Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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January 4, 2010
Reversible Cerebral Vasoconstriction Syndrome
- Reversible cerebral vasoconstriction syndrome is a poorly characterized clinical entity comprised by the association of severe headaches with or without additional neurological symptoms and a "string and beads" appearance of cerebral arteries, which resolves spontaneously in 1'3 months; more common in females.
- In a series of 67 patients, the most common presentation (97%) was thunderclap headaches occurring over a period of 1 week.
- Complications observed included: cortical subarachnoid hemorrhage (22%), intracerebral hemorrhage (6%), seizures (3%) and a reversible posterior leukoencephalopathy (9%); all these occurred mostly during the 1st week. Ischemic events, including TIAs (16%) and cerebral infarctions (4%) occurred later than hemorrhages, mainly during the 2nd week.
- Causes of this syndrome: postpartum, exposure to vasoactive substances, catecholamine-secreting tumors, exposure to immunosuppressants or blood products, and miscellaneous.
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