Hyperintense posterior cerebral artery sign in patients with reversible cerebral vasoconstriction syndrome

Background: This study investigated hyperintense vessel signs (HVS) on fluid-attenuated inversion recovery imaging in the P1–2 portions of posterior cerebral arteries (PCAs) as a “hyperintense PCA sign” and HVS of cortical arteries. We retrospectively examined whether these signs would be useful in diagnosing reversible cerebral vasoconstriction syndrome (RCVS) in the acute phase. Methods: Eighty patients with RCVS who underwent initial magnetic resonance imaging (MRI) within 7 days of onset were included in this study. HVS and related clinical factors were examined. Results: On initial MRI of RCVS patients, hyperintense PCA sign and HVS of cortical arteries were seen in 21 cases (26%) and 38 cases (48%), respectively. In patients showing hyperintense PCA sign, vasoconstriction of the A2–3 portion was a significant clinical factor. Conversely, vasoconstriction of the M1 and P1 portions and the presence of white matter hyperintensity on initial and chronic-stage MRI were significantly associated with the presence of HVS in cortical arteries. Conclusion: Because rich collateral flow exists around PCAs, the frequency of hyperintense PCA sign is not high. However, hyperintense PCA sign findings in patients with suspected RCVS offer credible evidence of extreme flow decreases due to vasoconstriction in peripheral PCAs and other arteries associated with the collateral circulation of PCAs. Conversely, HVS in cortical arteries tend to reflect slow antegrade circulation due to vasoconstriction of peripheral vessel and major trunks. Both signs appear useful for auxiliary diagnosis of acute-phase RCVS.

Download Full-text

Pediatric Reversible Cerebral Vasoconstriction Syndrome: Two Unique Cases with a Review of all Reported Children

Journal of Pediatric Neurology ◽

10.1055/s-0041-1722959 ◽

2021 ◽

Author(s):

Neelu Desai ◽

Rahul Badheka ◽

Nitin Shah ◽

Vrajesh Udani

Keyword(s):

Magnetic Resonance ◽

Cerebral Arteries ◽

Brain Mri ◽

Brain Magnetic Resonance Imaging ◽

Reversible Cerebral Vasoconstriction Syndrome ◽

Neurological Deficits ◽

Cerebral Vasoconstriction ◽

Magnetic Resonance Imaging Mri ◽

Reversible Encephalopathy ◽

Mr Angiogram

AbstractReversible cerebral vasoconstriction syndrome (RCVS) has been well described in adults, but pediatric cases are yet under recognized. We describe two children with RCVS and review similar already published pediatric cases. The first patient was a 10-year-old girl who presented with severe headaches and seizures 3 days after blood transfusion. Brain magnetic resonance imaging (MRI) showed changes compatible with posterior reversible encephalopathy syndrome and subarachnoid hemorrhage. Magnetic resonance angiogram showed diffuse vasoconstriction of multiple cerebral arteries. The second patient was a 9-year-old boy who presented with severe thunderclap headaches. Brain MRI showed isolated intraventricular hemorrhage. Computed tomography/MR angiogram and digital subtraction angiogram were normal. A week later, he developed focal neurological deficits. Repeated MR angiogram showed diffuse vasospasm of multiple intracranial arteries. Both children recovered completely. A clinico-radiological review of previously reported childhood RCVS is provided.

Download Full-text

Lessons Learned from a Fulminant Case of Reversible Cerebral Vasoconstriction Syndrome: Past Medical History Misleads the Diagnosis and Intra-Arterial Milrinone Offers Diagnostic Utility

Neurointervention ◽

10.5469/neuroint.2020.00416 ◽

2021 ◽

Vol 16 (1) ◽

pp. 78-82

Author(s):

Paul J Alapatt ◽

Ajay Panwar ◽

Gigy Varkey Kuruttukulam ◽

Kaushik Sundar

Keyword(s):

Post Partum ◽

Cerebral Arteries ◽

Pulse Therapy ◽

Computed Tomographic Angiography ◽

Lessons Learned ◽

Reversible Cerebral Vasoconstriction Syndrome ◽

Diagnostic Utility ◽

Computed Tomographic ◽

Cerebral Vasoconstriction ◽

Seizure Episode

A 34-year-old post-partum female having dermatomyositis developed headache and became comatose after a seizure episode. Magnetic resonance imaging of brain showed a massive left ganglio-capsular bleed for which decompressive surgery was done. Computed tomographic angiography showed multiple foci of narrowing and irregularities in distal cerebral vessels. In view of dermatomyositis, the diagnosis of vasculitis was considered and pulse therapy of intravenous methylprednisolone was started. The patient, however, showed no improvement and developed new brain infarcts. She was subsequently taken up for a diagnostic cerebral angiography which showed multifocal severe narrowing in bilateral major cerebral arteries. These angiographic abnormalities showed excellent reversibility to intra-arterial milrinone and hence, reversible cerebral vasoconstriction syndrome (RCVS) was diagnosed. Normal angiographic findings in the first week do not rule out the disease and a repeat angiography should be considered if the clinical suspicion of the RCVS is high. Intra-arterial milrinone has a high diagnostic utility.

Download Full-text

Prevalence of non-contrast CT abnormalities in adults with reversible cerebral vasoconstriction syndrome: protocol for a systematic review and meta-analysis

BMJ Open ◽

10.1136/bmjopen-2020-041776 ◽

2020 ◽

Vol 10 (9) ◽

pp. e041776

Author(s):

Ryan Daniel Gotesman ◽

Naomi Niznick ◽

Brian Dewar ◽

Dean A Fergusson ◽

Risa Shorr ◽

...

Keyword(s):

Systematic Review ◽

Meta Analysis ◽

Cerebral Arteries ◽

Reversible Cerebral Vasoconstriction Syndrome ◽

Vascular Lesions ◽

Primary Data ◽

Cochrane Library ◽

Cerebral Vasoconstriction ◽

Contrast Ct ◽

The Impact

IntroductionReversible cerebral vasoconstriction syndrome (RCVS) is characterised by severe, recurrent thunderclap headaches (TCHs) and vasoconstriction of cerebral arteries that resolve within 3 months. Abnormalities on non-contrast CT (NCCT) such as ischaemic strokes, intracerebral haemorrhage and subarachnoid haemorrhages are frequently observed on brain imaging of patients with RCVS though their prevalence varies considerably between studies. The aim of this systematic review and meta-analysis is to estimate the prevalence of NCCT abnormalities seen on neuroimaging of adult patients with RCVS.Methods and analysisWe will search the Medline, Embase and the Cochrane Library databases for studies on the prevalence of NCCT abnormalities on neuroimaging of patients with RCVS. Search results will be screened for eligibility by title and abstract. Suitable studies will be fully reviewed and relevant data extracted using a data abstraction form. The studies will be assessed for methodological quality, risk of bias and heterogeneity. Prevalence estimates across studies will be pooled using a random-effects model and subgroup analysis will be performed to assess the impact of age, sex, publication year and study design on prevalence of vascular lesions. Sensitivity analysis will be used to investigate the robustness of the findings. This protocol has been devised using the Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols 2015 checklist.Ethics and disseminationFormal ethics is not required as primary data will not be collected. The findings of this study will be disseminated through a peer-reviewed publication and conference presentations.Trial registration numberCRD42020190637.

Download Full-text

Isoflavones and gastrointestinal infection: Two potential triggers for reversible cerebral vasoconstriction syndrome

Cephalalgia ◽

10.1177/0333102417714245 ◽

2017 ◽

Vol 38 (5) ◽

pp. 984-987 ◽

Cited By ~ 1

Author(s):

Björn Machner ◽

Tobias Boppel ◽

Thomas Münte

Keyword(s):

Differential Diagnosis ◽

Post Partum ◽

Cerebral Arteries ◽

Thunderclap Headache ◽

Reversible Cerebral Vasoconstriction Syndrome ◽

Gastrointestinal Infection ◽

Cerebral Vasoconstriction ◽

Vasoactive Substances ◽

Important Differential Diagnosis ◽

Estrogenic Potential

Background Reversible cerebral vasoconstriction syndrome (RCVS) is an important differential diagnosis of singular or recurrent thunderclap headache. Prognosis is generally good, however complications of the transient segmental vasospasms of cerebral arteries such as stroke, subarachnoidal hemorrhage and brain edema may worsen the clinical outcome. Although the exact pathomechanism is still unclear, various vasoactive substances and conditions (e.g. post partum) have been identified as triggering RCVS. Cases We report on the clinical course and management of two cases of typical RCVS that were associated with two different precipitants previously not described: A gastrointestinal infection and isoflavones, which are phytoestrogens used for menopausal vasomotor symptoms. Discussion In the case of gastrointestinal infection, either systemic inflammatory processes might lead to disturbances of vascular tone, or the repetitive vomiting that resembles Valsalva manoeuvers known to trigger RCVS. In the case of isoflavone intake, it may be their estrogenic potential that induces dysregulation of cerebral arteries, a mechanism known from other states of hormonal change such as post-partum angiopathy. However, the association of both precipitating factors with RCVS in our two cases is not a proof for a causal relationship, and there may have been additional potential triggers for RCVS. Conclusion In patients with (gastrointestinal) infection and concomitant thunderclap headache, RCVS should be considered as an important differential diagnosis due to its major complications. Since RCVS may be triggered by various vasoactive substances, taking the medical history should always include over-the-counter drugs and dietary supplements (such as the isoflavones) beside the regular medication.

Download Full-text

Reversible Cerebral Vasoconstriction Syndrome with Posterior Leucoencephalopathy after Oral Contraceptive Pills

Cephalalgia ◽

10.1111/j.1468-2982.2009.01868.x ◽

2009 ◽

Vol 30 (1) ◽

pp. 42-45 ◽

Cited By ~ 24

Author(s):

Y Soo ◽

AB Singhal ◽

T Leung ◽

S Yu ◽

H Mak ◽

...

Keyword(s):

Oral Contraceptive ◽

Cerebral Arteries ◽

Haemorrhagic Stroke ◽

Sudden Onset ◽

Reproductive Hormones ◽

Reversible Cerebral Vasoconstriction Syndrome ◽

Neurological Deficits ◽

Oral Contraceptive Pills ◽

Cerebral Vasoconstriction ◽

Contraceptive Pills

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden-onset recurrent ‘thunderclap’ headaches with reversible multifocal narrowing of the cerebral arteries, often associated with focal neurological deficits from ischaemic or haemorrhagic stroke. It has been associated with exposure to vasoconstrictive drugs, pregnancy, migraine, and a variety of other conditions. Whereas the pathophysiology of RCVS remains unclear, changes in the levels of female hormones are considered important because RCVS predominantly affects women and is frequently associated with pregnancy. We report a patient with angiographically confirmed RCVS whose MRI showed reversible brain oedema, suggesting an overlap between RCVS and the reversible posterior leucoencephalopathy syndrome. The only identified risk factor was oral contraceptive pills started 1 month prior to onset, supporting a role for female reproductive hormones in precipitating this overlap syndrome.

Download Full-text

Reversible Cerebral Vasoconstriction Syndrome Associated with Infundibular Dilation

European Journal of Case Reports in Internal Medicine ◽

10.12890/2020_001839 ◽

2020 ◽

Author(s):

Héctor Montenegro-Rosales ◽

Blanca Karina González-Alonso ◽

Omar Cárdenas-Sáenz ◽

Alonso Gutierrez-Romero

Keyword(s):

Cerebral Arteries ◽

Imaging Study ◽

Sudden Onset ◽

Severe Headache ◽

Reversible Cerebral Vasoconstriction Syndrome ◽

Initial Image ◽

Case Presentation ◽

Cerebral Vasoconstriction ◽

History Of ◽

Visual Disturbances

Background: Reversible cerebral vasoconstriction syndrome (RCVS) is defined as a clinical and radiological syndrome that comprises a group of disorders characterized by sudden-onset severe headache and segmental vasoconstriction of the cerebral arteries with resolution within 3 months. Case presentation: A 51-year-old female patient with a 2-week history of sudden-onset severe headache, visual disturbances and cerebellum; no relevant imaging findings, except for an infundibular dilation at the origin of the posterior communicating artery, and so, angiography was performed. When symptoms persisted, a new imaging study was carried out with findings of RCVS as the cause of the symptoms from the beginning. Conclusions: Findings of RCVS can be obtained in various vasculopathies of the nervous system and vasculitis, being misdiagnosed, and so, clinical suspicion is essential; if vasoconstriction is not demonstrated on the initial image and other diagnoses have been excluded, the patient should be managed as having possible or probable RCVS.

Download Full-text

Case Report: Reversible Cerebral Vasoconstriction Syndrome in a cancer patient using ZoladexTM

10.5327/1516-3180.142 ◽

2021 ◽

Author(s):

Paolla Giovanna Rossito de Magalhães ◽

Marina Buldrini Filogonio Seraidarian ◽

Bernardo Tardin Caetano ◽

Barbara Oliveira Paixão ◽

Tassila Oliveira Nery de Freitas ◽

...

Keyword(s):

Case Report ◽

Cerebral Angiography ◽

Postpartum Period ◽

Cerebral Arteries ◽

Superficial Temporal Artery ◽

Chronic Administration ◽

Temporal Artery ◽

Reversible Cerebral Vasoconstriction Syndrome ◽

Gnrh Analogue ◽

Cerebral Vasoconstriction

Context: The Reversible Cerebral Vasoconstriction Syndrome (SVCR) is characterized by rapid and reversible vasoconstriction and segmental dilation of cerebral arteries, usually preceded by thunderclap headache. The involvement of second and third-order branches of the cerebral arteries is the most commom finding in a cerebral angiography. This report is about a SVCR case with atypical involvement, significantly compromising extracranial vessels and raising the hypothesis of association between the use of hormonal blocker gosserelin acetate (ZoladexTM) with SVCR. Case report: Female, 39 years old, with breast cancer and bone metastasis using ZoladexTM that presented with a sudden headache and vomiting, progressing to global afasia and paresis in the right upper limb. Magnetic resonance identified hyperacute intraparenchymal hematoma in left frontoparietal convexity and subarachnoid haemorrhage. Cerebral angiography showed irregularities in the distal branches (M3 and M4) of the middle cerebral arteries, as well as in the superficial temporal artery, characterized by focal strictures. Conclusion: Studies show that hormonal fluctuations in the postpartum period can trigger SVCR due to the drop in estrogen and progesterone (gonadotropins). During postpartum, the stimulus of breastfeeding increases prolactin levels leading to GnRH suppression, which decreases the level of gonadotropins. ZoladexTM is a GnRH analogue and its chronic administration results in suppression of these hormones - similar to the postpartum period. Therefore, there may be an association of hormonal blockers with SVCR.

Download Full-text

Reversible cerebral vasoconstriction syndrome in a patient with COVID-19

Neurology Asia ◽

10.54029/2021auv ◽

2021 ◽

Vol 26 (4) ◽

pp. 829-834

Author(s):

Arpan Dutta ◽

Atanu Chandra ◽

Subhadeep Gupta ◽

Biman Kanti Ray ◽

Deep Das ◽

...

Keyword(s):

Cerebral Angiography ◽

Rare Complication ◽

Cerebral Arteries ◽

Neurological Complications ◽

Cerebrovascular Diseases ◽

Reversible Cerebral Vasoconstriction Syndrome ◽

Angiotensin Converting Enzyme 2 ◽

Cerebral Vasoconstriction ◽

Middle Cerebral Arteries ◽

Novel Coronavirus

COVID-19 infection is well-known to produce different neurological complications, including cerebrovascular diseases. Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by transient segmental vasoconstriction of the cerebral vasculature, has been rarely reported in association with COVID-19 infection. The causative agent, the novel coronavirus (SARS-CoV-2), binds to the angiotensin-converting enzyme 2 (ACE-2) receptors for its entry into the host cell. This leads to downregulation of the ACE-2 and increased activity of the renin-angiotensin-aldosterone (RAAS) axis resulting in sympathetic overactivity and vasoconstriction. This might be the possible mechanism of RCVS in COVID-19. We hereby report a case of RCVS occurring in a SARS-CoV-2 infected patient. This was a 38-year-old male without any comorbidities or risk factors, who presented with headache and confusion. His SARS-CoV-2 RT-PCR was positive. MRI of the brain was normal but cerebral angiography revealed segmental vasoconstriction in bilateral middle cerebral arteries and the terminal part of the internal carotid arteries, which resolved almost completely after 2 weeks. He was treated with oral nimodipine 60 mg every 6 hourly. A database search revealed 2 previous cases of RCVS associated with COVID-19. In conclusion, RCVS is a rare complication of COVID-19. It is possibly under-recognized as only a few COVID-19 patients with headaches undergo cerebral angiography especially when parenchymal brain imaging is normal.

Download Full-text

Abstract TMP1: Distal Hyperintense Vessels Score on Flair is a Marker of Collateral Flow Grade and Outcomes in Patients with Acute Ischemic Stroke After Endovascular Therapy

Stroke ◽

10.1161/str.48.suppl_1.tmp1 ◽

2017 ◽

Vol 48 (suppl_1) ◽

Author(s):

Masatomo Miura ◽

Makoto Nakajima ◽

Takuya Kanamaru ◽

Kazutaka Uchida ◽

Manabu Shirakawa ◽

...

Keyword(s):

Clinical Outcome ◽

Endovascular Therapy ◽

Cerebral Angiography ◽

Favorable Outcome ◽

Stroke Severity ◽

Collateral Flow ◽

Flair Sequence ◽

Inversion Recovery Imaging ◽

Fluid Attenuated Inversion Recovery ◽

American Society

Background: Distal hyperintense vessels (DHV) on fluid-attenuated inversion recovery imaging (FLAIR) is a noninvasive and useful imaging marker that reflects leptomeningeal collateral flow. We investigated relationship between DHV and collateral grade on cerebral angiography, and clinical outcome after endovascular therapy (EVT) in patients with AIS. Methods: We retrospectively analyzed the patients with AIS in two comprehensive stroke centers from October 2013 to February 2016. We selected the patients who underwent FLAIR sequence before EVT. DHV score (range 0-6) was evaluated based on the presence of DHV in each ASPECTS territory (M1-M6). Collateral grades on cerebral angiography were assessed with the American Society of Interventional and Therapeutic Neuroradiology Collateral Grading System (grade 0-4). Favorable clinical outcomes was defined as modified Rankin Scale [mRS] of 0-2 at 90 days. Results: A total of 60 patients (aged 71.2 ± 10.5 years; initial NIHSS score, 15 ± 8; median DWI-ASPECTS, 7) were included. DHV was detected in 42 patients (70%); median DHV score was 2 (IQR, 1-4). Higher DHV score was significantly related to better collateral flow grade ( p <0.001), and favorable outcome ( p <0.001). In multivariate analysis, DHV score predicted favorable outcome (OR 2.83 per 1 point; 95% CI 1.71-5.48; p <0.001), independent of stroke severity or successful reperfusion. Conclusions: DHV score on FLAIR is a marker of collateral flow status and can predict better clinical outcome in patients with AIS after EVT.

Download Full-text

093 Clinical and imaging discordance in reversible cerebral vasoconstriction syndrome

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2018-anzan.92 ◽

2018 ◽

Vol 89 (6) ◽

pp. A37.2-A37 ◽

Cited By ~ 1

Author(s):

Michael J Devlin ◽

Archana Dwivedee ◽

Richard P White

Keyword(s):

Subarachnoid Haemorrhage ◽

Cerebral Arteries ◽

Clinical Status ◽

Reversible Cerebral Vasoconstriction Syndrome ◽

Clinical State ◽

Cerebral Vasoconstriction ◽

Csf Analysis ◽

Elevated Protein ◽

Digital Subtraction Angiogram ◽

Reversible Encephalopathy

IntroductionReversible cerebral vasoconstriction syndrome (RCVS) is to be suspected in cases of thunderclap headache particularly when associated with convex subarachnoid haemorrhage without cerebral aneurysm. Angiographic changes and clinical state do not always correlate. We present a case report from the Townsville Hospital.CaseA 49 year old female presented to another hospital with recurrent thunderclap headaches first commencing with exercise. The medical history included idiopathic intracranial hypertension in remission, chronic migraine treated with botulinum toxin, and depression treated with amitriptyline and desvenlafaxine. MR imaging revealed cerebral vertex subarachnoid haemorrhage with normal MR angiogram of the cerebral vessels. The patient was transferred to our institution in a confused and restless state for cerebral digital subtraction angiogram, which was normal. A lumbar puncture revealed elevated white cells of 17×106/L with normal CSF protein and opening pressure of 39 cm H2O. Nimodipine that had been commenced before the patient became encephalopathic was ceased. Her confusion and headaches resolved but relapsed after three days. A repeat MR brain revealed multiple T2 hyperintensities in bilateral anterior and posterior circulation territories demonstrating diffusion restriction, and MR angiography revealed new segmental strictures and beading of bilateral middle and anterior cerebral arteries. The patient was diagnosed with overlap RCVS/posterior reversible encephalopathy syndrome; desvenlafaxine was ceased, acetazolamide commenced for raised intracranial pressure and a several week course of nimodipine recommenced. The patient was discharged home after resolution of headache and confusion without neurological deficit.ConclusionThis case highlights the dynamic changes of vasoconstriction and that angiographic findings can be discordant from the clinical status. Serial imaging is required to detect abnormalities of RCVS when the clinical suspicion is high. CSF analysis can be normal, however may reveal elevated protein or pleocytosis. The cause of our patient’s RCVS was thought to be the chronic use of desvenlafaxine.

Download Full-text