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Start free with EleplanInfantile bilateral striatal necrosis
ORPHA:1576Clinical group
Also called Infantile striatonigral degeneration · Infantile striatonigral necrosis · IBSN
What it is
Infantile bilateral striatal necrosis (IBSN) comprises several syndromes of bilateral symmetric spongy degeneration of the caudate nucleus, putamen and globus pallidus characterized by developmental regression, choreoathetosis and dystonia progressing to spastic quadriparesis. IBSN can be familial or sporadic.
Key facts
- Prevalence
- 1-9 / 1 000 000 (Europe)
- Age of onset
- All ages
- Inheritance
- Autosomal dominant, Autosomal recessive, Mitochondrial inheritance, Not applicable
- Classified as
- Clinical group
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Genes reported in subtypes
Orphanet records these genes on 1 more specific entry under this disorder, not on this entry itself:
A well-studied disease is usually split into subtypes, and the gene is curated on whichever one the evidence belongs to. Open a subtype to see which gene goes with it.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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