BrainWaves: A Neurology Podcast

#144 RCVS vs. Primary Angiitis of the CNS

BrainWaves: A Neurology Podcast

A 50-year-old gentleman presents with severe headaches and speech disturbance. The MRI shows acute strokes and multifocal vascular irregularities. Is it reversible cerebral vasoconstriction syndrome or primary angiitis of the central nervous system? Or is it something else entirely?

RCVS and PACNS almost always pose a challenge--even to the most astute neurologist. But, because they are treated entirely differently and their clinical trajectories depend on these treatments, it is worth knowing how experts distinguish the two. This week on the BrainWaves podcast, Dr. Jesse Thon joins Jim Siegler in a discussion about the clinical and radiographic features of these two conditions, and shares his experience in managing these patients.

Produced by James E. Siegler and Jesse Thon. Music courtesy of Nuno Adelaida, Peter Rudenko on the piano, Squire Tuck on the guitar, Swelling, and Uncanny. Sound effects by Mike Koenig and Daniel Simion. BrainWaves' podcasts and online content are intended for medical education only and should not be used for clinical decision making. Be sure to follow us on Twitter @brainwavesaudio for the latest updates to the podcast.


REFERENCES

  1. Singhal AB. Diagnostic challenges in RCVS, PACNS, and other cerebral arteriopathies. Cephalalgia. 2011;31:1067-70.
  2. Miller TR, Shivashankar R, Mossa-Basha M and Gandhi D. Reversible Cerebral Vasoconstriction Syndrome, Part 2: Diagnostic Work-Up, Imaging Evaluation, and Differential Diagnosis. AJNR American journal of neuroradiology. 2015;36:1580-8.
  3. Miller TR, Shivashankar R, Mossa-Basha M and Gandhi D. Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical Course. AJNR American journal of neuroradiology. 2015;36:1392-9.
  4. Singhal AB, Topcuoglu MA, Fok JW, Kursun O, Nogueira RG, Frosch MP and Caviness VS, Jr. Reversible cerebral vasoconstriction syndromes and primary angiitis of the central nervous system: clinical, imaging, and angiographic comparison. Annals of neurology. 2016;79:882-94.
  5. Singhal AB and Topcuoglu MA. Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome. Neurology. 2017;88:228-236.
  6. Rocha EA, Topcuoglu MA, Silva GS and Singhal AB. RCVS2 score and diagnostic approach for reversible cerebral vasoconstriction syndrome. Neurology. 2019;92:e639-e647.
  7. Birnbaum J and Hellmann DB. Primary angiitis of the central nervous system. Archives of neurology. 2009;66:704-9.

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