Sporadic infantile bilateral striatal necrosis
Sporadic infantile bilateral striatal necrosis
Definition
Sporadic infantile bilateral necrosis is the sporadic form of infantile bilateral striatal necrosis (IBSN), a syndrome of bilateral symmetric spongy degeneration of the caudate nucleaus, putamen and globus pallidus characterized by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis.
Also known as ABSN, acute bilateral striatal necrosis, sporadic IBSN, sporadic infantile striatonigral degeneration, sporadic infantile striatonigral necrosis — per MONDO