MONDO_0010080 (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 characterized by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis. Also known as: familial IBSN, familial infantile striatonigral degeneration, familial infantile striatonigral necrosis, hereditary infantile bilateral striatal necrosis.