Cerebral Vasoconstriction Research Articles

Reversible cerebral vasoconstriction syndrome following carotid artery revascularization: About three case reports and review of literature

Complication after carotid artery revascularization is mainly represented by stroke. Reversible cerebral vasoconstriction syndrome triggering by carotid artery revascularization is exceptional but it is an unrecognized aetiology of stroke. It could be associated with brain edema and henceforth, a posterior reversible encephalopathy syndrome can be confused with post-carotid artery revascularization cerebral hyperperfusion syndrome. We reported three cases about reversible cerebral vasoconstriction syndrome following revascularization therapy whose one had also a posterior reversible encephalopathy syndrome. This complication occurred within two weeks after carotid artery revascularization. All took nimodipine and all had a functional improvement at 3-months follow-up. Reversible cerebral vasoconstriction syndrome with posterior reversible encephalopathy syndrome after carotid artery revascularization may mimic cerebral hyperperfusion syndrome. After a comprehensive review, nineteen cases of reversible cerebral vasoconstriction syndrome post-carotid artery revascularization have been reported in the literature mainly after symptoms. It occurs mainly in women around sixty years of age. The onset is expected four days after revascularization.

Reversible Cerebral Vasoconstriction Syndrome Following Blood Transfusion in a Patient With Chronic Anemia: A Case Report.

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare neurological disorder characterized by transient constriction and dilation of cerebral arteries, leading to severe headaches and neurological deficits. This case report describes a 41-year-old woman with chronic anemia, acute chronic kidney disease, type 2 diabetes mellitus, and rheumatoid arthritis who developed RCVS following transfusion of packed red blood cells (PRBCs). She experienced sudden-onset seizures and a thunderclap headache 5 days post-transfusion. Diagnostic imaging, including computed tomography (CT) and magnetic resonance imaging (MRI), revealed the characteristic features of vasogenic edema. The patient was treated with blood pressure control and symptomatic relief for pain, resulting in gradual improvement. This case highlights the importance of recognizing RCVS as a potential complication of blood transfusions, particularly in patients with significant comorbidities. Understanding the possible mechanisms, including rapid hemoglobin correction, and the effects of residual plasma and storage lesions in transfused blood, is essential to prevent and manage this rare but serious condition.

Real-world study of adverse events associated with triptan use in migraine treatment based on the U.S. Food and Drug Administration (FDA) adverse event reporting system (FAERS) database

BackgroundTriptans selectively agoniste 5-Hydroxytryptamine(5-HT) receptors and are widely used in the treatment of migraine. Nevertheless, there is a dearth of comprehensive real-world clinical research on the safety of triptans. In light of the growing prevalence of migraine, it is imperative to gain a deeper understanding of the true extent of adverse events (AEs) associated with triptans in the clinical management of migraine.MethodsA database query of AEs reported to the U.S. Food and Drug Administration (FDA) Adverse Event Reporting System (FAERS) database for triptans was performed using the online platform Open Vigil 2.1. The query spanned the period from 1 January 2018 to 31 December 2023 and extracted all AEs for ‘sumatriptan’, ‘zolmitriptan’, ‘rizatriptan’, and ‘naratriptan’ from the 15–49 years old population and retrospective quantitative analyses. A proportional reporting ratio (PRR), reporting odds ratio (ROR), and Bayesian Confidence Propagation Neural Network (BCPNN) methodology were utilized to contrast AEs across the four triptans.ResultsA total of 1.272 AEs reports for sumatriptan, 114 for zolmitriptan, 162 for rizatriptan, and 15 for naratriptan were identified. The ratio of females to males was approximately three times higher in all cases, with the highest number of reports originating from the Americas. A review of the FAERS database revealed that nervous system disorders were the primary SOC category for four drugs, with all four drugs exhibiting the AE indicative of reversible cerebral vasoconstriction syndrome, also classified as Nervous system disorders. The most frequently reported AE signal for sumatriptan was dyspnea, which is classified as respiratory, thoracic and mediastinal disorders. The most frequently reported AEs signals for the remaining three drugs were nausea, vomiting and terminal ileitis, all of which are classified as gastrointestinal disorders.ConclusionAnalyses have demonstrated that AEs are present in a range of systems, including cardiac, nervous, gastrointestinal, and musculoskeletal disorders. It should be noted, however, that the incidence and signal intensity of these AEs vary depending on the specific drug in question. In clinical practice, the selection of an appropriate drug and the monitoring of AEs should be tailored to the individual patient’s and specific characteristics.

Reversible Cerebral Vasoconstriction Syndrome

Reversible cerebral vasoconstriction syndrome (RCVS) is a collective term used to describe pathological conditions with a similar clinical and radiological picture which is characterized by thunderclap headaches with a sudden onset and reversible segmental (multifocal) constriction of the cerebral arteries. The article is devoted to issues of terminology, genetic and clinical features of RSCV, and also discusses risk factors, differential diagnosis, complications and prognosis. Modern pathogenetic mechanisms and possible approaches to the treatment of this condition are presented.

Investigation of brain diffusion changes in children with primary nephrotic syndrome

Primary nephrotic syndrome, which occurs with the deterioration of kidney function, can subsequently affect the brain with systemic immune activation, vasculopathy and ischemia. The main aim of this study was to investigate the effectiveness of apparent diffusion coefficient (ADC) and diffusion weighted imaging (DWI) in identifying and detecting brain changes in pediatric patients with primary nephrotic syndrome (PNS). The study included 24 pediatric patients with PNS and 60 healthy children as a control group. The apparent diffusion coefficient values of caudate nucleus, frontal cortex, thalamus, lentiform nucleus, anterior crus and posterior crus of the internal capsule, frontal and occipital white matter were measured quantitatively. The ADC values of thalamus, occipital white matter, caudate nucleus and frontal cortex in the PNS group were significantly lower than in the control group (p < 0.05 for all). No statistically significant difference was detected between the groups with respect of other brain locations. Systemic effects and possible complications of primary nephrotic syndrome may lead to diffusion changes in brain tissue. The decrease in ADC values in patients with PNS may be explained by decreased cerebral perfusion due to cerebral vasoconstriction and vasculopathy.

Convexity subarachnoid hemorrhage revealed contralateral internal carotid artery dissection due to Eagle syndrome: a case report

BackgroundAtraumatic localized convexity subarachnoid hemorrhage (cSAH) is an uncommon form of nonaneurysmal subarachnoid hemorrhage characterized by bleeding limited to the cerebral convexities. Ipsilateral cSAH can result from a variety of causes, such as internal carotid artery stenosis, obstruction, and dissection, although concomitant contralateral cSAH is exceptionally rare. In this case, the initial findings of cSAH led us to discovering contralateral internal carotid artery dissection (ICAD) and an elongated styloid process (ESP). ESP is recognized as a risk factor for ICAD, which is a hallmark of Eagle syndrome. This sequence of findings led to the diagnosis of Eagle syndrome, illustrating a complex and intriguing interplay between cerebrovascular conditions and anatomical variations.Case presentationA 47-year-old Japanese woman experienced acute onset of headache radiating to her neck, reaching its zenith approximately two hours after onset. Given the intractable nature of the headache and its persistence for three days, she presented to the emergency department. Neurological examination revealed no abnormalities, and the coagulation screening parameters were within normal ranges. Brain computed tomography (CT) revealed right parietal cSAH, while CT angiography (CTA) revealed ICAD and an ESP measuring 30.1 mm on the left side, positioned only 1.4 mm from the dissected artery. The unusual occurrence of contralateral cSAH prompted extensive and repeated imaging reviews that excluded reversible cerebral vasoconstriction syndrome (RCVS), leading to a diagnosis of left ICAD secondary to Eagle syndrome. The patient underwent conservative management, and the dissected ICA spontaneously resolved. The patient has remained recurrence-free for two and a half years.ConclusionsManaging cSAH requires diligent investigation for ICAD, extending beyond its identification to explore underlying causes. Recognizing Eagle syndrome, though rare, as a potential etiology of ICAD necessitates the importance of evaluating ESPs. The method for preventing recurrent cervical artery dissection due to Eagle syndrome is controversial; however, conservative management is a viable option.

Cerebral Artery Vasoconstriction After Galcanezumab Loading Dose for Migraine Prevention: A Case Report.

Anti-calcitonin gene-related peptide (CGRP) monoclonal antibodies that target CGRP ligands or receptors, may cause a very rare side effect of reversible cerebral vasoconstriction syndrome (RCVS). This study is a case report of a patient who developed cerebral artery vasoconstriction documented on serial brain magnetic resonance angiography (MRA) scans without the typical manifestations of RCVS following galcanezumab loading dose. Case report: A 40-year-old female patient with high-frequency episodic migraine with visual aura on topiramate 100mg/day developed transient numbness of the right upper and lower extremities and right face without headache and a normal neurological examination 10min after a loading dose of galcanezumab, which resolved over the next 2days. Magnetic resonance angiography brain imaging showed segmental arterial constriction of both middle cerebral arteries in the M1-2 segments and both posterior cerebral arteries in the P1-2 segments, which partial resolved in a subsequent study by the end of 6months. There were no other supporting examination data, such as transcranial Doppler, which might provide additional information on the progression and improvement of the vasoconstriction. Her differential diagnosis included prolonged migraine sensory aura without headache, RCVS, or cerebral vasoconstriction secondary to the effect of an anti-CGRP monoclonal antibody. Further research needs to be conducted.

Treatment-Related Reversible Cerebral Vasoconstriction Syndrome

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.

Blocking α1 Adrenergic Receptor as a Novel Target for Treating Alzheimer's Disease.

While amyloidopathy and tauopathy have been recognized as hallmarks in Alzheimer's disease (AD) brain, recently, increasing lines of evidence have supported the pathological roles of cerebrovascular changes in the pathogenesis and progression of AD. Restoring or ameliorating the impaired cerebrovascular function during the early phase of the disease may yield benefits against the cognitive decline in AD. In the present study, we evaluated the potential therapeutic effects of nicergoline [NG, a well-known α1 adrenergic receptor (ADR) blocker and vasodilator] against AD through ameliorating vascular abnormalities. Our in vitro data revealed that NG could reverse β-amyloid1-42 (Aβ1-42)-induced PKC/ERK1/2 activation, the downstream pathway of α1-ADR activation, in α1-ADR-overexpressed N2a cells. NG also blocked Aβ1-42- or phenylephrine-induced constrictions in isolated rat arteries. All these in vitro data may suggest ADR-dependent impacts of Aβ on vascular function and the reversal effect of NG. In addition, the ameliorating impacts of NG treatment on cerebral vasoconstriction, vasoremodeling, and cognitive decline were investigated in vivo in a PSAPP transgenic AD mouse model. Consistent with in vitro findings, the chronic treatment of NG significantly ameliorated the cerebrovascular dysfunctions and Aβ plaque depositions in the brain. Moreover, an improved cognitive performance was also observed. Taken together, our findings supported the beneficial effects of NG on AD through adrenergic-related mechanisms and highlighted the therapeutic potential of α1-adrenergic vasomodulators against AD pathologies.

Hemorrhagic versus non-hemorrhagic presentation of presumed reversible cerebral vasoconstriction syndrome (RCVS): Presentations and outcomes

Introduction Reversible cerebral vasoconstriction syndrome (RCVS) can present with hemorrhage, ischemia, or both. We aim to compare the differences in presentation and outcomes between patients with RCVS. Methods The hemorrhagic presentation group had 58 patients, and the non-hemorrhagic presentations had 30 patients. Subgroup analysis compared patients with evidence of one or more types of hemorrhage ( n = 53), no evidence of hemorrhage or infarction ( n = 23), evidence of infarction only ( n = 7), and combination of hemorrhage and infarction ( n = 5). Clinical and radiographic data were analyzed. Results Migraine ( p = 0.030) and intracranial tumors ( p = 0.004) were more frequent in non-hemorrhagic presentation. Seizures on admission ( p = 0.047) and higher than average C-reactive protein (CRP) ( p = 0.037) were seen at a higher rate in patients with hemorrhagic presentation. RCVS2 scores were not unexpectedly higher in patients with hemorrhage than non-hemorrhagic presentations ( p = 0.010). Outcomes between the hemorrhagic and non-hemorrhagic groups were comparable. Subgroup analysis found a higher subset of patients with opiate use ( p = 0.046) in the hemorrhage-only group. Patients with hemorrhage presented with a thunderclap headache ( p &lt; 0.001) more often when compared to the other three groups. RCVS2 score was not unexpectedly higher in the hemorrhage-only group compared with the other groups ( p = 0.004). Conclusion A history of migraines was associated with ischemia, while intracranial tumor was significantly associated with evidence of either an infarct, or no changes on imaging. Exposure to opiates, and seizures or thunderclap headache on presentation were associated with hemorrhage. If our data are reproducible, the RCVS2 score may benefit from inclusion of other, small hemorrhages as criterion for diagnosis for RCVS.

Etiology and characteristics of non-aneurysmal thunderclap headache presenting to an acute setting

ObjectivesTo describe the characteristics and causes of non-aneurysmal thunderclap headache (TCH) and compare serious from benign underlying causes. MethodsRetrospective cohort study of consecutive adult patients with TCH presenting to a tertiary care academic medical center between 2010 and 2020. Aneurysmal subarachnoid hemorrhage cases were excluded. Cases were categorized into serious or benign; serious TCH was defined as any condition in which delayed diagnosis and treatment could result in neurological disability or death. Risk factors for serious TCH were analyzed. We adhere to standardized guidelines for reporting observational studies. ResultsA total of 932 patients presented with TCH. After exclusion of 393 patients with aneurysmal-type subarachnoid hemorrhage, 539 were included in the analysis. One-half (n = 275, 51.0 %) had a serious cause. Median age was 51 years, 69.0 % were female. Most frequent diagnoses were intracranial hemorrhage (n = 102, 18.9 %), reversible cerebral vasoconstriction syndrome (n = 97, 18.0 %), and idiopathic TCH (n = 102, 38.6 %). A multivariable logistic regression model for prediction of serious TCH included age, hypertension, migraines, recurrent TCH, level of consciousness and other clinical exam findings, and achieved an AUROC of 0.732. This score had a sensitivity of 79.9 % (95 % CI 73.5–83.5 %) for the identification of serious TCH. A 0.5-point increase in the risk score was associated with a 73 % increase in the odds of serious TCH (odds ratio 1.73, 95 % CI 1.53–1.95, p < 0.001). ConclusionOur study describes the relative frequency of presentation and etiologies among patients with TCH This score can aide clinicians in recognising patients with potentially serious cause of TCH, for whom additional imaging and neurological consultation is necessary.

Management of Psychiatric Diagnoses in Reversible Cerebral Vasoconstriction Syndrome: The Dangers of Worsening Pathology with Serotonergic Medications: A Case Report and Literature Review.

Reversible cerebral vasoconstriction syndrome (RCVS) represents a group of conditions that show reversible multifocal narrowing or constriction of the cerebral arteries that supply blood to the brain. The initial manifestation of RCVS often includes a "thunderclap" headache that is sudden, severe, and often disabling. Stimulants, selective serotonin reuptake inhibitors, serotonin-norepinephrine reuptake inhibitors, and antipsychotics with serotonergic activity can alter the cerebral arterial tone, trigger vasoconstriction, and place patients at risk of a cerebrovascular accident. Thus, psychiatric medications are commonly discontinued on admission for RCVS, and psychiatry is often consulted for input on acute medication management and longitudinal treatment options. Currently, there is a dearth of literature on managing psychiatric medications in RCVS, resulting in variable practice patterns that place patients at risk of withdrawal, decompensation, and relapse. In this article, we provide a case example and aim to consolidate the limited data surrounding the management of psychiatric illness with comorbid RCVS in our discussion. There is a clear concern about worsening and even potentially lethal consequences due to serotonin or stimulant-induced vasospasm both during an acute episode and in long-term management of RCVS. We discuss the underlying pathophysiologic mechanisms proposed for serotonergic-, noradrenergic-, and dopaminergic-induced cerebral vasospasm and how this correlates with the clinical management of patients on psychiatric medications. These data will then be organized to create a risks versus benefits outline to equip psychiatrists to make decisions about when to stop and when to restart psychiatric medications in the setting of RCVS.

A case of postpartum reversible cerebral vasoconstriction syndrome due to excessive use of vasoconstrictive nasal spray

A case of postpartum reversible cerebral vasoconstriction syndrome due to excessive use of vasoconstrictive nasal spray

Pseudoephedrine-Induced Risks of Posterior Reversible Encephalopathy Syndrome and Reversible Cerebral Vasoconstriction Syndrome

Abstract Background Pseudoephedrine is a sympathomimetic drug used as a nasal/sinus decongestant in common cold remedies. Objective The purpose of this review article is to discuss the pseudoephedrine-associated posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS), which have been reported to the Medicines and Healthcare products Regulatory Agency (MHRA) of United Kingdom and Pharmacovigilance Risk Assessment Committee (PRAC) and Committee for Medicinal Products for Human Use (CHMP) of European Medicines Agency (EMA). Methods The aim is to review the literature pertinent to PRES and RCVS linked to the use of pseudoephedrine; the literature was searched in databases such as Medline/PubMed/PMC, Google Scholar, Science Direct, Ebsco, Scopus, Web of Science, Embase, and reference lists. Results The review by MHRA of the United Kingdom and PRAC and CHMP of EMA, all available evidence, including postmarketing safety data, and a few case reports, have confirmed the rare risk of PRES and RCVS associated with the use of pseudoephedrine. Conclusion Health care professionals should be aware of pseudoephedrine-associated PRES and RCVS. Patients with severe or uncontrolled hypertension or those with severe acute or chronic renal disease should avoid using products containing pseudoephedrine.

Approach to Altered Mental Status in Pregnancy and Postpartum.

The evaluation and diagnosis of altered mental status in the pregnant or postpartum patient largely parallels the approach used for any other patient; however, there are several critical differences including that some neuroobstetric diagnoses require emergent delivery of the fetus. Being familiar with the physiological changes and medical complications of pregnancy and delivery is therefore essential. This review first addresses pregnancy-specific disorders that may result in altered mental status, such as the hypertensive disorders of pregnancy and pregnancy-related metabolic and endocrinopathies. The focus then shifts to the complex physiologic changes in pregnancy and how these changes contribute to the distinct epidemiology of pregnancy-related cerebrovascular complications like intracranial hemorrhage, ischemic stroke, and reversible cerebral vasoconstriction syndrome. Medical disorders that are not unique to pregnancy, such as infections and autoimmune conditions, may present de novo or worsen during pregnancy and the peripartum period and require a thoughtful approach to diagnosis and management. Finally, the unique nervous system complications of obstetric anesthesia are explored. In each section, there is a focus not only on diagnosis and syndrome recognition but also on the emergent treatment needed to reverse these complications, bearing in mind the unique physiology of the pregnant patient.

When transfusion causes a splitting headache: A case report and rapid review of transfusion-associated reversible cerebral vasoconstriction syndrome.

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare and understudied transfusion reaction most commonly seen in adult females after correction of chronic, severe anemia. Transfusion-associated RCVS (TA-RCVS) typically presents with thunderclap headaches and one or more systemic (hypertension, nausea/vomiting) or neurologic (seizure, stroke, visual changes) symptoms within a week after red blood cell transfusion. Treatment of RCVS is based on blood pressure control; a recent study suggested that early use of nimodipine could shorten the disease course.

Reversible cerebral vasoconstriction syndrome post-cardiac transplantation: a therapeutic dilemma: case report

BackgroundReversible cerebral vasoconstriction syndrome (RCVS) is characterized by diffuse, multifocal segmental narrowing of cerebral arteries and can result in ischaemic stroke. Causal factors, identified in 60% of cases, include immunosuppressant pharmacotherapy. The few reports following heart transplantation are almost all in Asian recipients. We report on a Caucasian Australian patient with immunotherapy induced RCVS post heart transplantation to highlight the state of knowledge of the condition and the treatment dilemma it poses.Case presentationA 51-year-old female underwent orthotopic heart transplantation at our institution. Induction immunotherapy comprised basiliximab, mycophenolate mofetil and methylprednisolone. On day 6 post-transplantation the patient was transitioned to oral prednisolone and tacrolimus. On day 7 the patient began to experience bilateral, severe, transient occipital and temporal headaches. On day 9 tacrolimus dose was up-titrated. A non-contrast computed tomography brain (CTB) was normal. Endomyocardial biopsy on day 12 demonstrated moderate Acute Cellular Rejection (ACR), which was treated with intravenous methylprednisolone. That evening the patient experienced a 15-minute episode of expressive dysphasia. The following morning she became confused, aphasic, and demonstrated right sided neglect and right hemianopia. A CT cerebral perfusion scan demonstrated hypoperfusion in the left middle cerebral artery (MCA) territory and cerebral angiography revealed widespread, focal multi-segmental narrowing of the anterior and posterior circulations. A diagnosis of RCVS was made, and nimodipine was commenced. As both steroids and tacrolimus are potential triggers of RCVS, cyclosporin replaced tacrolimus and methylprednisolone dose was reduced. A further CTB demonstrated a large left MCA territory infarct with left M2 MCA occlusion. The patient made steady neurological improvement. She was discharged 34 days post-transplantation with mild residual right lower limb weakness and persistent visual field defect on verapamil, cyclosporine, everolimus, mycophenolate mofetil and prednisolone.ConclusionReversible cerebral vasoconstriction syndrome is rare after orthotopic heart transplantation. Until now, RCVS has been almost exclusively described in Asian recipients, and is typically caused by immunotherapy. The condition may lead to permanent neurological deficits, and in the absence of definitive treatments, early recognition and imaging based diagnosis is essential to provide the opportunity to remove the causal agent(s). Co-existent ACR, can pose unique treatment difficulties.

Reversible Cerebral Vasoconstriction and Vertebral Artery Dissection Presenting as Positional Headache

Positional headache refers to a headache that worsens or improves with changes in posture. It is often secondary in nature, associated with various pathologies that may have serious consequences if left undiagnosed or untreated. The authors report a patient who complained of severe positional headache and was diagnosed with concurrent reversible cerebral vasoconstriction syndrome and vertebral artery dissection, thus highlighting the importance of considering such conditions in a patient with postural headaches.

Drugs associated with reversible cerebral vasoconstriction syndrome: A pharmacovigilance study in vigiBase®.

Data on drug-induced reversible cerebral vasoconstriction syndrome (RCVS) are scarce. We aimed to describe RCVS characteristics with drugs previously identified as associated with RCVS and investigate potential signals related to other drugs. VigiBase® was queried for all reports of RCVS until 31 May 2023. A descriptive study was performed on reports concerning drug classes known to precipitate RCVS. To identify new drugs, a disproportionality analysis was conducted. In total, 560 reports were included. RCVS occurred in patients aged between 45-64 years (40%) and 18-44 years (35%), mainly in females (72.5%). Drugs were antidepressants (38.4%), triptans (6.4%), nasal decongestants (3.7%) and immunosupressants (8.7%). In 50 cases, antidepressants were in association with drugs known to precipitate RCVS. The median time to onset was 195 days for antidepressants and much shorter (1-10 days) for triptans, nasal decongestants and immunosuppressants. The outcome was favorable in 87% of cases, and fatal in 4.4%. We found a disproportionality signal with 14 drugs: glucocorticoids, bupropion, varenicline, mycophenolic acid, aripiprazole, trazodone, monoclonal antibodies (erenumab, ustekinumab and tocilizumab), leuprorelin and anastrozole. The present study confirms the role of vasoconstrictors in the onset of RCVS, particularly when used in combination and found potential signals, which may help clinicians envisage an iatrogenic etiology of RCVS.

Multifocal Intracranial Stenosis and Thunderclap Headache in a Patient with Heterozygous MFAP5 Mutation for Familial Thoracic Aortic Aneurysm and Dissection

Recent investigations on familial thoracic aortic aneurysm and dissection (TAAD) identified several genetic variants. Meanwhile, intracranial vasculopathy in familial TAAD has been scarcely reported. We report a case of a young man with Marfanoid habitus and familial TAAD carrying MFAP5, c.472C&gt;T variant. He presented with recurrent thunderclap headache and multifocal intracranial vasculopathy, which is predominantly suggestive of reversible cerebral vasoconstriction syndrome. While the role of MFAP5 in vasculopathy requires clarification, we propose its haploinsufficiency may contribute to both TAAD and intracranial stenosis, highlighting a potential risk of cerebrovascular disease in familial TAAD.