The diagnosis of primary central nervous system vasculitis

Pract Neurol. 2020 Apr;20(2):109-114. doi: 10.1136/practneurol-2018-002002. Epub 2019 Oct 24.


The diagnosis of primary central nervous system (CNS) vasculitis is often difficult. There are neither specific clinical features nor a classical clinical course, and no blood or imaging investigations that can confirm the diagnosis. Contrast catheter cerebral angiography is neither specific nor sensitive, yet still underpins the diagnosis in many published studies. Here we describe an approach to its diagnosis, emphasising the importance of obtaining tissue, and present for discussion a new, binary set of diagnostic criteria, dividing cases into only 'definite' primary CNS vasculitis, where tissue proof is available, and 'possible,' where it is not. We hope that these criteria will be modified and improved by discussion among experts, and that these (improved) criteria may then be adopted and used as the basis for future prospective studies of the clinical features and diagnosis of this difficult and dangerous disorder, particularly for coordinated multicentre therapeutic trials.

Keywords: cerebrovascular disease; vasculitis.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex Hormones / administration & dosage
  • Biopsy / methods
  • Cerebral Angiography / methods*
  • Cyclophosphamide / administration & dosage
  • Diagnosis, Differential
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Vasculitis, Central Nervous System / cerebrospinal fluid*
  • Vasculitis, Central Nervous System / diagnostic imaging*
  • Vasculitis, Central Nervous System / drug therapy


  • Adrenal Cortex Hormones
  • Immunosuppressive Agents
  • Cyclophosphamide