[Non-progressive congenital cerebellar ataxia].

The authors studied the main clinical and genetic aspects of congenital cerebellar ataxia in children, from the data obtained in 121 cases. The case reports were divided into 2 groups, according to the severity of the static ataxia. In the absence of absolute criteria allowing the recognition of the various forms of congenital cerebellar ataxia, they give the following genetic counsel: in cases with congenital cerebellar ataxia of unknown origin, the risk or recurrence is higher in cases with severe static ataxia (near 25%) but not negligible in the other types of ataxia, especially in cases associated with severe mental retardation (risk between 5 and 10%).