Reversible cerebral vasoconstriction syndrome: literature review

Abstract

Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a neurovascular condition characterized by a severe sudden-onset headache that may be associated with focal neurological deficits. On imaging, the suggestive finding corresponds to multifocal vasoconstriction of the cerebral arteries, with a spontaneous resolution of approximately 12 weeks. The identification of precipitating factors and diagnosis must be carried out early, so that adequate management is established and the patient has a good prognosis, given the risk of secondary complications and residual neurological deficits. This study consists of a literature review based on the analysis of articles published between 2017 and 2022 in PubMed, SciELO, and ScienceDirect on RCVS, intending to understand the clinical and radiological characteristics, diagnosis, treatment, and prognosis of patients with RCVS. The pathophysiology, drug management, and prognosis still lack solid evidence; therefore, further studies on RCVS are needed to expand medical knowledge and avoid underdiagnosis and inadequate treatment of this important condition.

Concurrent spontaneous coronary dissection and reversible cerebral vasoconstriction syndrome during postnatal care

“…This condition is characterized by recurrent thunderclap headaches with or without other neurological symptoms, along with reversible multifocal cerebral vasoconstriction of medium-sized cerebral vessels [ 2 ]. The acute headache may be accompanied by vomiting, nausea, photophobia, phonophobia, and in some cases, seizures [ 12 ]. The diagnosis of RCVS is based on consistent clinical characteristics, physical examination, and neurovascular imaging with CT or MRA, revealing multifocal cerebral vasoconstrictions [ 2 , 4 , 13 ].…”

“…The diagnosis of RCVS is based on consistent clinical characteristics, physical examination, and neurovascular imaging with CT or MRA, revealing multifocal cerebral vasoconstrictions [ 2 , 4 , 13 ]. Furthermore, it is recommended to undergo a follow-up imaging test to demonstrate complete or at least significant improvement of vasoconstriction [ 12 , 14 ]. Approximately 25–33% of patients may experience complications such as seizures, ischemic stroke, brain hemorrhage, and posterior reversible encephalopathy syndrome according to previous reports [ 2 , 12 ].…”

“…Furthermore, it is recommended to undergo a follow-up imaging test to demonstrate complete or at least significant improvement of vasoconstriction [ 12 , 14 ]. Approximately 25–33% of patients may experience complications such as seizures, ischemic stroke, brain hemorrhage, and posterior reversible encephalopathy syndrome according to previous reports [ 2 , 12 ]. The majority of patients with RCVS show a self-limiting course, resulting in full recovery or only a few sequelae.…”

“…The majority of patients with RCVS show a self-limiting course, resulting in full recovery or only a few sequelae. The symptom of a headache usually improves within 3 weeks, while vasoconstriction in imaging tests typically improves within 3 months [ 2 , 12 , 15 ]. The patients require close monitoring for potential complications and, if experiencing symptoms, they should receive prompt medical treatment, which may include analgesia, antiepileptics, and antiemetics if necessary.…”

An Atypical Presentation of Childhood Paraganglioma with Seizures: A Case Report and Review of the Literature

“…It is characterised by thunder-clap headaches and neurological symptoms though rare [ 6 , 24 ]. Conditions of sympathomimetic overactivity as can occur in secretory PGLs are thought to be associated with vasoconstriction seen in the syndrome [ 25 , 26 ].…”