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A reference for what the literature reports. Not medical advice.

Disease

infantile bilateral striatal necrosis

MONDO:0015518Mondo

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Findings

No curated finding names infantile bilateral striatal necrosis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.

Definition

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.

Definition from the Mondo Disease Ontology (MONDO:0015518), read 2026-09-29. CC BY 4.0.

Where it sits

A kind of: neurodegenerative disease
Narrower terms (2): familial infantile bilateral striatal necrosis
sporadic infantile bilateral striatal necrosis

Other names

3 names

Resolves to: infantile bilateral striatal necrosis

Also called: IBSNinfantile striatonigral degenerationinfantile striatonigral necrosis