Syndrome de vasoconstriction cérébrale réversible

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is more frequent than previously thought and is probably underdiagnosed. The mean age of onset is 42 years, and it affects slightly more women than men. RCVS is attributed to a transient, reversible dysregulation of cerebral vascular tone, which leads to multifocal arterial constriction and dilation. More than half the cases (60%) are secondary to exposure to vasoactive substances (e.g., cannabis, antidepressants, and nasal decongestants) or occur in the postpartum period. RCVS has a characteristic clinical and radiological course, developing in a single phase after a sudden onset, and there is generally no new event after 1 month. The main pattern of presentation begins with recurrent thunderclap headaches, often triggered by sexual activity or various Valsalva's maneuvers, over a period of 1 to 3 weeks. Seizures and focal neurological deficits are less frequent and generally start after the headaches. Cortical subarachnoid hemorrhage (22%), intracerebral hemorrhage (6%), seizures (3%), and reversible posterior leukoencephalopathy (9%) are early complications, occurring mainly within the first week. Ischemic events, including TIAs (16%) and cerebral infarction (4%), occur significantly later than hemorrhagic strokes, mainly during the second week. Diagnosis requires the demonstration of the characteristic "string and beads" on cerebral angiography and can be difficult, for 21% of patients have a normal initial magnetic resonance angiography (MRA) and 9% both a normal MRA and a normal transcranial Doppler. In these cases, the initial investigations must be repeated after a few days. The final diagnosis is made when a follow-up MRA shows resolution or at least marked improvement of the arterial abnormalities within 12 weeks. RCVS is sometimes associated with other large artery lesions of the head and neck, including dissections and unruptured aneurysms, especially during the postpartum period. Nimodipine is the treatment most often recommended. In our experience, it is not especially effective in severe RCVS. Relapses are possible but rare and have not yet been reported in prospective series. Although the exact pathophysiology remains speculative, strong recommendations against vasoactive substances appear prudent.