Reversible Cerebral Vasoconstriction Syndrome Research Articles

Reversible cerebral vasoconstriction syndrome with concomitant COVID-19 infection

Reversible cerebral vasoconstriction syndrome with concomitant COVID-19 infection

Cerebrovascular Diseases and Autonomic Failure

Cerebral vessels are innervated by sympathetic and parasympathetic nerves, as well as the trigeminal nerve. Under physiological conditions, neural regulation contributes to coupling of cerebral blood flow (CBF) and metabolism and autoregulation of CBF against systemic blood pressure. It also plays a key role in vasodilatation distal to narrowed vascular lesions associated with atherosclerosis, hyperperfusion syndrome that occurs after vascular reconstruction in patients with chronic ischemic diseases, reversible cerebral vasoconstriction syndrome, and migraine. Moreover, cerebrovascular disease can cause autonomic dysfunction; frontal lobe and brain stem lesions are known to result in urinary incontinence, mydriasis, and Horner's syndrome.

Editors' Note: Blood-Brain Barrier Permeability in Patients With Reversible Cerebral Vasoconstriction Syndrome Assessed With Dynamic Contrast-Enhanced MRI

Using dynamic contrast-enhanced MRI, Dr. Wu and colleagues evaluated the blood-brain barrier function in a prospective cohort of patients with reversible cerebral vasoconstriction syndrome (RCVS). The investigators found marked elevations in Ktrans—a validated index of microscopic blood-brain barrier disruption—in the acute phase of RCVS, which declined with time. These findings paralleled the course of vasoconstriction and development of white matter hyperintensities. Dr. Gupta comments on one important limitation of these findings in that they do not explain the relationship between the predisposing risk factors and clinical manifestations with the pathogenesis of RCVS. For instance, there is a time gap between the thunderclap headache of RCVS and the subsequent MRI findings. The investigators agree that the relationships observed between blood-brain barrier dysfunction and clinical disease are more likely to be those of association, although causation cannot be wholly excluded. Using dynamic contrast-enhanced MRI, Dr. Wu and colleagues evaluated the blood-brain barrier function in a prospective cohort of patients with reversible cerebral vasoconstriction syndrome (RCVS). The investigators found marked elevations in Ktrans—a validated index of microscopic blood-brain barrier disruption—in the acute phase of RCVS, which declined with time. These findings paralleled the course of vasoconstriction and development of white matter hyperintensities. Dr. Gupta comments on one important limitation of these findings in that they do not explain the relationship between the predisposing risk factors and clinical manifestations with the pathogenesis of RCVS. For instance, there is a time gap between the thunderclap headache of RCVS and the subsequent MRI findings. The investigators agree that the relationships observed between blood-brain barrier dysfunction and clinical disease are more likely to be those of association, although causation cannot be wholly excluded.

Intra-arterial application of nimodipine in reversible cerebral vasoconstriction syndrome: a neuroradiological method to help differentiate from primary central nervous system vasculitis

BackgroundReversible cerebral vasoconstriction syndrome (RCVS) is characterized by a prolonged, but self-limiting segmental cerebral vasoconstriction. Neurological outcomes vary, but can be severe. The clinical hallmark of RCVS is thunderclap headache, which might come along with further neurological symptoms. Distinguishing RCVS from other entities, such as primary angiitis of the central nervous system (PACNS), is of utmost importance for appropriate therapy. The angiographic response to intra-arterial nimodipine application has been suggested as an additional diagnostic criterion for RCVS but confirmatory studies are limited. We aimed to evaluate the angiographic nimodipine test.MethodsWe reviewed retrospectively the clinical and imaging data of 13 RCVS patients, who were admitted to a single German neurological department between January 2013 and December 2020.ResultsOut of 13 patients diagnosed with RCVS, 4 patients underwent an angiographic nimodipine test. In all 4 patients cerebral vasoconstriction completely resolved during nimodipine application. Among the four patients with a positive test, there was one individual, in whom a response was detected after a delay of 60 min. In all patients, we found a complete resolution of cerebral vasoconstriction within 12 weeks.ConclusionOur findings support the usefulness of the application of nimodipine in diagnosing RCVS. Prolonged angiographic observation of the vascular response after nimodipine injection is important.

Abstract TMP83: Comparison Of Clinical Features And Vessel Involvement In Isolated Reversible Cerebral Vasoconstriction Syndrome And Associated Posterior Reversible Encephalopathy Syndrome

Introduction: Reversible cerebral vasoconstriction syndrome (RCVS) may co-occur with brain MRI T2/FLAIR sequence changes, and posterior reversible encephalopathy syndrome (PRES) may be associated with cerebral vasoconstriction. Both entities with overlapping features may be considered as a single group. We compared the isolated RCVS group to the overlapping group to investigate clinical and radiological features, with a focus on vessel involvement. Methods: We performed a retrospective study of adult patients admitted to a tertiary medical center from February 2008 until February 2018 and who were diagnosed with PRES or RCVS. Overlap cases consisting of PRES with vasoconstriction and RCVS with reversible MRI T2/FLAIR changes were compared to isolated RCVS cases. Clinical and radiological features of both groups were compared with each other. Involved vessel segments were classified as internal carotid artery, M1 and M2 middle cerebral artery, A1 and A2 anterior cerebral artery, P1 and P2 posterior cerebral artery, vertebral artery, and basilar artery. Severity was also graded (1-49% or >49%). Appropriate statistical tests were used to compare the variables and significant findings reported. Results: There were 86 cases with isolated RCVS and 44 cases of overlap. Seizures (19, 43%) and encephalopathy (13, 30%) were common in the overlap group, whereas headache was common in the isolated RCVS group (79, 92%). Immunosuppressant use was more often present in the overlap group (9, 20%), but there were no differences in antidepressant, cocaine, or marijuana use. Intracerebral hemorrhage was more common in the overlap group (12/44, p=0.003), but there were similar numbers of ischemic strokes and subarachnoid hemorrhages. There were no differences in the number or location of segments involved or stenosis severity between the groups. Home discharge was more frequent among pure RCVS (73, 85%) than among overlap patients (27, 61%, p=0.004). Conclusion: Clinical presentations and short-term prognosis differed among isolated RCVS and group with PRES-RCVS overlap characteristics. There was no difference in vascular involvement between the groups.

Abstract WP157: Outcomes Of Reversible Cerebral Vasoconstriction Syndrome: Analysis Of The National Inpatient Sample Databases, 2016-2018

Introduction: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by severe headaches accompanied by reversible segmental vasoconstriction of cerebral arteries. Ischemic and hemorrhagic stroke are considered major complications of RCVS. We aimed to assess outcomes of RCVS patients with and without stroke, and to compare presentation with ischemic or hemorrhagic stroke. Methods: Data was extracted from the National Inpatient Sample (NIS) spanning 2016 to 2018. All adult patients diagnosed with RCVS were identified using ICD-10 codes after excluding patients with a concurrent diagnosis of primary angiitis. Measured outcomes included adverse events during hospitalization and discharge disposition. Standard bivariate statistical tests were applied to compare groups. Results: Of 3170 patients (mean age 48.0±15.7 years; 73.3% female) who were admitted with the diagnosis of RCVS, 1565 patients (49.4%) had no stroke and 1605 patients (50.6%) presented with stroke. In patients who presented with stroke, 375 patients (26.1%) had only ischemic stroke and 1060 (73.9%) presented with only hemorrhagic stroke. Rates of routine discharge were higher in patients with no stroke (75.4% vs 57.9%, p<0.001) versus those presenting with stroke (Table). In patients who presented with stroke, rates of percutaneous gastrostomy (PEG) placement were higher in ischemic stroke group compared to patients with hemorrhagic stroke (5.3% vs 2.4%, p=0.004), whereas rates of ventilation/intubation were higher in patients with hemorrhagic stroke (10.8% vs 4.0%, p=0.001). Routine discharge was more frequent in RCVS patients presenting with hemorrhagic rather than ischemic stroke (66.0% vs 48.0%, p<0.001). Conclusions: More than half of patients admitted with a diagnosis of RCVS presented with stroke. RCVS patients presenting with ischemic stroke have higher rates of PEG placement and lower rates of routine discharge than those presenting with hemorrhagic stroke.

Обратимый церебральный вазоконстрикторный синдром: клиника, диагностика и лечение

Представлен обзор литературы, посвященный вопросам клиники, диагностики и лечения обратимого церебрального вазоконстрикторного синдрома (ОЦВС). Описаны особенности клинической картины, диагностические критерии ОЦВС, лечебная тактика и последствия ОЦВС. Приведено описание собственного наблюдения за пациентом с суб­арахноидальным кровоизлиянием, ассоциированным с ОЦВС.

Reversible Cerebral Vasoconstriction Syndrome and Multisystem Inflammatory Syndrome in Children With COVID-19

BackgroundMultisystem inflammatory syndrome in children (MIS-C) involves multiple organs and shows increased inflammatory markers. Since the onset of the coronavirus disease 2019 (COVID-19) pandemic, several studies have reported the association between severe COVID-19 and MIS-C. Reversible cerebral vasoconstriction syndrome (RCVS) presents with thunderclap headaches and multifocal reversible vasoconstriction on imaging. RCVS is very rare in children. This article reports two cases of pediatric COVID-19 with severe MIS-C and clinical and imaging features indicative of RCVS. MethodsClinical, laboratory, and imaging data of the patients were reviewed. The diagnosis of RCVS was confirmed based on clinical symptomatology and brain magnetic resonance imaging findings. ResultsTwo pediatric patients with clinical findings compatible with severe MIS-C and hemodynamic compromise presented to the hospital. During their hospitalization course, they developed thunderclap headaches and neurological deficits. Both were receiving vasoactive agents, intravenous immunoglobulin, and immunosuppressants. Imaging studies showed marked multifocal cerebral vasoconstriction in both cases and infarcts in one. The course and management of the patients will be presented. After controlling inflammation and elimination of triggers, both patients were ultimately symptom free upon discharge. Cerebral vasoconstriction had completely resolved on follow-up imaging. ConclusionsAlthough a variety of symptoms including headaches may be seen in pediatric COVID-19 patients with MIS-C, RCVS should be considered as a differential diagnosis in cases of thunderclap headache accompanied by neurological signs in these patients. Imaging findings and follow-up are also key in establishing the diagnosis.

Subarachnoid hemorrhage: management considerations for COVID-19.

Subarachnoid hemorrhage (SAH) has deleterious outcomes for patients, and during the hospital stay, patients are susceptible to vasospasm and delayed cerebral ischemia. Coronavirus disease 2019 (COVID-19) has been shown to worsen hypertension through angiotensin-converting enzyme 2 (ACE2) activity, therefore, predisposing to aneurysm rupture. The classic renin-angiotensin pathway activation also predisposes to vasospasm and subsequent delayed cerebral ischemia. Matrix metalloproteinase 9 upregulation can lead to an inflammatory surge, which worsens outcomes for patients. SAH patients with COVID-19 are more susceptible to ventilator-associated pneumonia, reversible cerebral vasoconstriction syndrome, and respiratory distress. Emerging treatments are warranted to target key components of the anti-inflammatory cascade. The aim of this review is to explore how the COVID-19 virus and the intensive care unit (ICU) treatment of severe COVID can contribute to SAH.

Spontaneous postpartum intracranial haemorrhage: A case report

Pregnancy-associated strokes are rare but can have detrimental effects on both mother and baby. A young female patient, 6 days postpartum, suffered a spontaneous intraparenchymal haemorrhage (IPH) and subarachnoid haemorrhage (SAH). The patient exhibited aphasia, right facial droop and right hemiparesis. Serial imaging showed no vascular malformation or other cause of her haemorrhages. Our pregnant patient presented with concurrent IPH and SAH. Given the timing of her stroke and the absence of underlying vascular lesion, it is possible her stroke was a case of IPH and SAH due to reversible cerebral vasoconstriction syndrome (RCVS). More specifically, it may reflect postpartum angiopathy, a subtype of RCVS presenting around 5 days postpartum. Alternatively, this could simply be a subarachnoid extension of a primary IPH.

A case of reversible cerebral vasoconstriction syndrome (RCVS) with difficulty in differential diagnosis from bow hunter’s syndrome

A case of reversible cerebral vasoconstriction syndrome (RCVS) with difficulty in differential diagnosis from bow hunter’s syndrome

A case of reversible cerebral vasoconstriction syndrome with repeated hemorrhagic infarction

A case of reversible cerebral vasoconstriction syndrome with repeated hemorrhagic infarction

Guillain-Barré syndrome complicated by Takotsubo cardiomyopathy and cerebral infarction due to reversible cerebral vasoconstriction syndrome: A case report

Guillain-Barré syndrome complicated by Takotsubo cardiomyopathy and cerebral infarction due to reversible cerebral vasoconstriction syndrome: A case report

Transient Hypnic Headache-like Phenomenon in Guillain-Barre Syndrome: Lessons from a First Case Report

Headache is an uncommon feature of Guillain-Barre syndrome (GBS), which is more frequently associated with severe limb and/or back pain. However, when it does it occur, headache in GBS may emanate from a range of causes, including raised intracranial pressure, reversible cerebral vasoconstriction syndrome, posterior reversible encephalopathy syndrome, or due to complications of intravenous immunoglobulin (IVIg) therapy, such as aseptic meningitis and cerebral venous sinus thrombosis. Hypnic headache (HH)-like phenomenon has not been thus far described in association with GBS. We report herein a patient with GBS who developed HH-like phenomenon during the recovery phase; she developed a cyclical nocturnal headache lasting around four hours and demonstrated exquisite response to caffeine. We postulate potential mechanisms for the same. Keywords: Hypnic headache, reversible cerebral vasoconstriction syndrome, posterior reversible encephalopathy syndrome, IVIg

Diagnostic Value of Thunderclap Headache and Convexal Subarachnoid Hemorrhage for Reversible Cerebral Vasoconstriction Syndrome: A Case Report

Reversible cerebral vasoconstriction syndrome (RCVS) is an underdiagnosed cause of convexal subarachnoid hemorrhage, characterized by thunderclap headache associated with focal and segmental intracranial vasoconstriction. It can appear complications such as intracerebral hemorrhage, seizures, posterior reversible leukoencephalopathy, or ischemic stroke. Our objective is to present the case of a 51-year-old woman with an RCVS diagnosis, who had a normal digital subtraction angiography at the illness onset. We highlight the high diagnostic value of thunderclap headache and convexal subarachnoid hemorrhage. We also highlight the importance of repeating the angiographic studies in the second week when there is strong diagnostic suspicion.

Reversible Cerebral vasoconstriction syndrome associated with the use of sibutramine

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.

Large artery intracranial stenosis in young adults with ischaemic stroke

Large artery intracranial stenosis (ICS) is a common finding in stroke patients, but is much less prevalent in Western countries than in Asia and in young adults than in the elderly. We investigated the prevalence and causes of ICS among French young adults with ischaemic stroke. Clinical and radiological data of patients aged 18–54 years treated consecutively for acute ischaemic stroke in the anterior circulation at a tertiary stroke centre were analysed retrospectively. Patients with>50% ICS were identified. ICS was evaluated using TOF-MRA, vessel wall-MRI, digital subtraction angiography and CT-angiography. A total of 316 patients were included. ICS was diagnosed in 29 patients, resulting in a prevalence of 9.2% (95% CI, 6.2 to 13.3). The leading cause of ICS was atherosclerosis (n=13), ahead of moyamoya disease (n=4), dissection (n=2), vasculitis (n=2), and reversible cerebral vasoconstriction syndrome (n=1). The cause of ICAS could not be determined in 7 patients. ICS was found in nearly one in 10 ischaemic strokes among French young adults. Atherosclerosis was the leading cause of ICS. The cause of ICS could not be determined in almost a quarter of the patients.

Reversible cerebral vasoconstriction syndrome with cardiac involvement during treatment for iron deficiency anemia: caser report

BackgroundThe diagnosis and therapy of reversible cerebral vasoconstriction syndrome (RCVS) tends to focus on neurological symptoms, but less attention has been paid the occurrence of extracerebral lesion such as the myocardium.Case presentationA 40-year-old woman taking iron supplements for iron deficiency anemia due to menorrhagia had suffered from a thunderclap headache and seizure. Brain magnetic resonance imaging revealed high-intensity lesions bilaterally in the cerebellar and cerebral hemispheres. Her symptoms once subsided with steroids and anticonvulsant therapy; however, she experienced a severe headache again while bathing and was transferred to our hospital. Based on the clinical course and imaging data, she was diagnosed as having RCVS triggered by a rapid improvement of anemia. At the same time, she had cardiac involvement revealed by electro and echocardiographs despite without chest symptoms. After the administration of a calcium channel blocker and nitrite, her cerebral and cardiac involvements were rapidly improved.ConclusionsThe case presented RCVS with transient myocardial damage. With RCVS, we should always pay attention to the complication of extracerebral lesions.

Reversible cerebral vasoconstriction syndrome in a patient with COVID-19

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.

Reversible cerebral vasoconstriction syndrome: A narrative review for emergency clinicians.

Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a rare cause of severe headache that can mimic other causes of sudden, severe headache and result in frequent emergency department (ED) visits. This narrative review provides an evidence-based update concerning the presentation, evaluation, and management of RCVS for the emergency clinician. RCVS can present as recurrent, severe headaches that may be maximal in onset, known as a thunderclap headache. Distinguishing from other causes of thunderclap headache such as aneurysmal subarachnoid hemorrhage, cerebral venous thrombosis, and posterior reversible encephalopathy syndrome is challenging. Risk factors for RCVS include use of vasoactive substances, exertion, coughing, showering, sexual activity, and cervical artery dissection. Diagnosis relies on clinical features and imaging. Cerebral catheter digital subtraction angiography (DSA) is considered the gold standard imaging modality; however, computed tomography angiography or magnetic resonance angiography are reliable non-invasive diagnostic modalities. Treatment focuses on avoiding or removing the offending agent, administration of calcium channel blockers such as nimodipine, and reversing anticoagulation if bleeding is present. Although most cases have a benign course and resolve within 3months, focal subarachnoid hemorrhage, intracerebral hemorrhage, permanent neurologic disability, or death can occur in a minority of cases. Diagnosis and appropriate management of RCVS can be aided by understanding key aspects of the history and examination. The emergency clinician can then obtain indicated imaging, confirming the diagnosis and allowing for appropriate management.