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Comment & Response
March 2014

Reversible Cerebral Vasoconstriction Syndrome

Author Affiliations
  • 1Department of Neurology, Montpellier University Hospital, Montpellier, France
  • 2Center for Vasculitis Care and Research, Cleveland Clinic Foundation, Cleveland, Ohio
  • 3Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts
  • 4Department of Neurology, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan
JAMA Neurol. 2014;71(3):368. doi:10.1001/jamaneurol.2013.5875

To the Editor We congratulate Katz and colleagues for their article about reversible cerebral vasoconstriction syndrome (RCVS).1 This study confirmed that RCVS is common, similar to our own experience of more than 420 patients accumulated within a similar time span across 4 centers.2-5 We recently emphasized that RCVS has a “uniphasic course without new symptoms more than 1 month after clinical onset.”5 Katz et al1 seemed to have misinterpreted the term uniphasic and described initial progression as a new observation, although their results confirm our previous descriptions of the dynamic clinicoradiological course of RCVS. Stroke can occur a few days after benign presentation, and cerebral vasoconstriction is at a maximum 2 to 3 weeks after clinical onset.2-5 Importantly, regardless of this initial clinical-angiographic worsening, approximately 95% of our patients have had good neurological outcome. In the study by Katz et al,1 the degree of clinical worsening was not quantified, and the percentage with worsening or poor outcome likely overestimated owing to the inpatient setting and referral biases. Fortunately, their discussion appropriately stated that initial worsening is usually mild and self-limited and should not prompt consideration of alternate diagnosis, such as primary angiitis of the central nervous system, or warrant aggressive intervention. The ideal duration of observation or the management of patients who do worsen was not discussed.

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