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Rare disease
Rapidly progressive dementia due to leukocytoclastic vasculitis of the central nervous system
  1. Carolina Pires,
  2. Henda Foreid,
  3. Cândida Barroso,
  4. José Manuel Ferro
  1. Department of Neurosciences, Hospital de Santa Maria, Lisbon, Portugal
  1. Correspondence to Dr Carolina Pires, mcarolinapires{at}


A 70-year-old male was admitted with a 2-week progressive course of severe cognitive impairment, scoring three on the Mini Mental State Examination. MRI of the brain showed confluent hyperintense areas in T2/FLAIR in the periventricular and subcortical white matter, extending to right parietal cortex and basal ganglia. Intra-arterial angiography was unremarkable. A targeted stereotactic brain biopsy disclosed a leukocytoclastic vasculitis. The patient improved on steroids. Leukocytoclastic vasculitis adds to the spectrum of histopathologic subtypes of primary angiitis of the central nervous system.

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  • Competing interests None.

  • Patient consent Obtained.