Non-Ataxic Presenting Symptoms of Dominant Ataxias

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.