Brain Advance Access originally published online on November 19, 2007
Brain 2007 130(12):3091-3101; doi:10.1093/brain/awm256
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The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients
1Emergency Headache Centre, 2Neurology Department, 3Neuroradiology Department, Lariboisière Hospital, 2 rue Ambroise Paré, 75010 Paris, and 4Statistics, Saint-Louis Hospital, 1 avenue Claude Vellefaux, 75010 Paris; all from Assistance Publique-Hôpitaux de Paris and Paris-7 Dennis-Diderot University, Paris, France
Correspondence to: Anne Ducros, MD, PhD, Urgences Céphalées, Hôpital Lariboisière, 2 rue Ambroise Paré, 75475 Paris Cedex 10 E-mail: anne.ducros{at}lrb.aphp.fr
Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by the association of severe headaches with or without additional neurological symptoms and a ‘string and beads’ appearance on cerebral arteries, which resolves spontaneously in 1–3 months. We present the clinical, neuroimaging and outcome data of 67 consecutive patients prospectively diagnosed over 3 years in our institution with an angiographically confirmed RCVS. There were 43 females and 24 males with a mean age of 42 years (19–70). RCVS was spontaneous in 37% of patients and secondary in the 63% others, to postpartum in 5 and to exposure to various vasoactive substances in 37, mainly cannabis, selective serotonin-recapture inhibitors and nasal decongestants. The main pattern of presentation (94% of patients) was multiple thunderclap headaches recurring over a mean period of 1 week. In 51 patients (76%), headaches resumed the clinical presentation. Various complications were observed, with different time courses. Cortical subarachnoid haemorrhage (cSAH) (22%), intracerebral haemorrhage (6%), seizures (3%) and reversible posterior leukoencephalopathy (9%) were early complications, occurring mainly within the first week. Ischaemic events, including TIAs (16%) and cerebral infarction (4%), occurred significantly later than haemorrhagic events, mainly during the second week. Significant sex differences were observed: women were older, had more frequent single-drug exposure and a higher rate of stroke and cSAH. Sixty-one patients were treated by nimodipine: 36% had recurrent headaches, 7% TIAs and one multiple infarcts. The different time courses of thunderclap headaches, vasoconstriction and strokes suggest that the responsible vasospastic disorder starts distally and progresses towards medium sized and large arteries. No relapse was observed during the 16 ± 12.4 months of follow-up. Our data suggest that RCVS is more frequent than previously thought, is more often secondary particularly to vasoactive substances, and should be considered in patients with recurrent thunderclap headaches, cSAH or cryptogenic strokes with severe headaches.
Key Words: vasospasm; vasoconstriction; headache; thunderclap headache; subarachnoid haemorrhage; stroke; intracerebral haemorrhage; cerebral infarction; seizures; angiography
Abbreviations: cSAH, cortical subarachnoid haemorrhage; ICH, intracerebral haemorrhage; MRA, magnetic resonance angiography; MRI, magnetic resonance imaging; RCVS, reversible cerebral vasoconstriction syndromes; RPLS, reversible posterior leukoencephalopathy syndrome; TCD, transcranial Doppler; TIA, transient ischaemic attack
Received July 6, 2007. Revised September 25, 2007. Accepted September 27, 2007.
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