Reversible Cerebral Vasoconstriction Syndrome Research Articles

Reversible cerebral vasoconstriction syndrome in the postpartum period: A case report and review of the literature.

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare phenomenon that can present in the postpartum period. We show the experience of a 35-year-old patient who presented with headache after an uncomplicated pregnancy and vaginal delivery. She was initially diagnosed with pre-eclampsia, and subsequently with RCVS following discovery of multifocal vascular narrowing on magnetic resonance arteriography (MRA). Verapamil was initiated, and at 1 month there was improvement intracranially, but cervical vertebral arterial narrowing, likely dissection, was discovered. Verapamil was continued and aspirin was initiated. Follow-up imaging 5 months postpartum demonstrated near-complete resolution of previously noted abnormalities, which remained stable at reimaging at 10 months postpartum. In conclusion, the symptoms of RCVS can mimic or coexist with pre-eclampsia. Early intracranial imaging such as MRA can permit timely diagnosis and facilitate appropriate management and follow-up.

Reversible Cerebral Vasoconstriction Syndrome Treated by Continuous Intravenous Milrinone

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by severe headaches, with or without other acute neurological symptoms, and diffuse segmental constriction of cerebral arteries that resolves spontaneously within 3 months.[1] It can occur spontaneously, especially in middle-aged women[1]; at least half the cases occur after exposure to vasoactive drugs or post-partum. This syndrome is often associated with a favorable outcome in 95% of cases.[1] However, the prognosis of RCVS is especially determined by stroke occurrence. Its management is inspired by what is applied in aneurysmal subarachnoid hemorrhage (aSAH), whose main complication is vasospasm and delayed cerebral ischemia (DCI). Although the physiopathology of RCVS is unclear, it appears to be distinct from aSAH. Indeed, the vasoconstriction in RCVS may be dependent on the activity and sensitivity of vascular receptors, which can lead to a more systemic effect on all brain vessels, as opposed to being dependent on the vasospasm induced by local clot lysis, as has been suggested in aSAH.[2] As cerebral arteries are densely innervated, the release of norepinephrine or neuropeptide Y from the sympathetic nerve endings may cause vasoconstrictions.[3] Angiographically, SAH-induced vasospasm is more commonly long-segmental and mainly around the bleeding focus, compared with the multiple, short-segmental, and diffuse changes seen in RCVS.[4] However, by analogy, RCVS is treated by nimodipine, and in some rare cases, the use of milrinone might be considered.[3]

Peripartum Cerebral Angiopathy

AbstractPeripartum cerebral angiopathy (PCA) is a rare cerebrovascular disorder of unclear etiology that typically occurs in the first postpartum week. The aim of this case series is to present seven cases of PCA and the associated outcomes. Two of the cases were typical of a reversible cerebral vasoconstriction syndrome (RCVS) and had no long-term sequelae. Five of the cases involved intraparenchymal hemorrhage in the absence of an underlying vascular lesion, with three of the five cases having associated cerebral vasoconstriction. None of the patients had a documented history of preeclampsia or hypertension during pregnancy. It has been suggested that there is pathophysiological overlap between PCA and eclampsia and RCVS. There is limited evidence in the literature in this regard and, in most cases, treatment has been supportive only. As a condition, PCA may be under-recognized in the neurocritical care setting. Increased awareness of this condition, its manifestations, and options for management may optimize outcomes.

Brain Infarction in the Artery of Percheron Supply Area due to Reversible Cerebral Vasoconstriction Syndrome

An ischemic stroke caused by the occlusion of the artery of Percheron encompasses both posterior thalami, and the resulting clinical presentation can mask the clinical presentation of a stroke. We present the case report of a 62-year-old female patient who was admitted to our emergency department in a soporous state after initial headache and dizziness. A neurological exam found left-sided spasticity and a flexor response to pain. Systemic thrombolysis was administered after an urgent computerized tomography (CT) of the brain with angiography. The interventional radiologist proposed a digital subtraction angiography which found a vasospasm in the right medial cerebral artery from the M2 seg- ment reaching distally, and a balloon dilation was done. Following the endovascular procedure, a CT scan showed ischemia in both posterior thalami. Additionally, the patient’s neurological impairment improved at that moment showing Parinaud syndrome, drowsiness, and left-sided palsy. The patient experienced a possible reversible cerebral vasoconstriction syndrome, the cause of which is still unknown, which resulted in an ischemic stroke. The typical clinical presentation of that syndrome was lacking, probably due to the presence of the artery of Percheron ipsilateral to the vasospasm. Our goal in presenting this case study is to draw attention to the artery of Percheron syndrome as a potential clinical sign of an acute ischemic stroke that can easily lead us to be misled.

Eclampsia with hypothyroidism complicated with posterior reversible encephalopathy syndrome–a case report

BackgroundPosterior reversible encephalopathy syndrome (PRES) is a rare neurological disorder with complex physiopathological mechanisms that have not been fully understood. Early identification is of great prognostic significance, of which the symptoms and radiological abnormalities can be completely reversed. If the diagnosis and treatment are delayed, ischemia and massive infarction may be developed in some patients. Posterior reversible encephalopathy syndrome (PRES) has been reported mainly in association with postpartum eclampsia, which have been rarely reported, while the association with hypothyroidism has not been reported at home or abroad.Case presentationHere we report on a pregnant 29-year-old with multipara and a chief complication of hypothyroidism. She presented in the emergency department with frequent attacks of severe headache symptoms resulting from reversible cerebral vasoconstriction syndrome (RCVS), accompanied with prenatal eclampsia. PRES was determined by radiological examination.ConclusionTo the best of our knowledge, this is the first case of PRES complicated by hypothyroidism and prepartum eclampsia.Clinicians should be alert for the co-occurence of eclampsia, PRES, and RCVS when patients have convulsions after a typical throbbing headache. Moreover, regular monitoring of thyroid function during pregnancy should also occupy certain special attention.

Reversible Cerebral Vasoconstriction Syndrome with Intracerebral Hemorrhage in a Thalassemic Child—An Extremely Rare Complication

AbstractA 6-year-old known thalassemic boy presented with a posttransfusional thunderclap headache. A computed tomography scan showed left occipital lobar bleed and magnetic resonance angiography showed diffuse cerebral vasoconstriction, which resolved after 3 months, suggesting reversible cerebral vasoconstriction syndrome. He was treated with oral nimodipine for 3 months and had an excellent recovery without sequelae. To the best of the authors' knowledge, the index case is the first reported case of reversible cerebral vasoconstriction syndrome in a thalassemic child.

Abstract TMP60: Misdiagnosis Of Posterior Reversible Encephalopathy Syndrome And Reversible Cerebral Vasoconstriction Syndrome In The Emergency Department

Introduction: Syndromes of cerebrovascular dysregulation such as posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) can be challenging to diagnose given their rarity and the need for advanced neuroimaging to diagnose them. We sought to measure the rate of potential PRES/RCVS misdiagnosis in the ED and identify features associated with misdiagnosis. Methods: We conducted a retrospective cohort study of patients with PRES/RCVS using deidentified administrative claims data from all nonfederal EDs and hospitals across 11 states from 2016-2018. To identify patients with PRES/RCVS, we used the previously validated ICD-10-CM codes I67.841 and I67.83. We defined patients with a probable misdiagnosis of PRES/RCVS as those with an ED visit resulting in discharge to home that occurred within the 14 days prior to their PRES/RCVS hospitalization. Only preceding ED visits where a non-specific neurological condition (e.g., headache, dizziness, numbness) was diagnosed were considered instances of probable ED misdiagnosis. Standard tests of comparison between patients with versus without probable misdiagnosis were used to identify patient-level and ED facility-level features associated with misdiagnosis. Results: We identified 4,633 patients hospitalized for PRES/RCVS; the majority (4,169; 90.0%) had PRES. A total of 210 patients (4.5%, 95% CI: 3.95-5.17) had at least one preceding ED visit with a probable misdiagnosis; these patients were younger (mean age 47.7 vs. 53.8 years; P<0.001) and more often female (80.4% vs. 69.7%: P<0.001). Misdiagnosed patients generally had fewer vascular risk factors including hypertension (46.3% vs. 84.9%; P<0.001) and more often had a history of headache (81.1% vs. 22.6%; P<0.001) and psychiatric disease (48.6% vs. 34.9%; P<0.001) as compared to patients without an ED misdiagnosis. Facility factors inversely associated with probable misdiagnosis included an ACGME-approved residency (63.4% vs. 75.3%; P<0.001) and on-site neurological services (74.9% vs. 84.7%; P<0.001). Conclusion: Probable ED misdiagnosis occurred in nearly 1 of 20 cases of PRES/RCVS in a large, multistate cohort.

Diagnostic yield of cerebral angiography for intracranial hemorrhage in young patients: A single-center retrospective analysis.

Intracranial hemorrhage (ICH) secondary to hypertension (HTN) classically occurs in the basal ganglia, cerebellum, or pons. Vascular lesions such as aneurysms or arteriovenous malformations (AVMs) are more common in younger patients. We investigated the utility of diagnostic subtraction angiography (DSA) in young hypertensive patients with non-lobar ICH. A retrospective review (2013-2022) identified young (18-60 years) patients who underwent DSA for ICH. HTN history, ICH location, presence/absence of subarachnoid hemorrhage (SAH), and computed tomography angiography (CTA) findings were collected. The main outcome was DSA-positivity, defined as presence of an AVM, aneurysm, Moyamoya disease, reversible cerebral vasoconstriction syndrome, or dural arteriovenous fistula on DSA. Two hundred sixty patients were included, and the DSA-positivity rate was 19%.DSA-positivity was lower in hypertensive patients with ICHs in the cerebellum, pons, or basal ganglia compared to the rest of the patient sample (9% vs 26%, p = 0.0002, Fisher's exact test). We developed the ICH-Angio score (0-5 points) based on CTA findings, ICH location, HTN history, and presence of SAH to predict risk of underlying vascular lesions. DSA-positivity was lower in those with a score of 0 (0/62; 0%) compared to a score of 1 (5/52; 10%), 2 (17/48; 35%), 3 (10/20; 50%), 4 (5/6; 83%), or 5 (3/3; 100%). The ICH-Angio score was able to non-invasively rule out an underlying vascular etiology for ICH in up to one-third of patients. HTN, ICH location, CTA findings, and associated SAH can identify patients at low risk for harboring underlying vascular lesions.

Reversible cerebral vasoconstriction syndrome: Considerable differential diagnosis

Background: Headache is the most common symptom in the neurology department of the hospital. This makes it a very important topic to understand more about what causes headaches. There are many causes of headaches and based on the cause, there could be different locations for headaches. One of the causes of the headache could be reversible vasoconstriction in the brain. This type of headache overlaps with a lot of differential diagnoses. Hence, it is important to bring light to this differential diagnosis to improvise the treatment regimen for the patient. Objectives: The objectives of the study were to study reversible cerebral vasoconstriction syndrome (RCVS) and all the differential diagnosis of headache that overlaps with RCVS. Methods: We searched the recent studies done on RCVS along with meta-analysis, controlled clinical trials, and cohort studies. We made the differential tables with symptoms, diagnoses, and treatments to bring a better understanding of the overlapping symptoms of different diseases and RCVS. Result: Understanding the symptoms, diagnosis of RCVS, and other overlapping symptoms of diseases, is helpful to avoid the extra expenditure of the patient. It would also help in leading treatment in the right direction without putting the patient on the experimental drugs.

Cortical Superficial Siderosis and Transient Focal Neurological Episode Preceding Lobar Hemorrhage in Cerebral Amyloid Angiopathy.

Cortical Superficial Siderosis and Transient Focal Neurological Episode Preceding Lobar Hemorrhage in Cerebral Amyloid Angiopathy.

Reversible Cerebral Vasoconstriction Syndrome along with Posterior Reversible Encephalopathy Syndrome in a Postnatal Eclamptic Patient: A Case Report

Reversible Cerebral Vasoconstriction Syndrome (RCVS) and Posterior Reversible Encephalopathy Syndrome (PRES) are rare neurological disorders. Although rare, the diagnosis of these conditions is now possible due to increased clinical awareness and the availability of newer generation diagnostic methods such as Magnetic Resonance Imaging (MRI). The rarity of their occurrence can sometimes lead to misleading diagnoses, as both illnesses rarely coexist and share certain clinical and radiological characteristics. RCVS progresses rapidly and can result in chronic impairment or even in-hospital mortality in 5-10% of cases. On the other hand, PRES is a monophasic and reversible illness, although there have been instances of recurrence. In this case report, the authors present the case of a 22-year-old female who presented with post-natal seizures and severe headaches, suspected to have RCVS along with PRES. Since RCVS and PRES share some overlapping pathophysiological conditions, the findings of MRI, including Fluid-Attenuated Inversion Recovery (FLAIR), CR-Magnetic Resonance Angiography (MRA), Apparent Diffusion Coefficient (ADC), and Diffusion-Weighted Imaging (DWI), along with their correlation with clinical features, can help in differentiation. Vasoconstriction has been reported as the main cause; hence vasodilators are primarily suggested for controlling seizures. The patient survived and showed improvement. Initially, the patient was monitored every 15 days for one month, followed by monthly follow-ups up to 8 months after discharge. The purpose of presenting this case is to enhance understanding of RCVS with PRES, including associated risk factors, which can lead to better outcomes through timely diagnosis. Improved outcomes can be achieved through early recognition and improved clinical management.

PRIMARY ANGIITIS OF THE CENTRAL NERVOUS SYSTEM - A DIAGNOSTIC CHALLENGE.

Primary angiitis of the central nervous system (PACNS) is a rare and severe disease confined to the central nervous system, i.e., the brain and spinal cord. The etiology, pathogenesis and immune mechanism of PACNS have not yet been completely elucidated. The diagnosis is challenging; it is based upon constellation of clinical picture, cerebrospinal fluid analysis, imaging methods or tissue biopsy as the gold standard. In differential diagnosis of PACNS, it is necessary to rule out infectious, malignant or systemic inflammatory diseases, as well as reversible cerebral vasoconstriction syndrome. Immunosuppressants are cornerstone therapy for PACNS, although evidence-based strategies for the management are lacking so far. PACNS is an entity with considerable morbidity and mortality. Awareness of this rare and heterogeneous disease is crucial for establishing early diagnosis and treatment initiation.

ファスジル静脈投与が奏功した可逆性脳血管攣縮症候群の1例

ファスジル静脈投与が奏功した可逆性脳血管攣縮症候群の1例

Reversible Cerebral Vasoconstriction Syndrome following Exchange Transfusion and Steroids in a Child with Sickle Cell Disease: A Case Report

A 5-year-old female with SCD presented with thunderclap headaches, followed by stabbing headaches after receiving an exchange transfusion and prednisolone. She developed ischemic infarcts in the left parietal and right paramedian frontal and parietal lobes.

Vasoconstriction and long-term headache in reversible cerebral vasoconstriction syndrome.

Angiographic vasoconstriction in reversible cerebral vasoconstriction syndrome (RCVS) is often undetectable at symptom onset and the diagnosis relies on clinical presentation. Although thunderclap headache is a hallmark feature of RCVS, the incidence and predictors of long-term headaches (LTH) are incompletely understood. Our study aims were twofold: to examine the sensitivity and specificity of a recently developed score (RCVS2) for vasoconstriction detection in a real-world clinical context and describe the incidence and predictors of LTH beyond the acute phase of RCVS. Retrospective analysis of consecutive patients with clinical diagnosis of RCVS in a tertiary hospital between 2017 and 2021. We examined associations between demographic factors, comorbidities, medications, imaging characteristics, and LTH (defined as at least one episode present at greater than 6-months follow-up necessitating medication). We separately examined the association between RCVS2 score and angiographic vasoconstriction and computed its sensitivity, specificity, and negative and positive predictive value based on established cutoffs (certain ≥ 5, negative ≤ 2). We included 55 patients, 50.5 (± 13.7) years; 41 (75%) female. 25 (49%) patients had LTH; only prior history of headache was significantly associated with LTH [OR 4.3, 95% CI (1.1-16.2), p = 0.03]. We found a significant association between RCVS2 score and angiographic vasoconstriction [OR 1.49, 95% CI (1.18-1.88), p = 0.001]; sensitivity, specificity, and positive and negative predictive value were 64%, 94%, 95% and 58% respectively. Approximately 50% of RCVS patients experienced LTH; only prior headache history was associated with its incidence. The RCVS2 score had a significant association with high specificity and positive predictive value for angiographic vasoconstriction in our cohort, validating its utility in improving the accuracy of diagnosis in the clinical setting.

Severe headache in a middle-aged woman after device closure of a ventricular septal defect.

Reversible cerebral vasoconstriction syndrome presents with thunderclap headache and represents a group of conditions that show reversible multifocal narrowing of cerebral arteries. Some patients who undergo device closure of an atrial septal defect complain of headache, which are posited as a migraine. Here we report a case of severe headache due not to migraine but reversible cerebral vasoconstriction syndrome after device closure of a ventricular septal defect.

Improvement of intrapartum reversible cerebral vasoconstriction syndrome over 12 weeks after onset: A Case Report

Improvement of intrapartum reversible cerebral vasoconstriction syndrome over 12 weeks after onset: A Case Report

(89) Managing Reversible Cerebral Vasoconstriction Syndrome in a US Military Veteran with Complex PTSD and Borderline Personality Disorder

(89) Managing Reversible Cerebral Vasoconstriction Syndrome in a US Military Veteran with Complex PTSD and Borderline Personality Disorder

Posterior reversible encephalopathy syndrome and reversible cerebral vasoconstriction syndrome associated with acute exacerbation of chronic obstructive pulmonary disease

Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) are relatively uncommon neurological disorders. These two independent syndromes can be concurrent as a part of a continuum process; however, the specific mechanism is not well known. Although the relationship between RCVS and PRES is currently unclear, they could share a common pathophysiology. This case report aimed to determine the pathophysiology underlying the co-occurrence of PRES and RCVS in a patient with an acute exacerbation of chronic obstructive pulmonary disease.

Differentiation between Call Fleming syndrome and nervous system vasculitis

IntroductionCall Fleming syndrome is a reversible cerebral vasoconstriction syndrome of idiopathic etiology that causes thunderclap headache. It is a condition with a good prognosis, but clinically and radiologically similar to other diseases, such as nervous system vasculitis. Objectives To bring clinical, radiological and laboratorial aspects of Call Fleming Syndrome and nervous system vasculitis and provide the differentiation of both. MethodologyThe research is an integrative review, carried out in June 2022, using the SciElo and Pubmed databases. From the keywords: Call-Fleming syndrome) AND (Diagnosis), (Case report), (Radiology), the clinical, laboratorial and radiological profile of the Call-Fleming Syndrome was traced while the keywords (Nervous System) AND (Vasculitis) AND (Diagnosis), (Case report), (Radiology) were used to CNS vasculitis. Then, from case reports, radiology articles and laboratory analyses, the diseases in question were compared in terms of similarities and differences. ResultsBoth pathologies are similar from a clinical point of view: they are frequent causes of thunderclap headache, a sudden and severe headache that reaches its maximum intensity within 1 minute. Some patients with Call-Fleming Syndrome may present several neurological deficits, the more frequents in case reports are hemiparesis, hemianopia, and loss of balance. The disease has a limited course that lasts from 1 to 3 months, however, its main complications are hemorrhages and cerebral infarction. The age group affected in the reported cases ranges from 10 to 76 years and there is a slight predominance of cases in women. (To see the complete abstract, please, check out the pdf).