Reversible cerebral vasoconstriction syndrome with concurrent bilateral carotid artery dissection

Abstract

Background: The pathophysiological basis of reversible cerebral vasoconstriction syndrome is poorly understood but carotid artery dissection has been discussed as a rare possible cause. So far, only single cases of unilateral carotid artery dissection and reversible cerebral vasoconstriction syndrome have been reported. Case: Here, we describe the case of a 54-year old patient presenting to the emergency department with right hemiparesis, hypaesthesia and dysarthria. Furthermore, he reported two episodes of thunderclap headache after autosexual activity. Cerebral imaging showed ischaemic infarcts, slight cortical subarachnoid haemorrhage, bilateral carotid artery dissection and fluctuating intracranial vessel irregularities, compatible with reversible cerebral vasoconstriction syndrome. An extensive diagnostic work-up was normal. No typical trigger factors of reversible cerebral vasoconstriction syndrome could be found. The patient received intravenous heparin and the calcium channel blocker nimodipine. Followup imaging revealed no vessel irregularities, the left internal carotid artery was still occluded. Conclusion: This case supports the assumption that carotid artery dissection should be considered as a potential trigger of reversible cerebral vasoconstriction syndrome, possibly by altering sympathetic vascular tone.

The Link Between Migraine, Reversible Cerebral Vasoconstriction Syndrome and Cervical Artery Dissection

“…Clinical Evidence for an Association of CeAD and RCVS.-An association between RCVS and CeAD was initially suggested by a number of case reports 12,[73][74][75][76][77][78][79][80][81][82] and subsequently confirmed in a prospective cohort of 20 patients with both conditions, representing 12% of consecutive RCVS patients and 7% of consecutive CeAD patients. 83 In this cohort of 20 patients with both RCVS and CeAD, the clinical presentation of RCVS did not differ from that of isolated RCVS for most characteristics, with severe acute headache in all subjects (recurrent in 75% and unique in 25%) and associated neurological signs in 50% (seizure in 20%, transient focal signs in 20% and/or persistent focal signs in 25%).…”

Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical Course

“…44 Furthermore, PRES and RCVS often arise concurrently as complications of various medical conditions, including intravenous immunoglobin therapy, Guillain-Barre syndrome, immunosuppression, stem cell transplantation, blood transfusions, and septic shock. 2,7,28,54,56,66,67 Finally, both PRES and RCVS demonstrate similar clinical features, including an acute, self-limited course and symptomatology, such as headache, confusion, seizure, and transient or permanent neurologic deficits. 1,2,54 Given the significant overlap between the 2 entities, it is possible that RCVS and PRES may represent a spectrum of potential clinical manifestations of a common underlying pathophysiology involving various degrees of altered cerebral vascular tone and endothelial dysfunction.…”

“…6 A diverse group of possible exogenous triggers for secondary RCVS have been proposed, though the potential delay between exposure and development of the syndrome (in some cases weeks to months) and the ubiquity of some triggers (coughing, laughing, and so forth) raise the possibility that some of these associations may be coincidental (Table 1). 2,3,6,11,23,[28][29][30][31][32][33][34][35][36][37][38] However, the association of RCVS with the most commonly reported triggers is more compelling, including the use of vasoactive drugs and the postpartum state, which together account for more than half of cases in most published series (approximately 50% and 9%-10% of cases respectively). 7,17,39 Sympathomimetic drugs commonly taken over the counter for upper respiratory tract infections, including phenylpropanolamine and pseudoephedrine, as well as antimigrainous medications, have historically been associated with subarachnoid hemorrhage and ischemic stroke in rare cases, which in retrospect likely reflects the sequelae of drug-induced RCVS.…”

Reversible cerebral vasoconstriction syndrome – A narrative revision of the literature

“…17 This finding and the ubiquity of precipitating factors may be a result of chance in part of these associations. 8,16,17,28,[39][40][41][42][43][44][45][46][47][48][49][50][51] In spontaneous cases, there may be a genetic predisposition or there might be some unknown aetiologies that make individuals particularly susceptible. 16 …”