☆ 4.6 Review

Reversible cerebral vasoconstriction syndrome: A review of pathogenesis, clinical presentation, and treatment

INTERNATIONAL JOURNAL OF STROKE (2023)

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Article Clinical Neurology

Vasoconstriction and long-term headache in reversible cerebral vasoconstriction syndrome

Neha Kumar et al.

Summary: The study aims to examine the sensitivity and specificity of a newly developed score (RCVS2) for vasoconstriction detection in a real-world clinical context and describe the incidence and predictors of long-term headaches (LTH) beyond the acute phase of reversible cerebral vasoconstriction syndrome (RCVS). The results showed that approximately 50% of RCVS patients experienced LTH, and a prior history of headache was associated with its incidence. The RCVS2 score had a significant association with high specificity and positive predictive value for angiographic vasoconstriction.

JOURNAL OF NEUROLOGY (2023)

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Article Neurosciences

Fulminant Reversible Cerebral Vasoconstriction Syndrome in Breakthrough COVID 19 Infection

Somdattaa Ray et al.

Summary: We report a case of fulminant reversible cerebrovascular constriction syndrome (RCVS) in a patient with breakthrough COVID 19 infection who was fully vaccinated. RCVS following COVID infection has been reported to have a benign outcome. However, despite vaccination, fulminant RCVS following a breakthrough COVID infection was observed in our patient.

JOURNAL OF STROKE & CEREBROVASCULAR DISEASES (2022)

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Article Clinical Neurology

Type of headache at onset and risk for complications in reversible cerebral vasoconstriction syndrome

Kristin Sophie Lange et al.

Summary: Patients with reversible cerebral vasoconstriction syndrome (RCVS) who do not present with thunderclap headache (TCH) at onset have a higher risk for neurological complications, including cervical artery dissections, posterior reversible encephalopathy syndrome, seizures, and subarachnoid hemorrhage, compared to those with TCH. However, the functional outcome at 3 months is similar in both groups, with most patients having a good prognosis. Further multicentric studies are needed to confirm these findings.

EUROPEAN JOURNAL OF NEUROLOGY (2022)

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Article Immunology

COVID-19 Vaccination Might Induce Reversible Cerebral Vasoconstriction Syndrome Attacks: A Case Report

Anne Marie Lund et al.

Summary: A 30-year-old male diagnosed with reversible cerebral vasoconstriction syndrome (RCVS) experienced a series of attacks resembling RCVS after receiving the first Pfizer COVID-19 vaccine. However, treatment with the angiotensin 2 receptor antagonist, losartan, proved effective in preventing further RCVS attacks.

VACCINES (2022)

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Review Cell Biology

Pathophysiology of reversible cerebral vasoconstriction syndrome

Shih-Pin Chen et al.

Summary: Reversible cerebral vasoconstriction syndrome (RCVS) is a complex neurovascular disorder characterized by sudden severe headaches and widespread cerebral vasoconstriction. The pathophysiology of RCVS is not fully understood, but dysfunction of cerebral vascular tone and impairment of blood-brain barrier are believed to play key roles. Other factors such as genetic predisposition, sympathetic overactivity, endothelial dysfunction, and oxidative stress may also contribute to the development of RCVS.

JOURNAL OF BIOMEDICAL SCIENCE (2022)

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Article Clinical Neurology

Circulating microRNAs Associated With Reversible Cerebral Vasoconstriction Syndrome

Shih-Pin Chen et al.

Summary: This study identified a panel of miRNAs that distinguished patients with RCVS from controls, with let-7a-5p, let-7b-5p, and let-7f-5p showing higher abundance in patients with ictal migraine. Target prediction and pathway analysis suggested their involvement in RCVS pathogenesis, which was confirmed in vitro. MiR-130a-3p was associated with disruption of the blood-brain barrier and increased permeability in a human BBB model.

ANNALS OF NEUROLOGY (2021)

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Article Neurosciences

Reversible Cerebral Vasoconstriction Syndrome in Patients with Coronavirus Disease: A Multicenter Case Series

Kristine Arandela et al.

Summary: This study describes the clinical features, risk factors, and outcomes of RCVS in a group of COVID-19 patients. The findings suggest that COVID-19 patients may be at risk for developing RCVS, especially with additional risk factors such as exposure to vasoactive agents. There was variability in symptoms and severity of COVID-19, clinical characteristics, imaging abnormalities, and mRS scores among the patients with RCVS.

JOURNAL OF STROKE & CEREBROVASCULAR DISEASES (2021)

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Review Clinical Neurology

Reversible cerebral vasoconstriction syndrome in children: an update

Angel R. Maldonado-Soto et al.

Summary: Headaches are a common cause of emergency room visits in pediatric patients, with thunderclap headaches potentially being associated with reversible cerebral vasoconstriction syndrome (RCVS) in children. RCVS is characterized by diffuse vasoconstriction of the cerebral arterial vasculature and can lead to recurrent severe headaches, as well as focal neurological deficits. While RCVS is better understood in adults, it also occurs in children with some differences in presentation.

SEMINARS IN PEDIATRIC NEUROLOGY (2021)

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Article Clinical Neurology

Early Risk of Readmission Following Hospitalization for Reversible Cerebral Vasoconstriction Syndrome

Aayushi Garg et al.

Summary: Nearly 14% of patients with RCVS are readmitted within 90 days of discharge, with a significant proportion of readmissions due to ongoing or recurrent symptoms of RCVS. Diabetes, history of tobacco use, opioid use, and longer index hospitalization length were independent predictors of 90-day readmission.

NEUROLOGY (2021)

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Article Multidisciplinary Sciences

RCVS-TCH score can predict reversible cerebral vasoconstriction syndrome in patients with thunderclap headache

Soohyun Cho et al.

Summary: A new scoring system (RCVS-TCH score) was developed to predict reversible cerebral vasoconstriction syndrome (RCVS) in patients with thunderclap headache (TCH), showing good performance with a high sensitivity and specificity in discriminating RCVS from non-RCVS. The four predictors included recurrent TCHs, female sex, triggering factor for TCH, and blood pressure surge. Validation of the RCVS-TCH score demonstrated its effectiveness in diagnosing RCVS among TCH patients.

SCIENTIFIC REPORTS (2021)

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Article Clinical Neurology

Blood-Brain Barrier Permeability in Patients With Reversible Cerebral Vasoconstriction Syndrome Assessed With Dynamic Contrast-Enhanced MRI

Chia-Hung Wu et al.

Summary: The study found that patients with RCVS present increased microscopic brain permeability during the acute stage, even without discernible macroscopic BBB disruption. The dynamic changes in BBB permeability may be related to impaired cerebral microvascular compliance and WMH formation.

NEUROLOGY (2021)

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Article Clinical Neurology

Reversible Cerebral Vasoconstriction Syndrome Symptoms, Incidence, and Resource Utilization in a Population-Based US Cohort

Jessica Magid-Bernstein et al.

Summary: The study estimated the age- and sex-standardized incidence of hospitalization for reversible cerebral vasoconstriction syndrome (RCVS) in US adults to be approximately 3 cases per million per year. Many patients with RCVS had concomitant neurologic diagnoses, including subarachnoid hemorrhage, ischemic stroke, and intracerebral hemorrhage.

NEUROLOGY (2021)

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Article Clinical Neurology

Outcomes Among Patients With Reversible Cerebral Vasoconstriction Syndrome A Nationwide United States Analysis

Smit D. Patel et al.

Summary: RCVS is a well-established cause of stroke, affecting middle-aged women, with a portion of patients requiring rehabilitation or nursing home care, but inpatient mortality is low.

STROKE (2021)

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Article Clinical Neurology

Early Risk of Readmission Following Hospitalization for Reversible Cerebral Vasoconstriction Syndrome

Aayushi Garg et al.

Summary: Nearly 14% of patients with reversible cerebral vasoconstriction syndrome (RCVS) are readmitted within 90 days of discharge, with factors such as diabetes, history of tobacco use, opioid use, and longer length of index hospitalization being independent predictors of readmission. The most common reasons for readmissions are acute cerebrovascular events, continued or recurrent RCVS symptoms, infections, and headache, with a significant proportion of patients not being discharged to home after readmission.

NEUROLOGY (2021)

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Article Clinical Neurology

Concomitant reversible cerebral vasoconstriction syndrome and transient global amnesia

Rosalie Boitet et al.

JOURNAL OF NEUROLOGY (2020)

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Article Clinical Neurology

Urine metabolomics signatures in reversible cerebral vasoconstriction syndrome

Wei-Hsiang Hsu et al.

CEPHALALGIA (2020)

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Long-Term Outcomes After Reversible Cerebral Vasoconstriction Syndrome

Rosalie Boitet et al.

STROKE (2020)

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Temporal Profile of Blood-Brain Barrier Breakdown in Reversible Cerebral Vasoconstriction Syndrome

Soohyun Cho et al.

STROKE (2020)

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The clinical spectrum of reversible cerebral vasoconstriction syndrome: The Italian Project on Stroke at Young Age (IPSYS)

Filomena Caria et al.

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Review Clinical Neurology

Fatal reversible cerebral vasoconstriction syndrome: A systematic review of case series and case reports

Maria Valencia-Mendoza et al.

JOURNAL OF CLINICAL NEUROSCIENCE (2019)

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Article Clinical Neurology

RCVS2 score and diagnostic approach for reversible cerebral vasoconstriction syndrome

Eva A. Rocha et al.

NEUROLOGY (2019)

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Editorial Material Emergency Medicine

Managing Patients With Nontraumatic, Severe, Rapid-Onset Headache

Jonathan A. Edlow

ANNALS OF EMERGENCY MEDICINE (2018)

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Fatal reversible cerebral vasoconstriction syndrome

Amy Kunchok et al.

JOURNAL OF THE NEUROLOGICAL SCIENCES (2018)

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Primary angiitis of the CNS and reversible cerebral vasoconstriction syndrome A comparative study

Hubert de Boysson et al.

NEUROLOGY (2018)

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Article Clinical Neurology

Characteristics and demographics of reversible cerebral vasoconstriction syndrome: A large prospective series of Korean patients

Hyun Ah Choi et al.

CEPHALALGIA (2018)

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Article Clinical Neurology

Serial testing of the ICHD-3 beta diagnostic criteria for probable reversible cerebral vasoconstriction syndrome: A prospective validation study

Mi Ji Lee et al.

CEPHALALGIA (2018)

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Article Clinical Neurology

Cerebral vasomotor reactivity in reversible cerebral vasoconstriction syndrome

Mehmet Akif Topcuoglu et al.

CEPHALALGIA (2017)

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Article Clinical Neurology

Coexisting vascular lesions in reversible cerebral vasoconstriction syndrome

M. Akif Topcuoglu et al.

CEPHALALGIA (2017)

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Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome

Aneesh B. Singhal et al.

NEUROLOGY (2017)

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Cerebral Arteriopathies, Venous Thrombosis, and Migraine

Erica C. S. Camargo et al.

SEMINARS IN NEUROLOGY (2017)

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Association Between Cirrhosis and Stroke in a Nationally Representative Cohort

Neal S. Parikh et al.

JAMA NEUROLOGY (2017)

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Blood-Brain Barrier Breakdown in Reversible Cerebral Vasoconstriction Syndrome: Implications for Pathophysiology and Diagnosis

Mi Ji Lee et al.

ANNALS OF NEUROLOGY (2017)

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Long-term outcomes after reversible cerebral vasoconstriction syndrome

Seby John et al.

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Reversible Cerebral Vasoconstriction Syndromes and Primary Angiitis of the Central Nervous System: Clinical, Imaging, and Angiographic Comparison

Aneesh B. Singhal et al.

ANNALS OF NEUROLOGY (2016)

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Reversible Cerebral Vasoconstriction Syndrome Without Typical Thunderclap Headache

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Hemorrhagic Reversible Cerebral Vasoconstriction Syndrome Features and Mechanisms

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Gender and hormonal influences in reversible cerebral vasoconstriction syndrome

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Magnesium for Treatment of Reversible Cerebral Vasoconstriction Syndrome: Case Series

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A Novel Approach to Diagnose Reversible Cerebral Vasoconstriction Syndrome: A Case Series

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Reversible cerebral vasoconstriction syndrome with reversible renal artery stenosis

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Recurrence of reversible cerebral vasoconstriction syndrome A long-term follow-up study

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A Novel Approach to Diagnose Reversible Cerebral Vasoconstriction Syndrome: A Case Series

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Article Clinical Neurology

Reduced circulating endothelial progenitor cells in reversible cerebral vasoconstriction syndrome

Shih-Pin Chen et al.

JOURNAL OF HEADACHE AND PAIN (2014)

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Clinical Worsening in Reversible Cerebral Vasoconstriction Syndrome

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JAMA NEUROLOGY (2014)

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Article Biochemistry & Molecular Biology

Oxidative stress and increased formation of vasoconstricting F2-isoprostanes in patients with reversible cerebral vasoconstriction syndrome

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Autonomic dysfunction in reversible cerebral vasoconstriction syndromes

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JOURNAL OF HEADACHE AND PAIN (2013)

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Reversible cerebral vasoconstriction syndrome and cervical artery dissection in 20 patients

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Fulminant Postpartum Cerebral Vasoconstriction Syndrome

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Hyperintense vessels on flair imaging in reversible cerebral vasoconstriction syndrome

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Reversible cerebral vasoconstriction syndrome

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Vessel Wall MRI to Differentiate Between Reversible Cerebral Vasoconstriction Syndrome and Central Nervous System Vasculitis Preliminary Results

Daniel M. Mandell et al.

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Reversible Cerebral Vasoconstriction Syndromes Analysis of 139 Cases

Aneesh B. Singhal et al.

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Diagnostic challenges in RCVS, PACNS, and other cerebral arteriopathies

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Brain-Derived Neurotrophic Factor Gene Val66Met Polymorphism Modulates Reversible Cerebral Vasoconstriction Syndromes

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PLOS ONE (2011)

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Reversible cerebral vasoconstriction syndrome with posterior leucoencephalopathy after oral contraceptive pills

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S. Kumar et al.

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Hemorrhagic Manifestations of Reversible Cerebral Vasoconstriction Syndrome Frequency, Features, and Risk Factors

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Dantrolene Mediates Vasorelaxation in Cerebral Vasoconstriction: A Case Series

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Case 8-2009: A 36-Year-Old Woman with Headache, Hypertension, and Seizure 2 Weeks Post Partum.

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Transcranial color Doppler study for reversible cerebral vasoconstriction syndromes

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ANNALS OF NEUROLOGY (2008)

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Catheter angiography, MR angiography, and MR perfusion in posterior reversible encephalopathy syndrome

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Recurrent primary thunderclap headache and benign CNS angiopathy - Spectra of the same disorder?

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Thunderclap headache

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Postpartum angiopathy with reversible posterior leukoencephalopathy

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Evaluation of hyperintense vessels on FLAIR MRI for the diagnosis of multiple intracerebral arterial stenoses

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