BrainWaves #144 RCVS vs. Primary Angiitis of the CNS

MedLink Neurology Podcast is delighted to feature selected episodes from BrainWaves, courtesy of James E Siegler MD, its originator and host. BrainWaves is an academic audio podcast whose mission is to educate medical providers through clinical cases and topical reviews in neurology, medicine, and the humanities, and episodes originally aired from 2016 to 2021.

Originally released: July 25, 2019

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

Birnbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol 2009;66(6):704-9. PMID 19506130

Miller TR, Shivashankar R, Mossa-Basha M, Gandhi D. Reversible cerebral vasoconstriction syndrome, part 1: epidemiology, pathogenesis, and clinical course. AJNR Am J Neuroradiol 2015;36(8):1392-9. PMID 25593203

Miller TR, Shivashankar R, Mossa-Basha M, Gandhi D. reversible cerebral vasoconstriction syndrome, part 2: Diagnostic work-up, imaging evaluation, and differential diagnosis. AJNR Am J Neuroradiol 2015;36(9):1580-8. PMID 25614476

Rocha EA, Topcuoglu MA, Silva GS, Singhal AB. RCVS2 score and diagnostic approach for reversible cerebral vasoconstriction syndrome. Neurology 2019;92(7):e639-47. PMID 30635475

Singhal AB. Diagnostic challenges in RCVS, PACNS, and other cerebral arteriopathies. Cephalalgia 2011;31(10):1067-70. PMID 21673004

Singhal AB, Topcuoglu MA. Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome. Neurology 2017;88(3):228-36. PMID 27940651

Singhal AB, Topcuoglu MA, Fok JW, et al. Reversible cerebral vasoconstriction syndromes and primary angiitis of the central nervous system: clinical, imaging, and angiographic comparison. Ann Neurol 2016;79(6):882-94. PMID 27043703

We believe that the principles expressed or implied in the podcast remain valid, but certain details may be superseded by evolving knowledge since the episode's original release date.