Acute neurologic dysfunction associated with destructive lesions of the basal ganglia: A benign form of infantile bilateral striatal necrosis

Abstract

In 1982 Gouti6res and Aicardi ~ described three patients with a syndrome characterized by (1) an abrupt clinical onset after an infectious illness, (2) impaired consciousness, axial hypotonia, motor rigidity, and abnormal movements and postures, (3) sustained improvement of neurologic status, and (4) bilateral low-density lesions of the basal ganglia on the computed tomographie scan. Three similar patients also have been repor ted) 4 It is likely that some other patients described before the advent of modern neuroimaging techniques had this same syndrome) Huntington chorea, Wilson disease, Hallervorden-Spatz syndrome, mitochondrial eneephalomyopathies including Leigh syndrome, familial striatal necrosis, head trauma, hypoxia-ischemia, and various intoxications can occur in childhood with acute or subaeute neurologie dysfunction associated with bilateral low-density lesions in the basal ganglia. The outcome of all of them, however, is poorer than that of acute neurologic dysfunction associated with destructive lesions of the basal ganglia, as described by Gouti6res and Aicardi. 1 We describe two patients with A D B G who had a complete clinical recovery. The CT scan findings in the acute phase of the disease were identical in both patients and supported the diagnosis. Bilateral, symmetric, necrotic lesions of the basal ganglia were found in the magnetic resonance imaging studies from the early stages of the disease and persisted in our second patient up to 5 years later.