title: Non-Ataxic Presenting Symptoms of Dominant Ataxias.
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.
authors.affiliation: Department of Medicine (Neuroscience), Monash University, Alfred Hospital Campus, Commercial Road, Melbourne, VIC, 3004, Australia. elsdon.storey@monash.edu.|Department of Neuroscience, Alfred Hospital, Commercial Road, Melbourne, VIC, 3004, Australia. elsdon.storey@monash.edu.
authors.forename: Elsdon
authors.initials: E
authors.lastname: Storey
mesh_terms: D001259:Ataxia / Q000150:complications*; D003638:Deafness / Q000209:etiology*; D006801:Humans; D001523:Mental Disorders / Q000209:etiology*; D009069:Movement Disorders / Q000209:etiology*; D012640:Seizures / Q000209:etiology; D014786:Vision Disorders / Q000209:etiology*
keywords: Clinical presentation; Dominant ataxias; SCAs; Spinocerebellar ataxias