Catalog

Infantile bilateral striatal necrosis

No MMS code

Link to WHO Definition

Infantile bilateral striatal necrosis – http://id.who.int/icd/entity/1947032348

Infantile bilateral striatal necrosis (IBSN) comprises several syndromes of bilateral symmetric spongy degeneration of the caudate nucleus, putamen and globus pallidus characterised by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis. IBSN can be familial or sporadic.

Parents

Syndromic conditions with disorders of intellectual development as a relevant clinical feature

Children

Sporadic infantile bilateral striatal necrosis
Familial infantile bilateral striatal necrosis