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Up to: Syndromic disease · Infantile bilateral striatal necrosis · Striatonigral degeneration
Familial infantile bilateral striatal necrosis
The familial form of infantile bilateral striatal necrosis (IBSN), a syndrome of bilateral symmetric spongy degeneration of the caudate nucleus, putamen and globus pallidus characterized by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis.