scholarly journals Fulminant Reversible Cerebral Vasoconstriction Syndrome

2017 ◽  
Vol 8 (1) ◽  
pp. NP5-NP8 ◽  
Author(s):  
Kushak Suchdev ◽  
Gregory Norris ◽  
Imad Zak ◽  
Wazim Mohamed ◽  
Mohammed Ibrahim

Reversible cerebral vasoconstriction syndrome (RCVS) is increasingly being recognized as a diagnosis in patients presenting with thunderclap headaches. In the vast majority of the cases, the syndrome follows a benign course and patients recover completely without any significant therapeutic intervention. In the rarest of cases, RCVS follows a monophasic course with rapid worsening, severe neurological deficits, and poor outcomes. We present the case of a 25-year-old female who presented with headaches which were worsening over 1 week. She was admitted to the hospital and rapidly worsened to develop severe neurological deficits over the next day. Initial computed tomography scan showed areas of hemorrhage and multiple ischemic strokes. Computed tomography angiogram and a conventional cerebral angiogram both revealed multifocal vasoconstriction, highly suggestive of RCVS. Despite aggressive medical and surgical management, the patient continued to worsen and eventually died. Autopsy findings did not show evidence of vasculopathy or any other underlying disorder, further supporting the diagnosis of RCVS. The RCVS is usually a benign self-remitting condition which commonly affects young females and presents with an insidious onset of headaches. Rarely, it can have a fulminant course with devastating outcomes. This case illustrates an exceptionally uncommon clinical course of RCVS and the challenges in its treatment.

2019 ◽  
Vol 34 (6) ◽  
pp. 944-944
Author(s):  
E Schultz ◽  
S DeDios-Stern ◽  
G Ovsiew ◽  
N Pliskin

Abstract Objective Reversible cerebral vasoconstriction syndrome (RCVS), a relatively rare condition presenting as severe thunderclap headache with or without neurological deficits, is characterized by vasoconstriction of cerebral arteries which often resolves within three months and may be associated with stroke. Previous case reports examining neuropsychological profiles of RCVS patients demonstrated stroke-specific and/or diffuse global impairments that often exceed impairment expected post-acute phase of clinical stroke and possibly suggest residual deficits related to RCVS alone (Perdices & Herkes, 2018). Method The present case is a 36-year-old right-handed African-American male who sustained a left thalamic hemorrhage with extension into the ventricular system following marijuana use. Cerebral angiogram was suggestive of RCVS. The Montreal Cognitive Assessment (MoCA), Western Aphasia Battery (WAB), Repeatable Battery for the Assessment of Neuropsychological Status- Update (RBANS), and Peabody Picture Vocabulary Teat- 4th edition (PPVT-4) were administered at bedside. Results Neuropsychological testing was abnormal with severe global impairments on tasks requiring attention and processing speed, visuospatial abilities, immediate and delayed memory, and executive functioning. The patient’s expressive speech was characterized by fluent output with pronounced provoked and unprovoked phonemic and semantic paraphasias. Additionally, he evidenced confabulatory responses during memory testing and lost mental set during tasks requiring simultaneous attention. Conclusions Considering the patients clear sensorium at the time of the evaluation, these deficits extend beyond what would be expected by a single thalamic lesion and implicate more generalized cerebral dysfunction secondary to his RCVS. Consistent with previous case studies, this case demonstrates how RCVS may confound and contribute to neurocognitive deficits even after hemorrhagic stroke.


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.


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.


2021 ◽  
Vol 32 (2) ◽  
pp. 187-192
Author(s):  
Joseph Franklin Chenisz da Silva ◽  
Caio Cesar Demore ◽  
Felipe Ibiapina dos Reis ◽  
Gabriel Abrahão Stoliar ◽  
Matheus Kahakura Franco Pedro ◽  
...  

The reversible cerebral vasoconstriction syndrome, also known as Call-Fleming syndrome, was initially described in 1988, and is characterized by a clinical syndrome of headaches episodes, generally the “thunderclap” pattern, due to a deregulation of the vascular tonus, leading to segmentary cerebral vasoconstriction and secondary neurological deficits, including those by ischemic or hemorrhagic stroke. In this paper, we present two illustrative cases of this syndrome due to the use of sibutramine. To our knowledge, this situation hasn’t been described as related drug before.


2021 ◽  
Vol 14 (1) ◽  
pp. e233877 ◽  
Author(s):  
Adam Ross Schertz ◽  
Anand Karthik Sarma ◽  
Sudhir Datar ◽  
Peter John Miller

A 59-year-old woman was found unresponsive at home. Initial neurologic examination revealed aphasia and right-sided weakness. Laboratory results demonstrated a serum calcium level of 17.3 mg/dL (corrected serum calcium for albumin concentration was 16.8 mg/dL). Extensive workup for intrinsic aetiology of hypercalcemia was unrevealing. Further discussion with family members and investigation of the patient’s home for over-the-counter medications and herbal supplements revealed chronic ingestion of calcium carbonate tablets. CT angiogram of the brain revealed multifocal intracranial vascular segmental narrowing, which resolved on a follow-up cerebral angiogram done 2 days later. These findings were consistent with reversible cerebral vasoconstriction syndrome.Appropriate blood pressure control with parenteral agents, calcium channel blockade with nimodipine and supportive care therapies resulted in significant improvement in neurologic status. By discharge, patient had near-complete resolution of neurologic symptoms.


Stroke ◽  
2021 ◽  
Vol 52 (Suppl_1) ◽  
Author(s):  
Meghan Purohit ◽  
Monica Scarsella ◽  
Karlo Toljan ◽  
Naresh Mullaguri ◽  
Dolora R Wisco ◽  
...  

Introduction: Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) are diagnoses that have a clinical and radiographic overlap. One particular overlap includes the presence of hemorrhage, which we studied in each population to determine its effect on outcomes. Objective: To compare characteristics and outcomes of hemorrhagic PRES and hemorrhagic RCVS populations. Methods: We conducted a review of the electronic health record at a single tertiary center from January 2008 to December 2018. Inclusion criteria were 18 years or older with clinical and radiographic evidence of PRES or RCVS. Patient demographics, presenting symptoms, imaging findings, and outcomes were compared between PRES and RCVS groups. Poor outcome was defined as discharge to skilled nursing facility or death. Analysis was performed using Pearson’s Chi-Square test. Results: Among 281 PRES and 98 RCVS cases, intracranial hemorrhage was seen on imaging in 51 PRES cases (18%) and 34 RCVS cases (35%). Headache was present in all patients with hemorrhagic RCVS but only seen in 40% of hemorrhagic PRES. Use of antidepressants or drugs of abuse was more frequent with hemorrhagic RCVS (53%) as compared to hemorrhagic PRES (7%, p <0.05). Among hemorrhagic PRES, presence of intraparenchymal hemorrhage but not subarachnoid hemorrhage was associated with vasoconstriction on arterial imaging (p<0.05). Length of stay was longer for both hemorrhagic PRES and hemorrhagic RCVS (p<0.001) compared those without hemorrhage. Presence of focal neurological deficits, motor and sensory, was associated with poor outcomes in hemorrhagic RCVS patients (p<0.05). Discharge to a rehabilitation facility was associated with hemorrhagic PRES (p<0.05), no association in those without hemorrhage. There is no evidence that discharge location is significantly different between RCVS hemorrhage and non-hemorrhage groups. Conclusion: Hemorrhage in PRES and RCVS is associated with more clinical deficits at presentation and longer hospital stays. Underlying vasoconstriction is frequent and associated with hemorrhagic PRES.


Cephalalgia ◽  
2015 ◽  
Vol 36 (3) ◽  
pp. 289-293 ◽  
Author(s):  
Makoto Kobayashi

Background Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by recurrent thunderclap headaches with reversible cerebral vasoconstriction, and often precipitated by the postpartum state and vasoactive medications. We describe a case of a patient with RCVS induced by amezinium metilsulfate, a sympathomimetic drug, in whom magnetic resonance angiography (MRA) initially revealed diffusely dilated cerebral arteries. Case description A 34-year-old woman was prescribed amezinium metilsulfate for hypotension. Twelve days later, she suffered from abrupt severe headaches and was referred to our department. She had no neurological deficits; however, MRA revealed diffusely dilated anterior, middle, and posterior cerebral arteries with vasoconstriction. She was tentatively diagnosed with RCVS and successfully treated with verapamil for headache. Nevertheless, follow-up MRAs disclosed widespread segmental vasoconstriction that resolved in two months. Discussion Diffuse cerebrovascular dilation has not been addressed but may be associated with RCVS pathophysiology. In addition, physicians should bear in mind that amezinium metilsulfate can potentially induce RCVS.


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.


Author(s):  
Philip Gerretsen ◽  
Ralph Z. Kern

Background:Reversible Cerebral Vasoconstriction Syndrome (RCVS) may present as thunderclap headache (TCH), accompanied by reversible cerebral vasospasm and focal neurological deficits, often without a clear precipitant. RCVS may be mistaken for Primary Angiitis of the Central Nervous System (PACNS) due to the presence of similar angiographic features of segmental narrowing of cerebral arteries. We discuss the clinical features of a young female migraine patient who developed TCH and was found to have RCVS following initial treatment with corticosteroids for PACNS, in the context of a systematic review of the available medical literature.Methods:A Medline™ search was performed to identify all case reports since 1966 describing RCVS and PACNS that provide sufficient clinical detail to permit diagnostic classification according to published criteria. RCVS included case studies in which there was angiographic or transcranial Doppler ultrasound evidence of near-to-complete resolution of cerebral vasoconstriction in the absence of a well-recognized secondary cause. PACNS included reports of histologically confirmed PACNS either through biopsy or necropsy.Results:Reversible Cerebral Vasoconstriction Syndrome occurs primarily in females and is characterized by sudden, severe headache at onset, normal CSF analysis, vasoconstriction involving the Circle of Willis and its immediate branches, and angiographic or TCD ultrasound evidence of near-to-complete vasospastic resolution within 1-4 weeks. It occurs typically in the context of vasoconstrictive drug use, the peripartum period, bathing, and physical exertion.Conclusion:Initial and follow-up (within 4 weeks) non-invasive angiographic studies are indicated in patients who present with TCH or who have clinical presentations that could be consistent with RCVS or PACNS in the absence of a well-recognized secondary cause, such as subarachnoid haemorrhage. Early reversibility of cerebral vasospasm is the key neuroradiological feature that supports the clinical diagnosis of RCVS.


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