Reversible cerebral vasoconstriction syndrome: A thunderclap headache-associated condition

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by a sudden, severe headache at onset, vascular narrowing involving the circle of Willis and its immediate branches, and angiographic evidence of vasoconstriction reversibility within minutes to weeks of onset. RCVS is underrecognized and often misdiagnosed; it can defy clinical detection because it can mimic common conditions such as migraine and ischemic stroke. A lack of shared nosology has hampered awareness and understanding of the syndrome. Clinicians must consider primary angiitis of the central nervous system because of its high rates of morbidity and mortality if left untreated. RCVS has a number of primary and secondary associations (cerebral hemorrhage, vasoactive substances, the peripartum period, bathing, and physical exertion) but also occurs in isolation. RCVS can present in conjunction with hypertensive encephalopathy, preeclampsia, and reversible posterior leukoencephalopathy. This review provides an up-to-date account of RCVS.

Autonomic dysfunction in reversible cerebral vasoconstriction syndromes

“…The proposed pathophysiology of RCVS has been linked to sympathetic overactivity based on clinical observations or hypotheses including central vascular tone changes [8,24], aberrant sympathetic response [10], blood pressure surge [5], triggers [3,5], sympathomimetic agents [3,12,13], pheochromocytoma and hypertensive crises [25], and autonomic dysreflexia [26], etc. Our findings provided more direct evidence in support of these speculations.…”

Cerebral vasospasm following tumor resection

“…Chang et al 12 also speculated that direct and excessive vessel manipulation may impair the process of vasodilatation, by preventing the release of relaxant factors, which will alter the balance of vascular tone to a spasmogenic one. This imbalance of tone could be part of the ‘reversible cerebral vasoconstriction syndrome’52 which has been reported to happen in head trauma and neurosurgical operations although its pathophysiology is still not clearly understood.…”

Neuroimaging of Headaches Associated with Vascular Disorders

“…RCVS frequently occurs during the postpartum period, and headaches are the most frequent symptom. Although headaches are frequently sudden and severe at onset and are often accompanied by nausea, vomiting, or photophobia, RCVS can also present with gradual insidious headache onset [56,57]. Because headaches are a common manifestation of other conditions, such as central nervous system vasculitis, and the pattern and characteristics of headache can be nonspecific, CSF analysis and imaging data are necessary to establish the diagnosis of RCVS [55,58,59].…”