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Infantile bilateral striatal necrosis

Disease definition

Infantile bilateral striatal necrosis (IBSN) comprises 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 (see these terms).


  • Synonym(s):
    • IBSN
    • Infantile striatonigral degeneration
    • Infantile striatonigral necrosis
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal dominant or Autosomal recessive or Mitochondrial inheritance or Not applicable 
  • Age of onset: All ages
  • ICD-10: G23.2
  • OMIM: 271930  500003
  • UMLS: C0795996
  • MeSH: -
  • GARD: 5040
  • MedDRA: -

Additional information

Further information on this disease

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