Catalog

Familial infantile bilateral striatal necrosis

No MMS code

Link to WHO Definition

Familial infantile bilateral striatal necrosis – http://id.who.int/icd/entity/1873983370

Familial infantile bilateral striatal necrosis is the familial form of infantile bilateral striatal necrosis (IBSN), a syndrome of bilateral symmetric spongy degeneration of the caudate nucleus, putamen and globus pallidus and is characterised by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis.