Acute neurological dysfunction associated with destructive lesions of the basal ganglia in children

Abstract

Three children ranging in age from 1 to 4 1/2 years suffered an acute illness with disturbance of consciousness followed by motor rigidity, loss of spontaneous movements and language, axial hypotonia, and a stereotyped response to any kind of stimulus. In two cases, images on computerized tomography were consistent with necrosis of the lenticular and caudate nuclei. The acute onset was followed by later improvement and stabilization. One child died, probably not as a direct consequence of the neurological disorder, autopsy showed bilateral necrosis of the putamina. We suggest that these three patients, together with two previously reported cases, constitute a clinically and radiologically recognizable subgroup among the heterogeneous disorder that produce bilateral striatal necrosis in children. The cause of the syndrome is unknown.