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infantile bilateral striatal necrosis (IBSN) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

infantile bilateral striatal necrosis

MONDO:0015518

Also known as:IBSNinfantile striatonigral degenerationinfantile striatonigral necrosis

Overview

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.

ResearchLimited published research (1 article)

Prognosis

Estimated prevalence: 1-9 in 1,000,000 (Rare).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for infantile bilateral striatal necrosis.

1 publication has been identified in PubMed for infantile bilateral striatal necrosis. Research spans Review / Meta-Analysis (100%).

Recent Publications

Fare CM (2024). [PMID: 38383349](https://pubmed.ncbi.nlm.nih.gov/38383349/). *Nucleus (Austin, Tex.)*. [Review / Meta-Analysis]

Data assembled from 3 of 12 sources · Last updated Sep 19, 2026, 11:56 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:1576

GARD

Genetic and Rare Diseases Info Center

GARD:5040

Questions for Your Care Team

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infantile bilateral striatal necrosis

Overview

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Subtypes

Related Diseases

Questions for Your Care Team

Questions for Your Care Team