Orphanet: Sporadic infantile bilateral striatal necrosis
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Sporadic infantile bilateral striatal necrosis

Disease definition

Sporadic infantile bilateral necrosis is the sporadic form of infantile bilateral striatal necrosis (IBSN; see this term), 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.

ORPHA:225147

Classification level: Disorder
  • Synonym(s):
    • ABSN
    • Acute bilateral striatal necrosis
    • Sporadic IBSN
    • Sporadic infantile striatonigral degeneration
    • Sporadic infantile striatonigral necrosis
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: G23.2
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -
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