Clinical report on and CT findings in two siblings with bilateral striatal necrosis

Two siblings, a 13-year-old girl and a 9-year-old boy, presented a similar progressive extrapyramidal disorder. The onsets were at the age of 4 years and at that of 2 1/2 years respectively, and a certain febrile illness had preceded it for two or three months in both cases. The major clinical features were progressive gait disturbance, dysarthria, and dystonia; they were associated with secondary skeletal deformities in the sister and with abnormal ocular movements in the brother. The CT findings, essentially similar in both cases, were characterized by symmetrical hypodensity lesions and an atrophy of the corpora striata, namely, the putamen and the caudate nucleus. Based on the clinical features and the CT findings, and on a comparison with the previous clinico-pathological reports in the literature, the present cases were diagnosed as bilateral striatal necrosis. The disorder termed ''bilateral striatal necrosis'' has not been widely known; this report stresses the great usefulness of CT examination in the clinical diagnosis of this rare disorder. (author)