Striatal degeneration in childhood.

Abstract

SUMMARY The clinical features, and the radiological and neuropathological findings of 3 unrelated children with striatal degeneration are presented. In one case the father had recently developed choreiform movements while in the other two the family history was negative for neurological disorders. Two patients had juvenile onset of psychiatric symptoms, seizures, and rigidity. The 3rd child presented with focal seizures at 9 weeks of age. The neuropathological findings are virtually identical in all 3 cases. The classification of striatal degeneration in childhood is discussed.

Post‐streptococcal autoimmune dystonia with isolated bilateral striatal necrosis

“…This uncommon disease entity consists of two distinct subgroups. One subgroup presents insidiously in the first year of life with a progressive dystonia (Erdohazi et al 1979, Mito et al 1986). In these cases, a family history is frequently present and the outcome is often poor.…”