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

Ann Neurol. 2016 Jun;79(6):882-94. doi: 10.1002/ana.24652. Epub 2016 Apr 28.


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). Ann Neurol 2016;79:882-894.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Cerebral Angiography*
  • Cerebrovascular Disorders / diagnosis*
  • Cerebrovascular Disorders / diagnostic imaging
  • Computed Tomography Angiography
  • Diagnosis, Differential
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Neuroimaging*
  • Syndrome
  • Vasculitis, Central Nervous System / diagnosis*
  • Vasculitis, Central Nervous System / diagnostic imaging
  • Vasoconstriction*
  • Young Adult

Supplementary concepts

  • Primary angiitis of the central nervous system