[Primary angiitis of CNS (PACNS)]

Nervenarzt. 2001 Oct;72(10):798-801. doi: 10.1007/s001150170038.
[Article in German]


Primary angiitis of the CNS (PACNS) is a rare inflammatory disease affecting middle-aged patients. The angiitis is focal and segmental in distribution, involving small and medium-sized leptomeningeal and intracranial vessels. The most frequent presenting symptoms are headaches, focal neurologic deficits, and confusion. In addition, aphasia, neuropsychological deficits, and seizures may occur. The paper presents a review of the literature. In addition, a typical case of PACNS is presented. This case demonstrates that diagnosis of PACNS is difficult and brain biopsy is essential to confirm it.

Publication types

  • Case Reports
  • English Abstract
  • Review

MeSH terms

  • Adult
  • Anti-Inflammatory Agents / therapeutic use
  • Biopsy
  • Brain / blood supply
  • Brain / pathology*
  • Cerebral Angiography
  • Diagnosis, Differential
  • Humans
  • Male
  • Prednisolone / therapeutic use
  • Syndrome
  • Treatment Outcome
  • Vasculitis, Central Nervous System / diagnosis*
  • Vasculitis, Central Nervous System / drug therapy


  • Anti-Inflammatory Agents
  • Prednisolone