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December 03, 2020Research

Fampridine and Acetazolamide in EA2 and Related Familial EA: A Prospective Randomized Placebo-controlled Trial

Carolin Muth, Julian Teufel, Ludger Schöls, Matthis Synofzik, Christiana Franke, Dagmar Timmann, Ulrich Mansmann, Michael Strupp
First published December 3, 2020, DOI: https://doi.org/10.1212/CPJ.0000000000001017
Carolin Muth
1 Department of Neurology and German Center for Vertigo and Balance Disorders (DSGZ), Ludwig Maximilians University, Munich, LMU University Hospital, Campus Grosshadern, Germany
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Julian Teufel
1 Department of Neurology and German Center for Vertigo and Balance Disorders (DSGZ), Ludwig Maximilians University, Munich, LMU University Hospital, Campus Grosshadern, Germany
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Ludger Schöls
2 Department of Neurology and Hertie-Institute for Clinical Brain Research, Eberhard Karls University and German Center for Neurodegenerative Diseases (DZNE), Tübingen, Germany
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Matthis Synofzik
2 Department of Neurology and Hertie-Institute for Clinical Brain Research, Eberhard Karls University and German Center for Neurodegenerative Diseases (DZNE), Tübingen, Germany
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Christiana Franke
3 Department of Neurology, Charité-Universitätsmedizin Berlin, Berlin, Germany, formerly Department of Neurology, University of Dresden, Dresden, Germany
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Dagmar Timmann
4 Department of Neurology, Essen University Hospital, University of Duisburg-Essen, Essen, Germany
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Ulrich Mansmann
5 Department of Medical Information Sciences, Biometry, and Epidemiology (IBE), Ludwig Maximilian University, Munich, Germany
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Michael Strupp
1 Department of Neurology and German Center for Vertigo and Balance Disorders (DSGZ), Ludwig Maximilians University, Munich, LMU University Hospital, Campus Grosshadern, Germany
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Fampridine and Acetazolamide in EA2 and Related Familial EA: A Prospective Randomized Placebo-controlled Trial
Carolin Muth, Julian Teufel, Ludger Schöls, Matthis Synofzik, Christiana Franke, Dagmar Timmann, Ulrich Mansmann, Michael Strupp
Neurol Clin Pract Dec 2020, 10.1212/CPJ.0000000000001017; DOI: 10.1212/CPJ.0000000000001017

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Abstract

Objective We determine the efficacy and safety of the treatment with prolonged-release 4-aminopyridine (fampridine) and acetazolamide for patients with episodic ataxia type 2 (EA2). Therefore, 30 patients with EA2 were treated with a random sequence of fampridine, acetazolamide, and placebo in a three-period crossover trial.

Methods A total of 30 patients with EA2 (eight female; aged 20-71 years; 18 genetically confirmed, four with a positive family history, eight with the clinical diagnosis) were enrolled in this phase III, randomised, double-blind, placebo-controlled, three-period crossover trial. Each period lasted 12 weeks with a four-week washout-period. Each patient received a random sequence of 20 mg/d fampridine, 750 mg/d acetazolamide, and placebo. The primary endpoint was the number of attacks during the last 30 days within the 12-week treatment-period. Participants, caregivers, and those assessing the outcomes were blinded to the intervention.

Results Compared to placebo, fampridine reduced the number of attacks to 63% (95% CI 54% - 74%) and acetazolamide to 52% (95% CI 46% - 60%). A total of 39 (26.5%) adverse events were observed under treatment with fampridine (mostly tingling paraesthesia, and fatigue), 66 (44.9%) happened under acetazolamide (mostly taste disturbance, gastrointestinal complaints), and 42 (28.6%) under placebo (mostly gastrointestinal complaints).

Conclusion Both fampridine and acetazolamide significantly reduce the number of attacks in patients with EA2 and related EA in comparison to placebo. Fampridine 10 mg twice daily had fewer side effects than acetazolamide 250 mg three times daily.

  • Received June 25, 2020.
  • Accepted October 22, 2020.
  • © 2020 American Academy of Neurology

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