Journal
JOURNAL OF NEUROLOGY
Volume 270, Issue 2, Pages 673-688Publisher
SPRINGER HEIDELBERG
DOI: 10.1007/s00415-022-11425-z
Keywords
Reversible cerebral vasoconstriction syndrome; Posterior reversible encephalopathy syndrome; Thunderclap headache; Primary angiitis of the central nervous system
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Reversible cerebral vasoconstriction syndrome is a common but potentially underdiagnosed condition characterized by thunderclap headache and widespread vasoconstriction. The pathophysiology is believed to involve sympathetic overactivity and endothelial dysfunction. Precipitating factors include the use of vasoactive substances and postpartum status.
Background/Objective Reversible cerebral vasoconstriction syndrome may be underdiagnosed. It can be accompanied by various complications, mainly intracerebral hemorrhage and ischemic stroke. The clinical presentation of this condition varies according to its localization. The aims of this review are to raise awareness of the disease, especially in the presence of corresponding risk factors; to connect its precipitating factors, pathophysiology, and complications; and to compare various differential diagnoses of vasoconstriction. Methods A review of the literature in PubMed/MEDLINE and Google Scholar was conducted from May 1997 until May 2022. Results Reversible cerebral vasoconstriction syndrome, which is a clinical-radiological syndrome, is mainly characterized by the occurrence of thunderclap headache and widespread vasoconstriction. The most common precipitating factors are the use of vasoactive substances and postpartum status. The pathophysiology is currently assumed to include two mechanisms: sympathetic overactivity and endothelial dysfunction. From these mechanisms, it is possible to derive potential complications as well as the most important differential diagnoses: posterior reversible encephalopathy syndrome, convexity subarachnoid hemorrhage, ischemic and hemorrhagic stroke, and primary angiitis of the central nervous system. Conclusion In general, the outcome of reversible cerebral vasoconstriction syndrome is very good. Vasospasm as well as thunderclap headache attacks can be fully reversible, and > 90% of patients are functionally independent at discharge.
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