Unique combination of hyperintense vessel sign on initial FLAIR and delayed vasoconstriction on MRA in reversible cerebral vasoconstriction syndrome: A case report

Cephalalgia ◽  
2014 ◽  
Vol 34 (13) ◽  
pp. 1093-1096 ◽  
Author(s):  
Tomoaki Kameda ◽  
Michito Namekawa ◽  
Haruo Shimazaki ◽  
Daisuke Minakata ◽  
Tohru Matsuura ◽  
...  

Background Reversible cerebral vasoconstriction syndrome is characterized by thunderclap headache and reversible cerebral vasoconstriction on angiographic findings. It can be difficult to diagnose when initial angiography is normal. Case results A 30-year-old woman was admitted because of sudden-onset thunderclap headache and seizure on postpartum day 7. Brain MRI on fluid-attenuated inversion recovery (FLAIR) showed hyperintense vessel sign (HVS), which usually means slow flow due to severe proximal arterial stenosis. However, magnetic resonance angiography (MRA) indicated that proximal arteries was normal. After nicardipine treatment, her symptoms improved dramatically. Follow-up FLAIR on day 7 showed complete resolution of HVS, while a series of MRAs revealed reversible multifocal segmental vasoconstriction. Conclusions HVS on initial FLAIR is useful for an early diagnosis of reversible cerebral vasoconstriction syndrome. As the delayed vasoconstriction on MRA can be observed, reversible cerebral vasoconstriction syndrome may progress from distal small to proximal larger arteries.

Stroke ◽  
2020 ◽  
Vol 51 (2) ◽  
pp. 670-673 ◽  
Author(s):  
Rosalie Boitet ◽  
Solène de Gaalon ◽  
Claire Duflos ◽  
Grégory Marin ◽  
Jérôme Mawet ◽  
...  

Background and Purpose— We aimed to further investigate the long-term outcomes after reversible cerebral vasoconstriction syndrome (RCVS). Methods— A longitudinal follow-up study was conducted in 173 RCVS patients. Results— Of the 172 patients who completed a mean follow-up of 9.2±3.3 years, 10 had a recurrent RCVS that was benign in all. Independent predictors of relapse were having a history of migraine and having exercise as a trigger for thunderclap headache during initial RCVS. After new delivery, the rate of postpartum RCVS was 9%. Conclusions— Overall, long-term outcome after RCVS is excellent.


Author(s):  
Ji Y. Chong ◽  
Michael P. Lerario

Reversible cerebral vasoconstriction syndrome can cause thunderclap headache, subarachnoid hemorrhage, and stroke. The clinical presentation can be similar to aneurysmal subarachnoid hemorrhage and therefore requires rapid angiography. Angiographic findings of vasculopathy and resolution of clinical symptoms and imaging abnormalities support this diagnosis. This syndrome is most often incited following pregnancy or the ingestion of a vasoactive medication.


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.


2020 ◽  
Author(s):  
Kiyomitsu Fukaguchi ◽  
Tadahiro Goto ◽  
Hiroyuki Fukui ◽  
Ichiro Sekine ◽  
Hiroshi Yamagami

Abstract Background: In patients with thunderclap headache, reversible cerebral vasoconstriction syndrome (RCVS) should be considered as a differential diagnosis. However, RCVS diagnosis in the emergency department (ED) remains challenging. This study described the clinical features of RCVS, determined the factors related to RCVS diagnosis, and suggested treatment strategies for its management. Methods: We retrospectively identified eight patients diagnosed with RCVS and reviewed their medical records. From January 2010 to March 2019, eight patients with RCVS (ages 18-69 years, 5 females) were identified. Results: The median duration from the ED visit to RCVS diagnosis was 6 days (range, 1-11 days). Of the eight patients, seven were middle-aged, six had apparent triggers, six had subarachnoid haemorrhage (SAH), five had high systolic blood pressure, and none had any specific abnormality observed upon physical examination. At the ED visit, RCVS was diagnosed in only one patient with a history of RCVS. In other patients, SAH was diagnosed in two patients, and primary headache was diagnosed in four patients with negative computed tomography (CT) findings. Based on the follow-up imaging, seven of eight patients with convexal SAH were diagnosed as having RCVS (as the cause of SAH) using angiography (e.g., magnetic resonance angiography). Conclusions: RCVS with negative CT findings at the ED visit was likely to be misdiagnosed as primary headache. In patients with thunderclap headache and negative CT findings, physicians should consider RCVS as a differential diagnosis, inform patients of the risk of RCVS and the likelihood of a negative image evaluation early in the course of the disease, and carry out follow-up imaging within 2-weeks of the visit.


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.


Author(s):  
E. G. Klocheva ◽  
V. V. Goldobin

Data of 130 patients with thunderclap headache are presented. The data include clinical manifestation analysis and neuroimaging results (magnetic resonance angiography). Magnetic resonance angiography was performed 15 days after acute clinical manifestation and permitted to verify cerebral vasoconstriction, that led to treatment modification with clinical and neuroimaging signs of vasoconstriction reverse.


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.


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.


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.


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