, J. C. Hoyle, S. Boesch, W. Nachbauer, C. Mariotti, K. D. Mathews, P. Giunti, G. Wilmot, T. Zesiewicz, S. Perlman, A. Goldsberry, M. O'Grady and C. J. Meyer, “Safety and Efficacy of Omaveloxolone in Friedreich Ataxia (MOXIe Study),” Annals of Neurology, vol. 89, no. 2, pp. 212-225, 2021.  
[3]   D. R. Lynch, A. Goldsberry, C. Rummey, J. Farmer, S. Boesch, M. B. Delatycki, P. Giunti, J. C. Hoyle, C. Mariotti, K. D. Mathews, W. Nachbauer, S. Perlman, S. H. Subramony, G. Wilmot, T. Zesiewicz, L. Weissfeld and C. Meyer, “Propensity matched comparison of omaveloxolone treatment to Friedreich ataxia natural history data,” Annals of clinical and translational neurology, vol. 11, no. 1, pp. 4-16, 2024.  
[4]   G. Paparella, C. Straga, N. Pesenti, V. D. Molin, G. A. Martorel, V. Merotto, C. Genova, A. Piazza, G. Piccoli, E. Panzeri, A. Rufini, R. Testi and A. Martinuzzi, “A Pilot Phase 2 Randomized Trial to Evaluate the Safety and Potential Efficacy of Etravirine in Friedreich Ataxia Patients,” Children (Basel), vol. 11, no. 8, p. 958, 2024.  
[5]   Y. Shi, G. Zou, Z. Chen, L. Wan, L. Peng, H. Peng, L. Shen, K. Xia, R. Qiu, B. Tang and H. Jiang, “Efficacy of cerebellar transcranial magnetic stimulation in spinocerebellar ataxia type 3: a randomized, single-blinded, controlled trial,” Journal of Neurology, vol. 270, no. 11, pp. 5372-5379, 2023.  
[6]   R. P. P. W. M. Maas, S. Teerenstra, I. Toni, T. Klockgether, D. J. L. G. Schutter and B. P. C. van de Warrenburg, “Cerebellar Transcranial Direct Current Stimulation in Spinocerebellar Ataxia Type 3: a Randomized, Double-Blind, Sham-Controlled Trial,” Neurotherapeutics, vol. 19, no. 4, pp. 1259-1272, 2022.  
[7]   G. Naeije, A. Rovai, V. Destrebecq, N. Trotta and X. De Tiege, “Anodal Cerebellar Transcranial Direct Current Stimulation Reduces Motor and Cognitive Symptoms in Friedreich's Ataxia: A Randomized, Sham-Controlled Trial,” Movement Disorders, vol. 38, no. 8, pp. 1443-1450, 2023.  
[8]   I. Libri, V. Cantoni, A. Benussi, J. Rivolta, C. Ferrari, R. Fancellu, M. Synofzik, A. Alberici, A. Padovani and B. Borroni, “Comparing Cerebellar tDCS and Cerebellar tACS in Neurodegenerative Ataxias Using Wearable Sensors: A Randomized, Double-Blind, Sham-Controlled, Triple-Crossover Trial,” Cerebellum, vol. 23, no. 2, pp. 570-578, 2024.  
[9]   R. Brito, J. V. Fabricio, A. Araujo, G. Barreto, A. Baltar and K. Monte-Silva, “Single-Session Cerebellar Transcranial Direct Current Stimulation Improves Postural Stability and Reduces Ataxia Symptoms in Spinocerebellar Ataxia,” Cerebellum, vol. 23, no. 5, pp. 1993-2002, 2024.  
[10]   X. Chen, X. Liu, W. Lin, L. Zhang, X. Cheng, Z. Huang, W. Zhang, H. Zeng, Y. Lian, Y. Zhang, M. Li, N. Chen, S. Huang, Z. Wang, X. Wang, Z. Liu, R. Yuan, X. Chen, Z. Ye, B. Cheng, Y. Zhang, Q. Chen, D. Wang, J. Ni, N. Wang, Y. Fu and S. Gan, “Transcranial alternating current stimulation for treating spinocerebellar ataxia type 3: A randomized controlled trial,” Cell reports. Medicine, vol. 6, no. 6, p. 102162, 2025.  
[11]   M. Grobe-Einsler, F. Bork, A. Faikus, R. Hurlemann and O. Kaut, “Effects of cerebellar repetitive transcranial magnetic stimulation plus physiotherapy in spinocerebellar ataxias - A randomized clinical trial,” CNS neuroscience and therapeutics, vol. 30, no. 6, p. e14797, 2024.  
[12]   A. Sanna, M. Pau, M. Porta, G. Pilia, V. Secci, E. Cartella, A. Demattia, A. Paribello, G. Cossu, A. Milia and P. Tacconi, “Efficacy of Cerebellar Transcranial Direct Current Stimulation in Degenerative Ataxia. A Sham-Controlled Clinical and Quantitative Analysis,” Cerebellum, vol. 25, no. 1, p. 11, 2026.  
[13]   J. Seemann, A. Traschutz, W. Ilg and M. Synofzik, “4-Aminopyridine improves real-life gait performance in SCA27B on a single-subject level: a prospective n-of-1 treatment experience,” Journal of Neurology, vol. 270, no. 11, pp. 5629-5634, 2023.  
[14]   D. Pellerin, F. Heindl, C. Wilke, M. C. Danzi, A. Traschutz, C. Ashton, M.-J. Dicaire, A. Cuillerier, G. Del Gobbo, K. M. Boycott, J. Claassen, D. Rujescu, A. M. Hartmann, S. Zuchner, B. Brais, M. Strupp and M. Synofzik, “GAA-FGF14 disease: defining its frequency, molecular basis, and 4-aminopyridine response in a large downbeat nystagmus cohort,” EBioMedicine, vol. 102, no. 105076, 2024.  
[15]   C. Wilke, D. Pellerin, D. Mengel, A. Traschutz, M. C. Danzi, M.-J. Dicaire, M. Neumann, H. Lerche, B. Bender, H. Houlden, S. Zuchner, L. Schols, B. Brais and M. Synofzik, “GAA-FGF14 ataxia (SCA27B): phenotypic profile, natural history progression and 4-aminopyridine treatment response,” Brain, vol. 146, no. 10, pp. 4144-4157, 2023.  

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