sporadic infantile bilateral striatal necrosis
Findings
No curated finding names sporadic infantile bilateral striatal necrosis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
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.
Definition from the Mondo Disease Ontology (MONDO:0016394), read 2026-09-29. CC BY 4.0.
Features
38 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormal posturingHPOHP:0002533
- Frequent (30% to 79% of cases)
- Abnormality of movementHPOHP:0100022
- Frequent (30% to 79% of cases)
- Axial hypotoniaHPOHP:0008936
- Frequent (30% to 79% of cases)
- DysarthriaHPOHP:0001260
- Frequent (30% to 79% of cases)
- DysphagiaHPOHP:0002015
- Frequent (30% to 79% of cases)
- Facial grimacingHPOHP:0000273
- Frequent (30% to 79% of cases)
- Inappropriate cryingHPO
Show the remaining 26
- BradykinesiaHPOHP:0002067
- Occasional (5% to 29% of cases)
- ChoreaHPOHP:0002072
- Occasional (5% to 29% of cases)
- DroolingHPOHP:0002307
- Occasional (5% to 29% of cases)
- DystoniaHPOHP:0001332
- Occasional (5% to 29% of cases)
- Floppy infantHPOHP:0008947
- Occasional (5% to 29% of cases)
- Focal-onset seizureHPOHP:0007359
- Occasional (5% to 29% of cases)
Where it sits
Other names
5 names
Resolves to: sporadic infantile bilateral striatal necrosis
- Also called
- ABSNacute bilateral striatal necrosissporadic IBSNsporadic infantile striatonigral degenerationsporadic infantile striatonigral necrosis