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Familial Infantile Bilateral Striatal Necrosis Neurol 59:983-989, Straussberg,R.,et al, 2002 See this aricle in Pubmed Article Abstract 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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