The Typical Thunderclap Headache of Reversible Cerebral Vasoconstriction Syndrome and its Various Triggers

Abstract

During the last 10 years, reversible cerebral vasoconstriction syndrome (RCVS) has emerged as the most frequent cause of thunderclap headache (TCH) in patients without aneurysmal subarachnoid hemorrhage, and as the most frequent cause of recurrent TCHs. The typical TCHs of RCVS are multiple, recurring over a few days to weeks, excruciating, short-lived, and brought up by exertion, sexual activities, emotion, Valsalva maneuvers, or bathing, among other triggers. All these triggers induce sympathetic activation. In a minority of cases with RCVS, TCH heralds stroke and rarely death. Early diagnosis of RCVS in patients who present with isolated headache enables proper management and might reduce the risk of eventual stroke. This review describes the characteristics, triggers, diagnosis, and management of TCH in RCVS. One aim is to underline that the TCH pattern of RCVS is so typical that it enables, according to the 2013 revision of the International Classification of Headache Disorders, the diagnosis of "probable RCVS" in patients with such a headache pattern, normal cerebral angiography, and no other cause. Another objective is to discuss the role of physical and emotional stress in RCVS and in other related conditions involving similar triggers.

Blood–brain barrier breakdown in reversible cerebral vasoconstriction syndrome: Implications for pathophysiology and diagnosis

“…Its specificity is further supported by a recent report that BBB integrity is not disrupted during acute migraine attack. 43 In contrast, CE-FLAIR assessment of BBB breakdown showed excellent interobserver agreement. 19,21,29,42 In addition to specificity, interobserver reliability can be enhanced by documentation of BBB breakdown.…”

“…Although the sensitivity is not excellent, it is specific for RCVS among patients with thunderclap headache. 5,8,11,43 However, sensitivity and specificity of MRA are not optimal for detecting vasoconstriction in distal vessels, causing substantial interobserver disagreement. 41 We found that the multifocal BBB breakdown located near the falx and convexity is characteristic of RCVS, which is Lee et al: BBB Breakdown in RCVS different from the territorial involvement in acute stroke or hyperperfusion syndrome.…”

Reversible Cerebral Vasoconstriction Syndrome Triggered by Typhoon Hagibis in 2019: Report of 2 Cases

“…Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by multifocal cerebral arterial constriction that resolves within 3 months. Individuals with RCVS often develop thunderclap headaches . Various risk factors are found in at least half of patients with RCVS .…”

“…Individuals with RCVS often develop thunderclap headaches . Various risk factors are found in at least half of patients with RCVS . These include exposure to vasoactive medications or illicit drugs, postpartum status, catecholamine‐secreting tumors, surgery, trauma, and various medical conditions .…”

“…Various risk factors are found in at least half of patients with RCVS . These include exposure to vasoactive medications or illicit drugs, postpartum status, catecholamine‐secreting tumors, surgery, trauma, and various medical conditions . RCVS can occur spontaneously, especially in middle‐aged women …”