Cerebellar ataxia in childhood: a review of clinical features, investigative findings and aetiology in 30 cases.

The symptoms, neurological deficits and aetiology of cerebellar ataxia were reviewed in 30 children aged from 4 to 16 years, who had been extensively investigated. Fourteen (46.6%) children had perinatal problems, and in 6 (20%), ataxia resulted from these perinatal events. Besides unsteady gait, a common presenting complaint was delayed motor milestones, noted in 15 (50%) patients. In most children (76.6%), clinical examination indicated involvement of more than one of the three main cerebellar divisions, viz archicerebellum, paleocerebellum and neocerebellum. Hypotonia, found in 11 (36.6%) children, was the third most common sign after gait and truncal ataxia. A variety of aetiological factors was identified, the two most frequent being hydrocephalus in 7 (23.3%) children, and perinatal problems in 6 (20%). The findings are discussed against the background of current literature on the subject.