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Primary angiitis of the central nervous system

Disease definition

A rare, medium or small vessel vasculitis characterized by focal and/or diffuse neurologic symptoms due to a documented arteritic process in the central nervous system, in the absence of other identified underlying cause (infectious, systemic, other neurologic diseases, etc.). It presents with non-specific symptoms of headache, stroke or transient ischemic attacks with cognitive impairment, hemiplegia, weakness, and rarely, with cranial nerve involvement, seizures and ataxia.

ORPHA:140989

Classification level: Disorder
  • Synonym(s):
    • Isolated angiitis of the central nervous system
    • PACNS
    • PCNSV
    • Primary central nervous system vasculitis
    • Primary vasculitis of the central nervous system
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: I67.7
  • OMIM: -
  • UMLS: C0751881  C2930862
  • MeSH: C535276  D020293
  • GARD: 8703
  • MedDRA: -
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