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Reversible cerebral vasoconstriction syndrome
Practical Neurology, 09/21/09

Ducros A et al. – Reversible cerebral vasoconstriction syndrome is characterised by severe headaches with or without seizures and focal neurological deficits, and constriction of cerebral arteries which resolves spontaneously in 1–3 months.

• The reversible cerebral vasoconstriction syndrome is more frequent than previously thought and affects patients of both genders, with a female preponderance.
• It is attributed to a transient disturbance in the control of cerebral vascular tone leading to multifocal arterial constrictions and dilatations.
• Some cases are spontaneous while others (60%) are secondary, mostly to exposure to vasoactive substances and to the postpartum state.
• The syndrome has a characteristic course; the onset is sudden followed by a monophasic course, generally without new events after 1 month. The main pattern is of recurrent thunderclap headaches.
• Cortical subarachnoid haemorrhage, intracerebral haemorrhage, seizures and the reversible posterior encephalopathy syndrome are early complications, occurring mainly within the first week.
• Ischaemic events, including transient ischaemic attacks and cerebral infarction, occur later than any haemorrhagic strokes, mainly during the second week.
• Diagnosis requires the demonstration of the characteristic "string of beads" on cerebral angiography, the definitive diagnosis being made when a later angiogram shows resolution or at least marked improvement of the arterial abnormalities after about 12 weeks.
• Nimodipine is the proposed treatment but in the authors' experience does not seem very effective; randomised trials are needed.
• Relapses do occur but are rare

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