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infantile bilateral striatal necrosis

Disease Summary
Associated Targets (4)
Tbio

4


GARD Rare
Mondo Description Infantile bilateral striatal necrosis (IBSN) comprises 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.
Mondo Term and Equivalent IDs
MONDO:0015518:  infantile bilateral striatal necrosis
GARD:0005040: 
Orphanet:1576: 
SCTID:718174008: 
UMLS:C0795996: