Pathophysiology of reversible cerebral vasoconstriction syndrome

Abstract Reversible cerebral vasoconstriction syndrome (RCVS) is a complex neurovascular disorder being recognized during the past two decades. It is characterized by multiple abrupt severe headaches and widespread cerebral vasoconstrictions, with potential complications such as ischemic stroke, con...

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Main Authors: Shih-Pin Chen, Shuu-Jiun Wang
Format: Article
Language:English
Published: BMC 2022-09-01
Series:Journal of Biomedical Science
Subjects:
Online Access:https://doi.org/10.1186/s12929-022-00857-4
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author Shih-Pin Chen
Shuu-Jiun Wang
author_facet Shih-Pin Chen
Shuu-Jiun Wang
author_sort Shih-Pin Chen
collection DOAJ
description Abstract Reversible cerebral vasoconstriction syndrome (RCVS) is a complex neurovascular disorder being recognized during the past two decades. It is characterized by multiple abrupt severe headaches and widespread cerebral vasoconstrictions, with potential complications such as ischemic stroke, convexity subarachnoid hemorrhage, intracerebral hemorrhage and posterior reversible encephalopathy syndrome. The clinical features, imaging findings, and dynamic disease course have been delineated. However, the pathophysiology of RCVS remains elusive. Recent studies have had substantial progress in elucidating its pathogenesis. It is now believed that dysfunction of cerebral vascular tone and impairment of blood–brain barrier may play key roles in the pathophysiology of RCVS, which explains some of the clinical and radiological manifestations of RCVS. Some other potentially important elements include genetic predisposition, sympathetic overactivity, endothelial dysfunction, and oxidative stress, although the detailed molecular mechanisms are yet to be identified. In this review, we will summarize what have been revealed in the literature and elaborate how these factors could contribute to the pathophysiology of RCVS.
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spelling doaj.art-8eba5141f1ce48aba35d304dd12288d82022-12-22T03:18:05ZengBMCJournal of Biomedical Science1423-01272022-09-0129111310.1186/s12929-022-00857-4Pathophysiology of reversible cerebral vasoconstriction syndromeShih-Pin Chen0Shuu-Jiun Wang1Department of Neurology, Neurological Institute, Taipei Veterans General HospitalDepartment of Neurology, Neurological Institute, Taipei Veterans General HospitalAbstract Reversible cerebral vasoconstriction syndrome (RCVS) is a complex neurovascular disorder being recognized during the past two decades. It is characterized by multiple abrupt severe headaches and widespread cerebral vasoconstrictions, with potential complications such as ischemic stroke, convexity subarachnoid hemorrhage, intracerebral hemorrhage and posterior reversible encephalopathy syndrome. The clinical features, imaging findings, and dynamic disease course have been delineated. However, the pathophysiology of RCVS remains elusive. Recent studies have had substantial progress in elucidating its pathogenesis. It is now believed that dysfunction of cerebral vascular tone and impairment of blood–brain barrier may play key roles in the pathophysiology of RCVS, which explains some of the clinical and radiological manifestations of RCVS. Some other potentially important elements include genetic predisposition, sympathetic overactivity, endothelial dysfunction, and oxidative stress, although the detailed molecular mechanisms are yet to be identified. In this review, we will summarize what have been revealed in the literature and elaborate how these factors could contribute to the pathophysiology of RCVS.https://doi.org/10.1186/s12929-022-00857-4Reversible cerebral vasoconstriction syndromeThunderclap headacheBlood–brain barrierNeurovascular unit
spellingShingle Shih-Pin Chen
Shuu-Jiun Wang
Pathophysiology of reversible cerebral vasoconstriction syndrome
Journal of Biomedical Science
Reversible cerebral vasoconstriction syndrome
Thunderclap headache
Blood–brain barrier
Neurovascular unit
title Pathophysiology of reversible cerebral vasoconstriction syndrome
title_full Pathophysiology of reversible cerebral vasoconstriction syndrome
title_fullStr Pathophysiology of reversible cerebral vasoconstriction syndrome
title_full_unstemmed Pathophysiology of reversible cerebral vasoconstriction syndrome
title_short Pathophysiology of reversible cerebral vasoconstriction syndrome
title_sort pathophysiology of reversible cerebral vasoconstriction syndrome
topic Reversible cerebral vasoconstriction syndrome
Thunderclap headache
Blood–brain barrier
Neurovascular unit
url https://doi.org/10.1186/s12929-022-00857-4
work_keys_str_mv AT shihpinchen pathophysiologyofreversiblecerebralvasoconstrictionsyndrome
AT shuujiunwang pathophysiologyofreversiblecerebralvasoconstrictionsyndrome