Infantile Bilateral Striatal Necrosis

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Abstract
• Two cases of infantile bilateral striatal necrosis (IBSN) were studied retrospectively, and the literature was reviewed. The two children had presented with progressive neurologic signs of involuntary movements or muscle hypertonia from infancy. Initial computed tomography scans showed mild atrophy of the caudate nuclei or basal ganglia, and the neuropathologic examination revealed diffuse neuronal loss with some patchy preservation and marked astrogliosis in the atriatum and globus pallidus. The 27 reported cases of IBSN were divided into three groups with characteristic clinical and pathologic features: (1) early, acute onset (four cases); (2) early, gradual onset (16 cases); and (3) late onset (seven cases). Although metabolic changes in the developing corpus striatum may be important in the pathogenesis of IBSN, the origin is uncertain.