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Non-Ataxic Presenting Symptoms of Dominant Ataxias

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Abstract

While the onset of a dominantly inherited ataxia is typically taken to be the onset of gait ataxia, a wide range of other symptoms related to central and/or peripheral nervous system impairment, or even to non-neurological involvement, can be the presenting feature. Knowledge of these is fairly robust for the commonest spinocerebellar ataxias (SCAs 1, 2, 3 and 6) and for those where a striking non-ataxic presentation is the norm (SCAs 7 and 12), but the literature is potentially misleading in the rarer dominant ataxias. This review summarises what is currently known of these non-ataxic presentations and outlines and explains the difficulties associated with determining non-ataxic presentations of dominant ataxias. The relevant literature was surveyed, including systematic reviews (where available) and case reports. Non-ataxic presentations of dominant ataxias are classified by symptom.

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The author declares no conflict of interest for this submission.

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Authors and Affiliations

  1. Department of Medicine (Neuroscience), Monash University, Alfred Hospital Campus, Commercial Road, Melbourne, VIC, 3004, Australia

    Elsdon Storey

  2. Department of Neuroscience, Alfred Hospital, Commercial Road, Melbourne, VIC, 3004, Australia

    Elsdon Storey

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  1. Elsdon Storey
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Correspondence to Elsdon Storey.

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Storey, E. Non-Ataxic Presenting Symptoms of Dominant Ataxias. Cerebellum 15, 26–29 (2016). https://doi.org/10.1007/s12311-015-0721-5

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  • DOI: https://doi.org/10.1007/s12311-015-0721-5

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