Sporadic infantile bilateral striatal necrosis
ORPHA:225147· ICD-10 G23.2
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.
- Prevalence
- 1-9 / 1 000 000
- Inheritance
- Not applicable
- Age of onset
- All ages