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Consensus Paper: Management of Degenerative Cerebellar Disorders

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Abstract

Treatment of motor symptoms of degenerative cerebellar ataxia remains difficult. Yet there are recent developments that are likely to lead to significant improvements in the future. Most desirable would be a causative treatment of the underlying cerebellar disease. This is currently available only for a very small subset of cerebellar ataxias with known metabolic dysfunction. However, increasing knowledge of the pathophysiology of hereditary ataxia should lead to an increasing number of medically sensible drug trials. In this paper, data from recent drug trials in patients with recessive and dominant cerebellar ataxias will be summarized. There is consensus that up to date, no medication has been proven effective. Aminopyridines and acetazolamide are the only exception, which are beneficial in patients with episodic ataxia type 2. Aminopyridines are also effective in a subset of patients presenting with downbeat nystagmus. As such, all authors agreed that the mainstays of treatment of degenerative cerebellar ataxia are currently physiotherapy, occupational therapy, and speech therapy. For many years, well-controlled rehabilitation studies in patients with cerebellar ataxia were lacking. Data of recently published studies show that coordinative training improves motor function in both adult and juvenile patients with cerebellar degeneration. Given the well-known contribution of the cerebellum to motor learning, possible mechanisms underlying improvement will be outlined. There is consensus that evidence-based guidelines for the physiotherapy of degenerative cerebellar ataxia need to be developed. Future developments in physiotherapeutical interventions will be discussed including application of non-invasive brain stimulation.

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Authors and Affiliations

  1. Department of Cognitive Neurology, Hertie Institute for Clinical Brain Research and Centre for Integrative Neuroscience, Tübingen, Germany

    W. Ilg

  2. Department of Neuroscience, Kennedy Krieger Institute, Johns Hopkins University, Baltimore, MD, USA

    A. J. Bastian & P. Celnik

  3. Department of Neurology, Medical University Innsbruck, Innsbruck, Austria

    S. Boesch & W. Nachbauer

  4. Department of Neurology, University Clinic Essen, University Duisburg—Essen, Hufelandstrasse 55, 45138, Essen, Germany

    R. G. Burciu, J. Claaßen & D. Timmann

  5. Department of Neurology and German Center for Vertigo and Balance Disorders, University Hospital Munich, Munich, Germany

    J. Claaßen, K. Feil, R. Kalla, M. Strupp & J. Teufel

  6. Neurorehabilitation Research Institute, Morinomiya Hospital, Osaka, Japan

    I. Miyai

  7. Department of Neurodegeneration, Hertie Institute for Clinical Brain Research and Centre of Neurology, Tübingen, Germany

    L. Schöls & M. Synofzik

  8. German Research Center for Neurodegenerative Diseases (DZNE), Tübingen, Germany

    L. Schöls & M. Synofzik

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  1. W. Ilg
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Correspondence to D. Timmann.

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Ilg, W., Bastian, A.J., Boesch, S. et al. Consensus Paper: Management of Degenerative Cerebellar Disorders. Cerebellum 13, 248–268 (2014). https://doi.org/10.1007/s12311-013-0531-6

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