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Infantile bilateral striatal necrosis

rare

Several syndromes of bilateral symmetric spongy degeneration of the caudate nucleaus, putamen and globus pallidus characterized by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis. IBSN can be familial or sporadic.

Also known as
IBSN · infantile striatonigral degeneration · infantile striatonigral necrosis · SNDI · striatal degeneration familial
Disease Subtypes
Familial infantile bilateral striatal necrosis 1
Sporadic infantile bilateral striatal necrosis

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