Infantile bilateral striatal necrosis
Infantile bilateral striatal necrosis
Definition
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.
Also known as IBSN, infantile bilateral striatal necrosis, infantile striatonigral degeneration, infantile striatonigral necrosis — per MONDO