Primary CNS vasculitis

Diagnosis is difficult. No specific clinical feature, no blood or imaging study that can confirm the diagnosis. No established consensus on making the diagnosis.

Common features

  • Headache
  • Encephalopathy
  • cognitive changes
  • seizure

Tests

  • MRI head abnormal 93%
  • CSF abnormal 74%
  • CTA or MRA: multiple stenotic lesion in multiple or a single vessel
  • Brain biopsy: sensitivity 50-70%

Treatment

  • Cyclophosphamide
  • Corticosteroid

Practical neurology BMJ Full article – reviewed 05/12/2020