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Reversible Cerebral Vasoconstriction Syndrome: Recognition and Treatment

Current Treatment Options in Neurology volume 19, Article number: 21 (2017) Cite this article

Opinion statement

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare but increasingly recognized disorder with over 500 cases published in the literature. The condition is characterized by recurrent severe thunderclap headaches with or without other neurological symptoms and diffuse segmental narrowing of the cerebral arteries which is reversible within 3 months. RCVS may occur spontaneously but in over 50% of cases, it is associated with various other conditions, including vasoactive medications or illicit drugs and the post-partum state. One third to a half of cases develop hemorrhagic or ischemic brain lesions or a combination of both. Posterior reversible encephalopathy syndrome (PRES) often occurs in association with RCVS and the conditions are likely to share a common pathophysiology. The pathogenesis of RCVS remains uncertain but autonomic dysregulation, oxidative stress, and genetic predisposition are postulated. Significant differential diagnoses include subarachnoid hemorrhage (SAH) due to aneurysmal rupture, cervical artery dissection, and primary angiitis of the central nervous system (PACNS). Although there is no proven treatment, calcium channel antagonists including nimodipine and verapamil have been administered with reported reduction of headache intensity but without effect on the time course of cerebral vasoconstriction. Glucocorticoids have been reported as an independent predictor of worse outcome and should be avoided. The cornerstone of RCVS management remains largely supportive with bed rest and analgesics and removal of precipitating factors. Invasive neurointerventional techniques should be reserved for severe deteriorating cases. The condition is usually benign and self-limited and the majority of patients have a favorable outcome but around 5–10% are left with permanent neurological deficits and rare cases may die. This review details the importance of the early recognition of this increasingly described condition and current treatment recommendations.

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Affiliations

  1. Department of Neurology and Neurophysiology, Liverpool Hospital, Clinical Building, Elizabeth St, Liverpool, New South Wales, Australia

    Cecilia Cappelen-Smith MBBS FRACP PhD, Zeljka Calic MBBS FRACP & Dennis Cordato MBBS FRACP PhD

  2. South Western Clinical School, University of New South Wales, Sydney, New South Wales, Australia

    Cecilia Cappelen-Smith MBBS FRACP PhD, Zeljka Calic MBBS FRACP & Dennis Cordato MBBS FRACP PhD

  3. Ingham Institute for Applied Medical Research, Liverpool, New South Wales, Australia

    Cecilia Cappelen-Smith MBBS FRACP PhD, Zeljka Calic MBBS FRACP & Dennis Cordato MBBS FRACP PhD

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  1. Cecilia Cappelen-Smith MBBS FRACP PhD
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  2. Zeljka Calic MBBS FRACP
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Correspondence to Cecilia Cappelen-Smith MBBS FRACP PhD.

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Cecilia Cappelen-Smith, Zeljka Calic, and Dennis Cordato declare that they have no conflict of interest.

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Cappelen-Smith, C., Calic, Z. & Cordato, D. Reversible Cerebral Vasoconstriction Syndrome: Recognition and Treatment. Curr Treat Options Neurol 19, 21 (2017). https://doi.org/10.1007/s11940-017-0460-7

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Keywords

  • Reversible cerebral vasoconstriction syndrome
  • Thunderclap headache
  • Primary angiitis of the central nervous system
  • Posterior reversible encephalopathy syndrome