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

2021 ◽  
Vol 16 (1) ◽  
pp. 78-82
Author(s):  
Paul J Alapatt ◽  
Ajay Panwar ◽  
Gigy Varkey Kuruttukulam ◽  
Kaushik Sundar

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.

Cephalalgia ◽  
2017 ◽  
Vol 38 (5) ◽  
pp. 984-987 ◽  
Author(s):  
Björn Machner ◽  
Tobias Boppel ◽  
Thomas Münte

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.


Author(s):  
Neelu Desai ◽  
Rahul Badheka ◽  
Nitin Shah ◽  
Vrajesh Udani

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.


Neurosurgery ◽  
2002 ◽  
Vol 51 (4) ◽  
pp. 939-943 ◽  
Author(s):  
Yasunari Otawara ◽  
Kuniaki Ogasawara ◽  
Akira Ogawa ◽  
Makoto Sasaki ◽  
Kei Takahashi

Abstract OBJECTIVE Multislice computed tomographic angiography (CTA) can provide clearer vascular images, even of the peripheral arteries, than conventional CTA. Multislice CTA was compared with digital subtraction angiography (DSA) for the detection of cerebral vasospasm in patients with acute aneurysmal subarachnoid hemorrhage (SAH) to analyze whether multislice CTA can replace DSA in the detection of vasospasm after SAH. METHODS Within 72 hours after the onset of symptoms, multislice CTA and DSA were performed in 20 patients with SAH. Multislice CTA and DSA were repeated on Day 7 to assess cerebral vasospasm. Regions of interest were established in the proximal and distal segments of the anterior and middle cerebral arteries on both multislice CTA and DSA images, and the agreement between the severity of vasospasm on multislice CTA and DSA images was statistically compared. The multislice Aquilon computed tomography system (Toshiba, Inc., Tokyo, Japan) used the following parameters: 1 mm collimation and 3.5 mm per rotation table increment (pitch, 3.5). RESULTS The degree of vasospasm as revealed by multislice CTA correlated significantly with the degree of vasospasm revealed by DSA (P < 0.0001). The agreement between the severity of vasospasm on multislice images obtained via CTA and DSA in the overall, proximal, and distal segments of the cerebral arteries was 91.6, 90.8, and 92.3%, respectively. CONCLUSION Multislice CTA can detect angiographic vasospasm after SAH with accuracy equal to that of DSA.


2019 ◽  
Vol 12 (12) ◽  
pp. e230606 ◽  
Author(s):  
Szilard Laszlo Safran ◽  
Christian Balmer ◽  
Georges Savoldelli

We describe the case of a 25-year-old parturient who presented sudden onset and short-lived severe headache caused by reversible cerebral vasoconstriction syndrome (RCVS) during an emergency caesarean section. The syndrome was triggered by phenylephrine administered intravenously to correct arterial hypotension following spinal anaesthesia. RCVS is a clinical and radiological syndrome attributed to transient disturbance in the control of cerebral arterial tone resulting in vasospasms. The syndrome can be precipitated by several triggers, including vasoactive drugs, often used during spinal anaesthesia, illicit drugs, pregnancy and postpartum state. Diagnosis and management can be challenging during pregnancy, peripartum or post partum, since many medications commonly used during these periods must be avoided to prevent triggering RCVS. The aim of this report is to raise the awareness, particularly for anaesthesiologists and obstetricians, of this rare and potentially serious syndrome. We discuss diagnosis, triggers, pathogenesis, clinical course and complications, as well as coordinated multidisciplinary management plans.


BMJ Open ◽  
2020 ◽  
Vol 10 (9) ◽  
pp. e041776
Author(s):  
Ryan Daniel Gotesman ◽  
Naomi Niznick ◽  
Brian Dewar ◽  
Dean A Fergusson ◽  
Risa Shorr ◽  
...  

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.


Cephalalgia ◽  
2009 ◽  
Vol 30 (1) ◽  
pp. 42-45 ◽  
Author(s):  
Y Soo ◽  
AB Singhal ◽  
T Leung ◽  
S Yu ◽  
H Mak ◽  
...  

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.


Author(s):  
Héctor Montenegro-Rosales ◽  
Blanca Karina González-Alonso ◽  
Omar Cárdenas-Sáenz ◽  
Alonso Gutierrez-Romero

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.


2021 ◽  
Author(s):  
Paolla Giovanna Rossito de Magalhães ◽  
Marina Buldrini Filogonio Seraidarian ◽  
Bernardo Tardin Caetano ◽  
Barbara Oliveira Paixão ◽  
Tassila Oliveira Nery de Freitas ◽  
...  

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.


2021 ◽  
Vol 26 (4) ◽  
pp. 829-834
Author(s):  
Arpan Dutta ◽  
Atanu Chandra ◽  
Subhadeep Gupta ◽  
Biman Kanti Ray ◽  
Deep Das ◽  
...  

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.


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