Reversible Cerebral Vasoconstriction Syndrome

Abstract & Commentary

By John J. Caronna, MD, Vice-Chairman, Department of Neurology, Cornell University Medical Center; Professor of Clinical Neurology, NewYork-Presbyterian Hospital. Dr. Caronna reports no financial relationship relevant to this field of study. This article originally appeared in the January 2008 issue of Neurology Alert. It was edited by Matthew Fink, MD, and peer reviewed by M. Flint Beal, MD. Dr. Fink is Vice Chairman, Professor of Critical Care Neurology, NewYork-Presbyterian Hospital, and Dr. Beal is Professor and Chairman, Department of Neurology, Cornell University Medical College. Drs. Fink and Beal report no financial relationships relevant to this field of study.

Synopsis: RCVS occurs in a variety of clinical settings, and should be considered and investigated in any person with sudden severe headache that is unexplained by other disorders.

Source: Ducros A, et al. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain. 2007;130:3091-3101.

Ducros and colleagues have reported the largest series of cases of the so-called "reversible cerebral vasoconstriction syndrome" (RCVS). RCVS is characterized by the association of severe or "thunderclap" headaches with or without neurological deficits, and a string of beads appearance of cerebral arteries that resolves spontaneously in 1-3 months. Ducros et al prospectively followed 67 consecutive patients, diagnosed at Lariboisiere Hospital in Paris, with angiographically confirmed RCVS. There were 43 women and 24 men, with a mean age of 42 years (range, 19-70 years). RCVS was spontaneous in one-third and secondary in two-thirds of patients. More than one-half of the patients (n = 37) reported the previous use of vasoactive substances (cocaine, cannabis, nasal decongestants, serotonin reuptake inhibitors, interferon, nicotine patches), which in some cases was combined with binge drinking. Five patients (12%) were just postpartum; only one had received bromocriptine after delivery to inhibit lactation.

Severe headache, by definition, was the presenting symptom in all patients, and was the only symptom of RCVS in 51 patients (76%). Sixty-three patients (94%) had multiple thunderclap headaches (mean number, 4.5; range, 2-18) that recurred over a mean period of 7.4 days (range, 1-21). In 16 patients with complications, cortical subarachnoid hemorrhage (SAH) (22%), intracerebral hemorrhage (ICH) (6%), seizures (3%), and reversible posterior leukoencephalopathy (RPLS) (9%) were early complications that occurred mainly during the first week. Ischemic events, including TIA (16%) and stroke (4%), occurred mainly during the second week.

Ducros et al, therefore, hypothesize that the different time course and evolution of thunderclap headaches, SAH, and strokes indicate that the vasospastic disorder starts in small distal arteries and progresses to involve medium and large arteries.

No relapses were observed during the mean follow-up of 16 months. Ducros et al concluded from their data that RCVS is more frequent than commonly thought, and is, more often than not, secondary to exposure to vasoactive substances.

Commentary

RCVS is a term that encompasses a group of disorders sharing angiographic and clinical features, namely reversible segmental and multifocal vasoconstriction of cerebral arteries, and severe headaches with or without focal neurological deficits. The names for this syndrome, among many others, include: benign cerebral angiopathy or vasculitis,1 Call-Fleming syndrome,2 and thunderclap headache with reversible vasospasm.3 The pathophysiology of RCVS is not understood, and the cause probably is multifactorial, given the numerous and heterogeneous precipitating events that may or may not be causally related to the syndrome.

In an editorial accompanying this report, van Gijn doubts that RCVS is a nosological entity because of the overlap with other syndromes such as migraine and the multitude of precipitating factors, as well as interobserver variation in what constitutes arterial narrowing on cerebral angiograms.4 Nevertheless, Ducros et al have done clinicians a great service by reporting their large series of RCVS patients in useful detail, thereby stimulating further research into this sometimes not-so-benign entity.

References

1. Snyder BD, McClelland RR. Isolated benign cerebral vasculitis. Arch Neurol 1978;35:612-614.

2. Call GK, et al. Reversible cerebral segmental vasoconstriction. Stroke 1988;19:1159-1170.

3. Dodick DW, et al. Thunderclap headache associated with reversible vasospasm and posterior leukoencephalopathy syndrome. Cephalalgia 2003;23:994-997.

4. van Gijn J. Cerebral vasoconstriction, headache and sometimes stroke: One syndrome or many? Brain. 2007;130(Pt 12):3060-3062.