Reversible Cerebral Vasoconstriction Syndromes and Primary Angiitis of the Central Nervous System: Clinical, Imaging, and Angiographic Comparison

Singhal A. B., Topcuoglu M. A., Fok J. W., KURŞUN O., Nogueira R. G., Frosch M. P., ...More

ANNALS OF NEUROLOGY, vol.79, no.6, pp.882-894, 2016 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 79 Issue: 6
  • Publication Date: 2016
  • Doi Number: 10.1002/ana.24652
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.882-894
  • Hacettepe University Affiliated: Yes


Reversible cerebral vasoconstriction syndromes (RCVS) and primary angiitis of the central nervous system (PACNS) are invariably considered in the differential diagnosis of new cerebral arteriopathies. However, prompt and accurate diagnosis remains challenging. Here we compared the features of 159 RCVS to 47 PACNS patients and developed criteria for prompt bedside diagnosis. Recurrent thunderclap headache (TCH), and single TCH combined with either normal neuroimaging, border zone infarcts, or vasogenic edema, have 100% positive predictive value for diagnosing RCVS or RCVS-spectrum disorders. In patients without TCH and positive angiography, neuroimaging can discriminate RCVS (no lesion) from PACNS (deep/brainstem infarcts).