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Curr Treat Options in Rheum (2020) 6:55–70
DOI 10.1007/s40674-020-00137-y
’
Reversible Cerebral
Vasoconstriction Syndrome:
an Update of Recent Research
Takashi Shimoyama
1
Ken Uchino
1
Rula A. Hajj-Ali
2,*
Address
1
Cerebrovascular Center, Neurological Institute, Cleveland Clinic, Cleveland, OH,
USA
*,2
Department of Rheumatic & Immunologic Disease, Orthopaedic and Rheuma-
tology Institute, Cleveland Clinic, Cleveland, OH, USA
Email: hajjalr@ccf.org
Published online: 24 January 2020
*Springer Nature Switzerland AG 2020
This article is part of the Topical Collection on Vasculitis
KeywordsReversible cerebral vasoconstrictionsyndrome IThunderclap headache IIntracranial vasculopathy IStroke I
Neuroimaging
Abstract
Purpose of the review Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by
recurrent thunderclap headaches (TCH) and reversible segmental and multifocal vasoconstric-
tion of cerebral arteries. Noninvasive neuroimaging such as computed tomography (CT), and
magnetic resonance imaging (MRI) are widely used to assess cerebral vasculature, ischemic or
hemorrhagic stroke, and posterior reversible encephalopathy (PRES). The significant differen-
tial diagnoses of RCVS center on the clinical presentation of headache and similar radiographic
features including aneurysmal subarachnoid hemorrhage (SAH), cervical artery dissection, and
central nervous system vasculitis (CNS-V). In this review we present a comprehensive overview
of RCVS with the newest findings in the reported literature.
Recent findings Pathophysiology: Several surrogate markers including plasma endothelin-1
(E-1) have been investigated to elucidate the pathogenesis of RCVS. Clinical evaluation and
diagnosis: The RCVS
2
score is a simple scoring system has been proposed to distinguish RCVS
from other intracranial vasculopathies. Neuroimaging: High-resolution contract-enhanced MRI
(HR-MRI) vessel wall imaging is a feasible tool in differentiating vessel wall patterns of RCVS
and CNS-V. Treatment: Early initiation of calcium channel blockers effectively prevents
recurrent TCH and shorten the clinical courses.
Summary Clinical scoring system and newer imaging tools are helpful for the early distinction
of RCVS. These approaches may serve increased sensitivity and specificity for the diagnosis and
lead to appropriate management in RCVS patients.
Vasculitis (L Barra, Section Editor)
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