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CLIPPERS

Disease definition

CLIPPERS is a rare neuroinflammatory disorder characterized by brainstem-predominant encephalomyelitis which typically presents with cerebellar and cranial nerve manifestations (gait ataxia, dysarthria, visual disorders, parasthesias), as well as brainstem, myelopathy and cognitive findings, that respond to steroid treatment. Punctate curvilinear post-gadolinium contrast enhancement predominantly in the pons and cerebellum is observed on brain MRI and prominent, perivascular, CD3+ T-cell predominantly lymphocytic inflammation in neuropathology.

ORPHA:284448

Classification level: Disorder
  • Synonym(s):
    • Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids
  • Prevalence: <1 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: G04.8
  • OMIM: -
  • UMLS: C3854437
  • MeSH: -
  • GARD: 10779
  • MedDRA: -
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