scholarly journals Reversible Cerebral Vasoconstriction Syndrome Associated with Infundibular Dilation

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.

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.


BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Bin Lin ◽  
Chao Wang ◽  
Nan Lu ◽  
Le Zhang ◽  
Biao Jiang

Abstract Background With the increasing production and use of ethylene oxide (EO) worldwide, its explicit bio-toxicity has drawn more and more attention. At present, most studies focus on chronic EO exposure. Studies on acute EO exposure are rare, especially with imaging studies. To our knowledge, this work is the first documented case of reversible cerebral vasoconstriction syndrome (RCVS) with cerebral infarction caused by EO. Case presentation A 58-year-old woman who worked in a capsule production factory got an unprotected acute EO inhalation due to accidental exposure to sterilization gas. She suffered from nausea, vomiting, and severe paroxysmal headaches, but the first brain MRI scan of the patient showed no significant abnormality. Nine days after inhalation, she developed recurrent thunderclap headaches and gradual complete blindness. The follow-up brain MRI, 12 days after inhalation, demonstrated extensive cytotoxic edema. Fifteen days and 21 days after EO (ethylene oxide) inhalation, head MRA and CTA respectively showed diffuse vasoconstriction of cerebral arteries. Fifty-nine days after EO inhalation, head MRA assessed reversibility of the vasoconstriction. According to clinical features and imaging findings, RCVS with cerebral infarction can be diagnosed. The patient was sensitive to light and light reflection but still blind after symptomatic and rehabilitation therapy. Conclusions We report an acute EO exposure case in which the patient suffered from RCVS with cerebral infarction, which previous literature has not reported. This article aimed to raise awareness of encephalopathy after EO acute exposure.


2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Joel M. Stary ◽  
Bonnie H. Wang ◽  
Seong-Jin Moon ◽  
Huan Wang

Reversible cerebral vasoconstriction syndrome (RCVS) refers to a number of disorders characterized by severe and sudden-onset (“thunderclap”) headaches and angiographic features of reversible, segmental, multifocal vasoconstriction of cerebral arteries. Although RCVS generally resolves without significant sequelae, a rare and possibly underrecognized hemorrhagic presentation has a worse potential course. We report three cases of hemorrhagic RCVS and review the literature. Three females (42, 54, and 33 years old, resp.) presented with severe headache, neurological deficits, and dramatic intracerebral hemorrhage (ICH). Patient 1 presented comatose with a 9 × 4 × 6.6 cm left deep intraparenchymal hemorrhage (IPH) and 1 cm midline shift. She underwent emergent surgical intervention. Patient 2 had a 3.3 × 1.5 cm left superior frontal IPH that enlarged to 4 × 2.5 cm within 12 hours with worsening headache and neurological deficits. She was successfully managed nonoperatively. Patient 3, after uncomplicated pregnancy and delivery, presented with a 1.5 cm left superior parietal IPH on postpartum day 7. Two days later, she acutely developed right hemiplegia. Repeat CT demonstrated a new 3.3 × 1.7 cm left frontal IPH. She was also successfully managed nonoperatively. Many diverse conditions are grouped within the category of RCVS. Dramatic ICH remains a rare and possibly underrecognized presenting feature. Prompt diagnosis and management are essential for obtaining the best outcome.


2016 ◽  
Vol 8 (4) ◽  
Author(s):  
Hideaki Ueki ◽  
Yasushi Sanayama ◽  
Akiyo Miyajima ◽  
Taichiro Tsuchimochi ◽  
Shunji Igarashi ◽  
...  

Reversible cerebral vasoconstriction syndrome (RCVS) is a syndrome characterized by severe headache with segmental vasoconstriction of the cerebral arteries that resolves within 12 weeks. A 16-year-old girl with refractory cytopenia of childhood, who was receiving the immunosuppressant cyclosporine, developed severe headache and was diagnosed with RCVS using magnetic resonance imaging, including magnetic resonance angiography (MRA). MRA is a non-invasive and very effective technique for diagnosing RCVS. MRA should be performed at the onset of severe headache during immunosuppressant administration for children with hematological disorders and may prevent sequelae such as posterior reversible encephalopathy syndrome or ischemic attack.


Cephalalgia ◽  
2017 ◽  
Vol 38 (6) ◽  
pp. 1207-1210 ◽  
Author(s):  
Sho Murase ◽  
Yasufumi Gon ◽  
Akihiro Watanabe ◽  
Kenichi Todo ◽  
Nobuo Kohara ◽  
...  

Background The temporal and anatomical features of vasoconstriction in patients with reversible cerebral vasoconstriction syndrome within hours after symptom onset, in the hyperacute phase, are unclear. Case result Herein we report the cases of two patients with acute severe headache who were diagnosed with reversible cerebral vasoconstriction syndrome. Magnetic resonance imaging within hours after symptom onset revealed multiple areas of isolated cortical vasogenic edema and hyperintense vessel signs of the distal cerebral arteries. Follow-up imaging performed four days later in both cases showed diffuse segmental arterial vasoconstriction in the proximal regions of the cerebral arteries. Both patients received antivasoconstrictive therapy shortly after admission, and neither had neurological sequelae at discharge. The magnetic resonance imaging findings improved gradually within three months after symptom onset. Conclusion Isolated cortical vasogenic edema and hyperintense vessel signs, when observed within hours from sudden severe headache onset, may be useful early markers of reversible cerebral vasoconstriction syndrome.


2014 ◽  
Vol 13 (2) ◽  
pp. 74-76
Author(s):  
Emily Montague ◽  
◽  
Christopher Murphy ◽  

Reversible cerebral vasoconstriction syndrome (rCVS) is an important cause of acute severe headache that is poorly understood and under-recognised. It typically presents with recurrent thunderclap headaches and is characterised by multifocal, segmental constriction and dilatation of the cerebral arteries, shown by a ‘string of beads’ appearance, on cerebral angiography. We describe a case of rCVS in a 39-year-old male presenting with post-coital thunderclap headaches following a whiplash-type injury.


2020 ◽  
Vol 13 (1) ◽  
pp. e232204
Author(s):  
Pooja Rao ◽  
Michael Francis McCullough ◽  
Jessica Stevens ◽  
Matthew A Edwardson

Stress is under-recognised as a potential causative factor for reversible cerebral vasoconstriction syndrome (RCVS). Here we present a case of RCVS occurring during a time of extreme emotional duress. A 46-year-old female patient with medical history of bipolar disorder developed a severe headache during her father’s funeral. The following day she was discovered to have bilateral hemiparesis, aphasia, encephalopathy and was brought emergently to the hospital. Neuroimaging revealed a 33 mL left fronto-parietal haematoma with subarachnoid blood near the vertex bilaterally. She underwent craniotomy, haematoma evacuation and external ventricular drain placement. The patient received two cerebral angiograms, the first showing multifocal cerebral vasoconstriction and the second showing resolution of these changes. She improved significantly over the course of her 3-week hospitalisation and eventually made a full recovery, including the ability to speak fluently in six languages with no significant deficits other than hypersomnia; she now requires 10 hours of sleep each night as compared with 7 hours prior to her brain injury.


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.


Stroke ◽  
2021 ◽  
Vol 52 (Suppl_1) ◽  
Author(s):  
Aayushi Garg ◽  
Amjad Elmashala ◽  
Santiago Ortega

Introduction: Ischemic stroke is the cause for major morbidity and mortality in reversible cerebral vasoconstriction syndrome (RCVS). While there is evidence to suggest that ischemic stroke in RCVS is associated with proximal vasoconstriction, it is still unclear why some patients develop ischemic lesions. The aim of this study was to evaluate the risk factors and outcomes of ischemic stroke in RCVS. Methods: We utilized the Nationwide Readmissions Database 2016-2017 to identify all hospitalizations with the discharge diagnosis of RCVS. Occurrence of acute ischemic stroke was identified. Hospitalizations with the diagnosis of hemorrhagic stroke were excluded. Survey design methods were used to generate national estimates. Independent predictors of ischemic stroke were analyzed using multivariable logistic regression analysis with results expressed as odds ratio (OR) and 95% confidence intervals (CI). Results: Among the total 1,065 hospitalizations for RCVS during the study period (mean±SD age: 49.0±16.7 years, female 69.7%), 267 (25.1%) had occurrence of acute ischemic stroke. Patients with ischemic stroke were more likely to have history of hypertension (OR 2.33, 95% CI 1.51-3.60), diabetes (OR 1.81, 95% CI 1.11-2.98), and tobacco use (OR 1.64, 95% CI 1.16-2.33) and less likely to have a history of migraine (OR 0.56, 95% CI 0.35-0.90). Patients with stroke were more likely to develop cerebral edema. They also had longer hospital stay, higher hospital charges, and lower likelihood of being discharged to home or inpatient rehabilitation facility. They had higher in-hospital mortality rate, the difference was however not statistically significant. Conclusion: In conclusion, ischemic stroke affects nearly 25% of patients with RCVS and is associated with an increased rate of other neurologic complications and worse functional outcomes. Patients with traditional cerebrovascular risk factors might have a higher predisposition for developing the ischemic lesions.


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.


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