Reversible Cerebral Vasoconstriction Syndrome: Recognition and Treatment

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare but increasingly recognized disorder with over 500 cases published in the literature. The condition is characterized by recurrent severe thunderclap headaches with or without other neurological symptoms and diffuse segmental narrowing of the cerebral arteries which is reversible within 3 months. RCVS may occur spontaneously but in over 50% of cases, it is associated with various other conditions, including vasoactive medications or illicit drugs and the post-partum state. One third to a half of cases develop hemorrhagic or ischemic brain lesions or a combination of both. Posterior reversible encephalopathy syndrome (PRES) often occurs in association with RCVS and the conditions are likely to share a common pathophysiology. The pathogenesis of RCVS remains uncertain but autonomic dysregulation, oxidative stress, and genetic predisposition are postulated. Significant differential diagnoses include subarachnoid hemorrhage (SAH) due to aneurysmal rupture, cervical artery dissection, and primary angiitis of the central nervous system (PACNS). Although there is no proven treatment, calcium channel antagonists including nimodipine and verapamil have been administered with reported reduction of headache intensity but without effect on the time course of cerebral vasoconstriction. Glucocorticoids have been reported as an independent predictor of worse outcome and should be avoided. The cornerstone of RCVS management remains largely supportive with bed rest and analgesics and removal of precipitating factors. Invasive neurointerventional techniques should be reserved for severe deteriorating cases. The condition is usually benign and self-limited and the majority of patients have a favorable outcome but around 5-10% are left with permanent neurological deficits and rare cases may die. This review details the importance of the early recognition of this increasingly described condition and current treatment recommendations.

Intracerebral Hemorrhages Secondary to Reversible Cerebral Vasoconstriction Syndrome: Case Report and Literature Review

“…The syndrome has been described since the 1960s with several names, including Call-Fleming syndrome, postpartum angiopathy, migrainous vasospasm, benign angiopathy of the CNS, benign cerebral vasculitis, CNS pseudovasculitis and reversible cerebral segmental vasoconstriction [2][3][4]. There are case reports of patients aged 9 to 76 years, but the syndrome typically occurs between 20-50 years (mean age of 42-45 years) and is more prevalent in women (2: 1 to 10: 1, varying among the series in the literature) [1,3].…”

“…Long-term prognosis depends on the presence or absence of stroke associated with the syndrome. Less than 10% of patients develop permanent deficits and less than 2% die [1].…”

“…The increase in the number of diagnoses observed recently may be related to a higher level of suspicion and improvement of the imaging techniques. More than 500 cases have been described in the literature, with one third of patients evolving with cerebral ischemia, intracranial hemorrhage or both, and only 5-10% with permanent deficits or death [1]. We report a case of a patient with RCVS that presented two foci of intracerebral hemorrhage besides bilateral subarachnoid hemorrhage, with neurological deficit ipsilateral to the biggest intracerebral lesion.…”

Cesarean section under general anesthesia for antepartum reversible cerebral vasoconstriction syndrome: A case report

“…As early recognition is critical, acute peripartum headache should lead to magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA), to search for arterial multifocal vasoconstriction, suggesting the diagnosis of RCVS . The condition is usually benign, with favorable outcome without neurologic deficit, but fatal cases have been reported . Associated abnormalities such as cervical artery dissection must be screened for during evolution of the pathology.…”

Reversible cerebral vasoconstriction syndrome presenting as convexity subarachnoid haemorrhage and posterior reversible encephalopathy syndrome during postpartum: a case report and literature review

“…Reversible cerebral vasoconstriction syndrome (RCVS) is quite a scarce clinical-radiological syndrome that can lead to death. It is with the characteristics of recurrent severe thunderclap headaches with or without focal neurological deficits and diffuse segmental constriction of the cerebral arteries which is usually spontaneously reversible within 3 months [1]. This recognized syndrome is increasingly supposedly due to a transient disturbance in the control of cerebral vascular tone with sympathetic overactivity.…”