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

Rare Disease

sporadic infantile bilateral striatal necrosis

MONDO:0016394

Also known as:ABSNacute bilateral striatal necrosissporadic IBSN

Overview

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.

ResearchLimited published research (3 articles)

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include common findings: Facial grimacing, Seizure, Dysarthria, and Specific learning disability and others; and sometimes findings: Urinary incontinence, Mask-like facies, Hypomimic face, and Short attention span and others.

Organ System	Phenotype Count	Example Features
Brain and nerves	20	Seizure, Dysarthria, Specific learning disability

Prognosis

Phenotype severity distribution: 10 common features.

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 sporadic infantile bilateral striatal necrosis.

3 publications have been identified in PubMed for sporadic infantile bilateral striatal necrosis. Research spans Review / Meta-Analysis (100%).

Recent Publications

Sousa Brandão P (2026). [PMID: 41582100](https://pubmed.ncbi.nlm.nih.gov/41582100/). *Neuroradiology*. [Review / Meta-Analysis]
Sakuma H (2025). [PMID: 39143740](https://pubmed.ncbi.nlm.nih.gov/39143740/). *Dev Med Child Neurol*. [Review / Meta-Analysis]
Fare CM (2024). [PMID: 38383349](https://pubmed.ncbi.nlm.nih.gov/38383349/). *Nucleus*. [Review / Meta-Analysis]

Data assembled from 4 of 12 sources · Last updated Sep 19, 2026, 3:02 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:225147

GARD

Genetic and Rare Diseases Info Center

GARD:20549

Questions for Your Care Team

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sporadic infantile bilateral striatal necrosis

Overview

Clinical Presentation

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

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