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Sporadic infantile bilateral striatal necrosis

rare

Sporadic infantile bilateral necrosis is the sporadic form of infantile bilateral striatal necrosis (IBSN), a syndrome of bilateral symmetric spongy degeneration of the caudate nucleaus, putamen and globus pallidus characterized by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis.

Identifiers
MONDO:0016394 Orphanet:225147
Part of
Infantile bilateral striatal necrosis
Also known as
ABSN · acute bilateral striatal necrosis · sporadic IBSN · sporadic infantile striatonigral degeneration · sporadic infantile striatonigral necrosis

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