x

Search for a rare disease

* (*) mandatory field

Suggest an update

(*) Required fields.

Attention

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

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).

ORPHA:1576

  • 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

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.