Preferred Label : Infantile bilateral striatal necrosis;
ICD-11 definition : 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.;
Origin ID : 1947032348;
UMLS CUI : C0795996;
Currated CISMeF NLP mapping
Semantic type(s)
UMLS correspondences (same concept)
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.