Search Results (14)

Background: Reversible cerebral vasoconstriction syndrome (RCVS) is an uncommon and often underrecognized neurovascular disorder. We aimed to investigate the clinical presentations associated with extracerebral vasoconstriction in patients diagnosed with RCVS. Methods: In this cross-sectional study, we analyzed data from a single-center cohort of patients with RCVS in Korea. Extracerebral vasoconstriction in individuals diagnosed with RCVS was defined by the following criteria: (1) the presence of sudden and severe pain in extracerebral regions (primarily the chest or abdomen) coinciding with the onset of RCVS, (2) resolution of the pain following the administration of vasodilators, and (3) confirmation of vasoconstriction through imaging studies or, at a minimum, the exclusion of other potential causes associated with the pain. Results: Among the 80 eligible patients (median age, 53 years; female sex, 82.5%), 8 patients (10%) experienced extracerebral vasoconstriction. Regarding pain location, four patients reported chest pain, two reported abdominal pain, and two reported pains in both the chest and abdomen. When comparing visit route, the patients were associated with emergency department (odds ratio [OR]: 6; 95% confidence interval [CI]: 1.1–33; reference: outpatient) and inpatient consultation (OR: 25; 95% CI: 1.1–560) compared to those without extracerebral vasoconstriction. Patients with extracerebral vasoconstriction had no prior history of precipitating conditions or medication use before the onset of RCVS. The treatment response to vasodilators was excellent in all patients, and none reported neurovascular or extracerebral complications during the bout of RCVS. Conclusions: A co-occurrence of extracerebral vasoconstriction was not exceptionally uncommon among patients with RCVS. Our findings suggest that extracerebral vasoconstriction may be underrecognized in individuals with RCVS. Full article

Background and Objectives: Although reversible cerebral vasoconstriction syndrome (RCVS) is a rare disease, the condition may occur with COVID-19 infection. We aimed to investigate the clinical characteristics of RCVS through a systematic review of case reports and case series that reported on COVID-19-related RCVS. Methods: A literature search was performed in PubMed (MEDLINE), SCOPUS, and Web of Science. The search was confined to articles published between 17 November 2019 and 14 August 2024. The search terms were (“COVID-19” OR “SARS CoV-2”) AND (“RCVS” OR “Reversible cerebral vasoconstriction syndrome”). The search protocol was registered in PROSPERO (CRD42024491818). A total of twenty-four cases were included, nine case series consisting of nineteen cases and five cases from our hospital. Clinical characteristics were investigated, including risk factors, symptoms, brain and angiographic findings, treatment options, and prognoses. Results: The average age was 37.1 years, and females comprised 70.8% of the cohort. COVID-19 vaccination was administered in five cases. Vasoconstriction was most frequently noted in middle cerebral arteries (90.9%). Among the included patients, 12 (50.0%) experienced strokes as a complication of RCVS, and the mortality rate was 9.1%. Follow-up imaging findings were available for 14 of the 24 cases (58.3%). Among these, vasoconstriction was fully improved in 64.3%, partially improved in 28.6%, and aggravated in 7.1%. Conclusions: While the recovery rate of vasoconstriction on brain angiographic findings was not uncommon, our systematic review indicates a potential for a relatively poor neurological prognosis in COVID-19-related RCVS. Full article

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare but significant cause of intracranial arteriopathy and stroke in young adults. The syndrome encompasses a spectrum of disorders radiologically characterized by reversible narrowing and dilation of intracranial arteries, often triggered by vasoactive drugs or the postpartum period. The hallmark clinical feature of RCVS is thunderclap headache with or without other neurological signs. Though endothelial dysfunction and sympathetic hyperactivation are hypothesized to be key mechanisms, the exact pathogenesis of RCVS is still unclear. RCVS’s diagnosis could be challenging, since vasospasm proceeds centripetally, initially involving distal small pial and cortical arteries, and angiographic studies, especially brain magnetic resonance angiography (MRA) and computed tomography angiography (CTA), may miss it in the early phase of the disease, while early signs such as vascular hyperintensities may be visible on T2/FLAIR sequences before vasospasm onset. Catheter angiography is the gold standard and it could be used to assess vasospasm reversibility post-intra-arterial vasodilator administration. Treatment is mainly symptomatic, and nimodipine is the most commonly administered therapy, given orally or intra-arterially in severe cases. Since many aspects of RCVS remain partially known, further research is needed to better understand the complex pathophysiology of this unique clinical condition and to optimize specific management strategies. Full article

Spontaneous convexity subarachnoid hemorrhage (cSAH) is a vascular disease different from aneurysmal SAH in neuroimaging pattern, causes, and prognosis. Several causes might be considered in individual patients, with a limited value of the patient’s age for discriminating among these causes. Cerebral amyloid angiopathy (CAA) is the most prevalent cause in people > 60 years, but reversible cerebral vasoconstriction syndrome (RCVS) has to be considered in young people. CAA gained attention in the last years, but the most known manifestation of cSAH in this context is constituted by transient focal neurological episodes (TFNEs). CAA might have an inflammatory side (CAA-related inflammation), whose diagnosis is relevant due to the efficacy of immunosuppression in resolving essudation. Other causes are hemodynamic stenosis or occlusion in extracranial and intracranial arteries, infective endocarditis (with or without intracranial infectious aneurysms), primary central nervous system angiitis, cerebral venous thrombosis, and rarer diseases. The diagnostic work-up is fundamental for an etiological diagnosis and includes neuroimaging techniques, nuclear medicine techniques, and lumbar puncture. The correct diagnosis is the first step for choosing the most effective and appropriate treatment. Full article

Reversible cerebral vasoconstriction syndrome (RCVS) typically manifests as a sudden, severe thunderclap headache due to narrowing of the cerebral arteries. Symptoms usually resolve within three months. An imbalance in cerebral vascular tone, an abnormal endothelial function, and a decreased autoregulation of cerebral blood flow are thought to be involved in the pathogenesis of RCVS. However, the precise origin of this condition is not yet fully understood. Symptoms of Raynaud’s phenomenon (RP) include vasospasm of arterioles of the digits. The pathophysiology of RP includes interactions between the endothelium, smooth muscle, and autonomic and sensory neurons that innervate arteries to help maintain vasomotor homeostasis. RP may occur before the clinical manifestation of a rheumatic condition. RCVS is rare in patients with autoimmune rheumatic disease. We describe a 54-year-old female who had a history of Raynaud’s phenomenon affecting her fingers and toes since the age of 12 years. The patient was diagnosed with RCVS in 2012. She described RCVS precipitants, including the regular use of cannabis, cocaine, and amphetamine and tobacco smoking. In 2021, she presented with oral ulcers, intermittent swallowing difficulties, and Raynaud’s phenomenon. Clinical examination revealed early sclerodactyly, and abnormal nail-fold capillaroscopy showed multiple giant capillaries, dilated capillary loops, and areas of capillary hemorrhage with capillary drop-out. The investigation revealed positive ANA, strongly positive SRP antibodies, and Ro60 antibodies. Our case report indicates that there may be a correlation between RCVS and Raynaud’s phenomenon, and a potential connection between RCVS and autoimmune rheumatic diseases. Hence, physicians must be aware of the red flags and subtle differences in neurological abnormalities, such as headaches, in patients with autoimmune rheumatic diseases who have an inactive clinical status to improve patient care and outcomes. Full article

Reversible cerebral vasoconstriction syndrome (RCVS) is a condition with variable outcomes presenting a new onset thunderclap headache accompanied by focal neurological symptoms or seizures. It can be idiopathic or arise secondarily to a variety of trigger factors. The condition is increasingly recognized in clinical practice, but many facets remain poorly understood. This article aims to clarify the headache characteristics in RCVS, the temporal association of angiographic findings, the potential association of the condition with SARS-CoV-2 infection, and the clinical presentation of RCVS in children and is based on a systematic PRISMA search for published analytical or large descriptive observational studies. Data from 60 studies that fulfilled specific criteria were reviewed. Most people with RCVS exhibit a typical thunderclap, explosive, or pulsatile/throbbing headache, or a similar acute and severe headache that takes longer than 1 min to peak. Atypical presentations or absence of headaches are also reported and may be an underrecognized phenotype. In many cases, headaches may persist after resolution of RCVS. Focal deficits or seizures are attributed to associated complications including transient ischemic attacks, posterior reversible encephalopathy syndrome, ischemic stroke, cerebral edema, and intracranial hemorrhage. The peak of vasoconstriction occurs usually within two weeks after clinical onset, possibly following a pattern of centripetal propagation, and tends to resolve completely within 3 months, well after symptoms have subsided. There are a few reports of RCVS occurring in relation to SARS-CoV-2 infection, but potential underlying pathophysiologic mechanisms and etiological associations have not been confirmed. RCVS occurs in children most often in the context of an underlying disease. Overall, the available data in the literature are scattered, and large-scale prospective studies and international collaborations are needed to further characterize the clinical presentation of RCVS. Full article

Neurological complications in pregnancy and the puerperium deserve particular attention from specialists due to the worsening of the clinical picture for both the mother and the fetus. This narrative review of existing data in the literature aims to analyze the most common “red flag symptoms” attributable to neurological complications such as pre-eclampsia (PE), eclampsia, HELLP syndrome, posterior reversible encephalopathy syndrome (PRES), cerebral vasoconstriction syndrome (RCVS), stroke, CVS thrombosis, pituitary apoplexy, amniotic fluid embolism and cerebral aneurysm rupture, with the aim of providing a rapid diagnostic algorithm useful for the early diagnosis and treatment of these complications. The data were derived through the use of PubMed. The results and conclusions of our review are that neurological complications of a vascular nature in pregnancy and the puerperium are conditions that are often difficult to diagnose and manage clinically. For the obstetrics specialist who is faced with these situations, it is always important to have a guide in mind in order to be able to unravel the difficulties of clinical reasoning and promptly arrive at a diagnostic hypothesis. Full article

High-resolution magnetic resonance imaging (HRMRI) is the most important and popular vessel wall imaging technique for the direct assessment of vessel wall and cerebral arterial disease. It can identify the cause of stroke in high-risk plaques and differentiate the diagnosis of head and carotid artery dissection, including inflammation, Moya Moya disease, cerebral aneurysm, vasospasm after subarachnoid hemorrhage, reversible cerebral vasoconstriction syndrome, blunt cerebrovascular injury, cerebral arteriovenous malformations, and other stenosis or occlusion conditions. Through noninvasive visualization of the vessel wall in vitro, quantified assessment of luminal stenosis and pathological features of the vessel wall can provide clinicians with further disease information. In this report, technical considerations of HRMRI are discussed, and current clinical applications of HRMRI are reviewed. Full article

(1) Background: Reversible cerebral vasoconstriction syndrome (RCVS) encompasses a clinical and radiological diagnosis characterized by recurrent thunderclap headache, with or without focal deficits due to multifocal arterial vasoconstriction and dilation. RCVS can be correlated to pregnancy and exposure to certain drugs. Currently, the data on prevalence of RCVS in the postpartum period is lacking. We aim to investigate the prevalence of RCVS in the postpartum period and the rate of hemorrhagic complications of RCVS among the same group of patients; (2) Methods: We conducted the metanalysis by using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA), and Meta-Analyses and Systematic Reviews of Observational Studies in Epidemiology (MOOSE) protocol. To analyze the Bias, we used the Ottawa Newcastle scale tool. We included only full-text observational studies conducted on humans and written in English. We excluded Literature Reviews, Systematic Reviews, and Metanalysis. Additionally, we excluded articles that did not document the prevalence of RCVS in the postpartum period (3). Results: According to our analysis, the Prevalence of RCVS in the postpartum period was 129/1083 (11.9%). Of these, 51/100 (52.7%) patients had hemorrhagic RCVS vs. 49/101 (49.5%) with non-hemorrhagic RCVS. The rates of Intracerebral Hemorrhage (ICH) and Subarachnoid Hemorrhage (SAH) were (51.6% and 10.7%, respectively. ICH seems to be more common than.; (4) Conclusions: Among patients with RCVS, the prevalence in PP patients is relativity high. Pregnant women with RCVS have a higher recurrence of hemorrhagic vs. non-hemorrhagic RCVS. Regarding the type of Hemorrhagic RCVS, ICH is more common than SAH among patients in the postpartum period. Female Sex, history of migraine, and older age group (above 45) seem to be risk factors for H-RCVS. Furthermore, recurrence of RCVS is associated with a higher age group (above 45). Recurrence of RCVS is more commonly idiopathic than being triggered by vasoactive drugs in the postpartum period. Full article

A 30-year-old male diagnosed three years previously with reversible cerebral vasoconstriction syndrome (RCVS) presented to the department of neurology with an accumulation of attacks mimicking previous RCVS attacks and fulfilling the diagnostic criteria for RCVS after receiving the first Pfizer COVID-19 vaccine. The neurologic exam, blood samples, electrocardiogram (ECG), and computer tomography of the head (CTC) were normal. The patient was treated with the angiotensin 2 receptor antagonist, losartan, with a good response and was discharged with a prescription for losartan lasting until three days after the second Pfizer COVID-19 vaccine. No further RCVS attacks were reported. These findings indicate that the COVID-19 vaccine might induce RCVS attacks in susceptible individuals, and targeting the angiotensin 2 receptor could be a preventive option. Full article

Reversible cerebral vasoconstriction syndromes (RCVS) is a rare disease that is characterized by reversible multifocal stenosis of the cerebral arteries with various clinical manifestations. Though the pathomechanism of RCVS was unclear, we reported RCVS related to the levonorgestrel-releasing intrauterine system (IUS). A previous healthy 36-year-old woman had thunderclap headache after implanting the levonorgestrel-releasing IUS a year ago. In the serial angiography, we initially found left vertebra artery (VA), and then additionally new stenosis of both anterior cerebral arteries and middle cerebral arteries (MCA). Bilateral MCA stenosis improved but developed stenosis of right VA after a week. The mean flow velocities of both MCA increased in the first transcranial doppler (TCD), but normalized in the follow up TCD. Levonorgestrel might act as the vasoconstrictitve factor that increased the level of endothelin-1, diminished the release of NO and raised oxidative low-density lipoprotein (LDL). Although the exact pathological mechanisms for RCVS were not yet elucidated, this case might help clinicians understand the mechanisms of RCVS. Full article

There is increasing evidence about a link between migraine, cerebral artery dissection and reversible cerebral vasoconstriction syndrome. We present a case of a patient who had all three diagnoses. The clinical evaluation of this patient was further complicated by a post-dural puncture headache, which added to the three types of headache already known to the patient. This clinical case supports the association between migraine, cerebral artery dissection and reversible cerebral vasoconstriction syndrome and highlights the importance of a precise history taking in establishing the correct diagnosis in patients with headache. Full article

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. Full article