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Familial infantile bilateral striatal necrosis was inherited as an autosomal recessive trait. Clinical features included developmental arrest, dysphagia, and choreoathetosis. Imaging and pathology showed atrophy and degeneration of the bas al ganglia. Oral biotin may have benefited three children. |
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basal ganglia basal ganglia,degeneration basal ganglia,lesion of basal ganglia,lesion,bilateral biotin choreoathetosis familial genetic neurologic disorders infantile bilateral striatal necrosis lenticular nucleus,lesion of,bilateral MRI,abnormal neuropathology striatum,lesion of treatment of neurologic disorder
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