Familial strio-pallido-dentate calcification

Introduction. Calcification of the dentate nucleus of the cerebellum may be seen on cerebral CT in 0.3-0.5% of patients with no symptoms or extra-pyramidal signs. Although there are many causes, some cases show a family incidence. Clinical cases. We present two cases, mother and son, in whom the predominant clinical findings were poor language and a bilateral extrapyramidal syndrome. Laboratory tests, hormone and immunological studies were normal. In both patients cerebral CT showed bilateral calcification of the basal nuclei and dentate nuclei of the cerebellum. Discussion. Our patients fulfilled the clinical and neuro-imaging criteria described in familial strio-pallido-dentate calcification There is no relationship between the duration and intensity of symptoms, and the extension of the calcification However, all patients with calcification show clinical features. The predominant features are poor language and a bilateral extra-pyramidal syndrome These observations indicate that in patients with calcification of the basal ganglia and dentate nuclei of the cerebellum it is necessary to study first degree relatives in or del to identify the condition [REV NEUROL 1997; 25: 1213-5].