scholarly journals Postpartum Reversible Cerebral Vasoconstriction Syndrome Presenting with Radiological Plethora

Author(s):  
Manisha Sharma ◽  
Bindu Menon ◽  
Gayatri Manam

AbstractReversible cerebral vasoconstriction syndrome (RCVS) is a clinicoradiological syndrome characterized by segmental vasoconstriction of cerebral arteries with resolution in most patients within 1 to 3 months. It has been known to be a benign uniphasic reversible illness but cases of fulminant RCVS with mortality have also been reported. The symptoms can be varied with presentations like transient ischemic attack (TIA), ischemic stroke (IS), intracerebral hemorrhage (ICH), subarachnoid hemorrhage (SAH), and posterior reversible encephalopathy syndrome (PRES). We report a postpartum female with RCVS with radiological features of ICH, SAH, atypical PRES, and atypical clinical presentation as well. RCVS can be frequently missed unless there is a high index of suspicion. Hence clinicians should be aware of the typical and the atypical presentation of RCVS along with the radiological findings with the potential complications. Early suspicion and detection in the emergency help in recovery and reducing morbidity.

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.


2018 ◽  
Vol 89 (6) ◽  
pp. A37.2-A37 ◽  
Author(s):  
Michael J Devlin ◽  
Archana Dwivedee ◽  
Richard P White

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.


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.


Stroke ◽  
2020 ◽  
Vol 51 (Suppl_1) ◽  
Author(s):  
Meghan Purohit ◽  
Monica Scarsella ◽  
Naresh Mullaguri ◽  
Dinesh Jillella ◽  
Dolora Wisco ◽  
...  

Introduction: Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome with typical neuroimaging findings of vasogenic cerebral edema in posterior brain regions. Reversible cerebral vasoconstriction syndrome (RCVS) is diagnosed when there is reversible cerebral vessel narrowing and often with thunderclap headache. The two diseases have overlap in clinical and radiographic features. Objective: To compare clinical presentation, vascular abnormalities and imaging findings among PRES and RCVS. Methods: We searched patients in electronic health record at a single tertiary center from January 2008 to December 2018. Inclusion criteria was 18 years or older with clinical and radiographic evidence of PRES or RCVS. PRES was diagnosed by clinical presentation and presence of vasogenic edema on radiographic imaging. RCVS was determined by clinical presentation and transient arterial stenosis. Patient demographics, presenting symptoms, comorbid conditions, and imaging findings were compared between PRES and RCVS populations. Analysis was performed using Fisher’s exact test Results: Seventy-four patients with PRES and 24 patients with RCVS met inclusion criteria. The median age and presenting blood pressure did not differ between PRES and RCVS groups. PRES population consisted of fewer females (70%) compared to RCVS (92%, p=0.05). There was no significant difference in frequency of DWI lesions in PRES (37%) and RCVS (24%, p=0.308). Among patients who had arterial imaging, arterial stenosis was seen in 28% (13/46) of PRES. The occurrence of > 50% FLAIR reversibility was more common in PRES (67%) and seen less frequently in RCVS (27%, p=0.008). Conclusion: There is radiographic overlap between PRES and RCVS. About a quarter of PRES have arterial stenosis, while about a quarter of the RCVS group had FLAIR reversibility.


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.


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.


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.


2020 ◽  
Vol 83 (5) ◽  
pp. 453-457
Author(s):  
Montserrat González Delgado ◽  
Julien Bogousslavsky

Cerebral vasoconstriction is a normal physiological response under determined conditions to preserve a normal cerebral blood flow. However, there are several syndromes, with impaired cerebral autoregulation and cerebral vasoconstriction, not related with infection or inflammation, which share the same radiological and clinical presentation. We review here the cerebral hyperperfusion syndrome and related conditions such as hypertensive encephalopathy, posterior reversible encephalopathy syndrome, and reversible cerebral vasoconstriction syndrome. These syndromes might share the same pathophysiological mechanism with endothelial damage, cerebral vasoconstriction, blood-brain barrier disturbance, cerebral edema, and, occasionally, intracerebral hemorrhage, with fatal cases described in all. Despite knowledge of these syndromes, they still remain unknown to us. Why these entities present in some patients and not in others goes further than the actual understanding of these diseases. We have to consider that a genetic susceptibility and molecular disturbances may be involved. Thus, more studies are needed in order to better characterize such syndromes.


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