RCVS\u2013TCH score can predict reversible cerebral vasoconstriction syndrome in patients with thunderclap headache

Objective: To develop a method to distinguish reversible cerebral vasoconstriction syndrome (RCVS) from other large/medium-vessel intracranial arteriopathies. Methods: We identified consecutive patients from our institutional databases admitted 2013-2017 with newly diagnosed RCVS (n=30) or non-RCVS arteriopathy (n=80). Admission clinical and imaging features were compared. Multivariate logistic regression modelling was used to develop a discriminatory score. Score validity was tested in a separate cohort of patients with RCVS and its closest mimic, primary angiitis of the central nervous system (PACNS). Additionally, key variables were used to develop a bedside approach to distinguish RCVS from non-RCVS arteriopathies. Results: The RCVS group had significantly more women, vasoconstrictive triggers, thunderclap headaches, normal brain imaging results, and better outcomes. Beta coefficients from the multivariate regression model yielding the best c-statistic (0.989) were used to develop the RCVS 2 score (range, -2 to +10; R ecurrent/single thunderclap headache; C arotid artery involvement; V asoconstrictive trigger; S ex; S ubarachnoid hemorrhage). Score ≥5 had 99% specificity and 90% sensitivity for diagnosing RCVS, and score ≤2 had 100% specificity and 85% sensitivity for excluding RCVS. Scores 3-4 had 86% specificity and 10% sensitivity for diagnosing RCVS. The score showed similar performance to distinguish RCVS from PACNS in the validation cohort. A clinical approach based on recurrent thunderclap headaches, trigger and normal brain scans, or convexity subarachnoid hemorrhage, correctly diagnosed 25 of 37 patients with RCVS 2 scores 3-4 across the derivation and validation cohorts. Conclusion: RCVS can be accurately distinguished from other intracranial arteriopathies upon admission, using widely available clinical and imaging features.

To develop a method to distinguish reversible cerebral vasoconstriction syndrome (RCVS) from other large/medium-vessel intracranial arteriopathies. We identified consecutive patients from our institutional databases admitted in 2013-2017 with newly diagnosed RCVS (n = 30) or non-RCVS arteriopathy (n = 80). Admission clinical and imaging features were compared. Multivariate logistic regression modeling was used to develop a discriminatory score. Score validity was tested in a separate cohort of patients with RCVS and its closest mimic, primary angiitis of the CNS (PACNS). In addition, key variables were used to develop a bedside approach to distinguish RCVS from non-RCVS arteriopathies. The RCVS group had significantly more women, vasoconstrictive triggers, thunderclap headaches, normal brain imaging results, and better outcomes. Beta coefficients from the multivariate regression model yielding the best c-statistic (0.989) were used to develop the RCVS2 score (range -2 to +10; recurrent/single thunderclap headache; carotid artery involvement; vasoconstrictive trigger; sex; subarachnoid hemorrhage). Score ≥5 had 99% specificity and 90% sensitivity for diagnosing RCVS, and score ≤2 had 100% specificity and 85% sensitivity for excluding RCVS. Scores 3-4 had 86% specificity and 10% sensitivity for diagnosing RCVS. The score showed similar performance to distinguish RCVS from PACNS in the validation cohort. A clinical approach based on recurrent thunderclap headaches, trigger and normal brain scans, or convexity subarachnoid hemorrhage correctly diagnosed 25 of 37 patients with RCVS2 scores 3-4 across the derivation and validation cohorts. RCVS can be accurately distinguished from other intracranial arteriopathies upon admission, using widely available clinical and imaging features. This study provides Class II evidence that the RCVS2 score accurately distinguishes patients with RCVS from those with other intracranial arteriopathies.

37 - Reversible Cerebral Vasoconstriction Syndromes

To report 2 cases of reversible cerebral vasoconstriction syndrome (RCVS) with posterior reversible encephalopathy syndrome (PRES) after blood transfusion for severe anemia. RCVS is presented with recurrent thunderclap headache and reversible constriction of cerebral arteries. PRES is a known complication of RCVS. Blood transfusion for severe anemia could be a cause for PRES in few cases; however, it is seldom mentioned as an etiology for RCVS. We report a case series. We report 2 women presented with RCVS with PRES after blood transfusion for anemia, and reviewed another 4 similar cases reported in the literature. Our 2 patients were middle-aged women, with severe chronic anemia (average hemoglobin: 1.45 g/dL), and received multiple blood transfusions (average: 3250 mL) over a period of 5-7 days. They developed thunderclap headache and other symptoms about 1 week after the last blood transfusion. Cerebral vasoconstrictions were demonstrated by magnetic resonance angiography and transcranial color-coded sonography. PRES was found in both of them using magnetic resonance imaging, and one of them also had cytotoxic edema on diffusion weighted image. RCVS with PRES is one complication of blood transfusion in patients under chronic severe anemia (especially when hemoglobin level increased for more than 5 g/dL), particularly in Asian women with menorrhagia. Blood pressure surge and the occurrence of severe headaches or other neurological symptoms should be aggressively monitored within 10 days after the last blood transfusion.

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by recurrent thunderclap headaches and evidence of vasoconstriction with subsequent resolution. The clinical course of RCVS is traditionally considered monophasic and benign. However, recurrent episodes of focal neurological symptoms have been described after initial presentation. To define the frequency, timing, and consequences of clinical worsening in patients with diagnosis of RCVS. Retrospective observational study of consecutive patients with RCVS at 2 referral institutions for neurological disease. Clinical worsening after diagnosis of RCVS. We defined clinical worsening as new permanent or transient neurological deficits (compared with presenting signs and symptoms) or new onset of seizures. We performed a logistic regression analysis to assess associations between patient characteristics and clinical worsening. Functional outcome was assessed at 1 to 3 months using the modified Rankin score. We identified 59 patients (median age, 47 years; interquartile range, 32-54 years) with RCVS. Twenty patients (34%) experienced clinical worsening after a median of 2.5 days (range, several hours to 14 days). Eight of the 20 patients who worsened had permanent deficits, including 4 who died. We did not find an association between age, sex, smoking, migraine, acute or chronic hypertension, peripartum state, or use of serotonergic drugs with clinical worsening. Clinical worsening was associated with radiological infarction (P = .001) and worse functional outcome (P < .004). Functional outcome was favorable (modified Rankin score 0-2) in 51 patients (86.4%). Clinical worsening after diagnosis is common in patients with RCVS. Thus, RCVS is self-limited but not strictly monophasic. Most patients have a very favorable outcome, but clinical worsening may result in permanent deficits.

Introduction:Reversible cerebral vasoconstriction syndrome(RCVS) is a rare condition that occurs as the result of a sudden constriction(tightening) of the vessels that supply blood to the brain. the main symptom of RCVS is thunderclap headaches. stroke or bleeding in to the brain and seizure may or may not be present. the condition is more commonly diagnosed in women between the ages of 20 to 50 and may be associated with changes that happen in the body immediately after giving birth or changing birth control pills. other risk factors associated with RCVS include: use of drugs, alcohol, nicotin patches and certain tumors.Objective:For the first time in our center we diagnose and treat a postpartum RCVS.Case Description:A 37year old lady was referred to our center with GTCS and thunderclap headache. she had an uncomplicated cesarian section last week. she had no significant past medical history and her blood pressure was normal during pregnancy. drug history was negative. in physical exam blood pressure and neurologic exam were normal. in our assessment brain imaging showed cortical SAH, lab data including blood biochemistries and csf were normal, in Angiography vasospasm in left A1 and in carotid duplex MCA velocity was 220 cm/s. Therefore we started nimodipin and metylprednisolon but her symptom aggravated and she showed increased MCA velocity in TCD.SO BRAIN Angiography was done for the second time that show generalized vasospasm in anterior circulation, bilateral v4 and basilar arteries. nimodipin and milrinone were injected slowly and vasospasm become better.TCD monitoring showed decreased MCAs velocities and her symptom did not recur and in TCD MCAs PSVs were below 150 cm/s.Discussion:We presented a case of postpartum RCVS , that aggravated with intra venous metylprednisolon, and resolved with intra arterial milrinone. RCVS is a syndrome of cerebral arterial vasoconstriction, usually presenting with recurrent thunderclap headaches, seizures and focal neurologic deficits. our patient s symptoms consist of a thunderclap headache and a GTCS. our patient was not a case of eclampsia and other predisposing factors of RCVS were also absent. she was one of the rare reported cases of RCVS in the postpartum period. treatment of RCVS in usually conservative and symptomatic cases. few existing trapeutic option include: oral or intra arterial of CCBs. Recent studies reported that steroids lack beneficial in RCVS and may even worsen vasoconstriction. this is what happened in our patient. She finally improved with intra arterial milrinone injection.

Microvascular brain injury is well recognized in neuropsychiatric systemic lupus erythematosus (SLE), but cerebral large artery involvement is being debated. Three females with SLE, aged 9 to 14years, had immunosuppressive treatment intensification because of lupus nephritis. Within the following days or weeks, they presented with intense cephalalgia - isolated or associated with neurological symptoms - and no or mild hypertension. Magnetic resonance angiography showed multiple stenoses within the circle of Willis. One patient had subsequent small subcortical cerebral infarction. Two patients were treated for neuropsychiatric SLE; one patient was treated for reversible cerebral vasoconstriction syndrome (RCVS). Angiography normalized within a few weeks in all three patients. Retrospectively, clinical and radiological features suggest that RCVS was the most likely diagnosis in all patients. Multidisciplinary analysis of clinical and angiographic features is recommended, as RCVS is rare in children and its recognition may help to adjust treatment. WHAT THIS PAPER ADDS: Reversible vasoconstriction syndrome was observed in paediatric systemic lupus erythematosus. Thorough imaging analysis was necessary to address this diagnosis in paediatric patients.

Reversible cerebral vasoconstriction syndrome (RCVS) is a disorder with distinct features: recurrent thunderclap headaches with reversible vasoconstriction of intracranial arteries. Substantial studies regarding outcomes after RCVS were conducted, showing favorable functional outcomes in most patients despite the potentially life-threatening complications of RCVS, including ischemic stroke, intracranial hemorrhage, or convexity subarachnoid hemorrhage. However, patients may report headaches after the resolution of RCVS while relative studies were scarce. Two prospective studies from different cohorts consistently revealed that RCVS recurred in at least 5% of patients. Patients with prior migraine history and patients whose thunderclap headaches are elicited by sexual activity or exertion are at higher risk for RCVS recurrence. On the other hand, several retrospective studies and case reports reported that chronic headaches are common in RCVS patients after the resolution of acute bouts. The chronic headaches after RCVS are sometimes disabling in certain patients. Headaches after RCVS are not uncommon but usually overseen. Medical attention and examinations are warranted in patient with RCVS who reported recurrence of thunderclap headaches or chronic headaches after RCVS.

Reversible cerebral vasoconstrictionsyndrome (RCVS) is a condition that usually presents with recurrent thunderclap headaches, focal neurological deficits, and seizures, secondary to transient cerebral vascular spasms. Although the mechanism is not well understood, medications that modulate serotonergic activity, such as selective serotonin reuptake inhibitors (SSRIs), may induce RCVS. In this case, we present a 39-year-old female patient with thunderclap headaches and a multitude of neurological deficits, including vision loss and musculoskeletal weakness. Her medical history is significant for depression, migraines, and opioid-use disorder in remission, treated with paroxetine and mirtazapine, sumatriptan, and buprenorphine-naloxone, respectively. The diagnosis of RCVS was supported by imaging that demonstrated multiple acute cerebral infarcts and stenoses. The patient spent a total of eight days as an inpatient, and during that time, her symptoms showed gradual improvement. RCVS is a rare condition that should be considered in a patient with risk factors for whom high-acuity diagnoses such as subarachnoid hemorrhage have been ruled out.

Reversible cerebral vasoconstriction syndrome (RCVS) is a condition characterized by thunderclap headaches, which are sudden and severe headaches that peak within a few seconds. These headaches present diagnostic difficulties due to their diversity and low specificity, often leading to misdiagnoses and patient dissatisfaction. We present the case of a 52-year-old woman with a 10-day history of recurrent thunderclap headaches. Initial imaging revealed no abnormalities, but she experienced further episodes of thunderclap headaches during hospitalization. Subsequent neurovascular imaging revealed multiple intracranial stenoses with a "string of beads" appearance, confirming the diagnosis of reversible cerebral vasoconstriction syndrome. She was treated with nimodipine, and most symptoms had resolved upon discharge, with no recurrence of headache reported during a 3-month follow-up. Prior reviews on reversible cerebral vasoconstriction syndrome predominantly emphasized isolated symptoms or advanced neuroimaging findings, offering limited applicability in primary care services. More attention should be given to identifying clinical manifestations warranting heightened reversible cerebral vasoconstriction syndrome suspicion. Early recognition of reversible cerebral vasoconstriction syndrome counts in primary care services. We proposed a revised diagnostic routine that begins with clinical suspicion prompted by typical manifestations, like recurrent thunderclap headaches, female sex, and specific triggers, and recommends advanced neurovascular imaging when accessible. Extreme headache severity or deviation from prior migraine patterns should raise suspicion for reversible cerebral vasoconstriction syndrome, while diagnostic consideration should still remain in patients with transient neurological deficits, seizures, or cerebrovascular events.

Introduction Reversible cerebral vasoconstriction syndrome (RCVS) is a less considered etiology of acute severe headache in the pediatric population. It is classically characterized by thunderclap headache that is recurrent and reversible multifocal cerebral artery narrowing. While it has been linked to vasoactive medication, immunosuppression, and systemic illness, case reports in the field of pediatric hematology‐oncology are rare. No previous cases have linked RCVS with tovorafenib, an oral pan‐RAF inhibitor in clinical investigation for pediatric BRAF V600E‐mutated malignancies. Because RCVS can be imitated by other neurologic disorders and noninvasive vascular testing can be normal, the diagnosis usually necessitates catheter‐based angiography. Intra‐arterial calcium channel blockers have been utilized in endovascular therapy to obtain both diagnostic accuracy and immediate therapeutic benefit. We describe the first documented case of childhood RCVS associated with tovorafenib with successful endovascular diagnosis and intra‐arterial verapamil treatment. Materials and Methods A 12‐year‐old female with multifocal osseous relapsed Langerhans cell histiocytosis (LCH) harboring BRAF V600E mutation started tovorafenib in February 2025. After six cycles, she developed recurrent thunderclap headache that woke her up from sleep, sometimes followed by brief confusion characteristic of postictal states. Tovorafenib was withheld, and she was evaluated via multidisciplinary evaluation. Comparison brain MRI revealed minimal Chiari I malformation and small sphenoid hyperintensity on the left side. Head and neck CT angiogram was normal. EEG, with 2‐hour study during symptomatic attack, revealed no epileptiform discharge. Ophthalmological examination revealed no papilledema. Because of the persistence of thunderclap headache and nonrevealing noninvasive studies, diagnostic cerebral angiography was done. Results Digital subtraction angiography disclosed focal segmental narrowing of the right A3 segment of the anterior cerebral artery characteristic of RCVS. Verapamil (5 mg) was administered intra‐arterially, with resultant immediate angiographic resolution of the vasospasm. Lumbar puncture ruled out infection and inflammation. The patient was switched to oral verapamil with rapid resolution of her thunderclap headaches. Interestingly, tovorafenib was resumed on hospitalization day with continuation of verapamil, and no additional RCVS events were experienced. Discharge on oral verapamil with follow‐up as scheduled in neurology, neurosurgery, and oncology clinics was successful. Conclusion This case highlights the critical importance of endovascular evaluation and treatment of potential pediatric RCVS, especially when vasoconstriction is not detected by noninvasive imaging. In this patient, both therapeutic and diagnostic angiography was in keeping with RCVS and reversible following intra‐arterial verapamil infusion. Temporal correlation of tovorafenib exposure and onset of symptoms, together with angiographic response to calcium channel blockade, is compatible with drug‐related mechanism. One possible explanation would be inhibition of RAF pathway altering vascular smooth muscle or endothelial tone, which predisposes to vasoconstriction. To our knowledge, this is the first description of tovorafenib‐associated RCVS. This presentation underscores the need to heighten awareness of RCVS as an emergent side effect of new targeted therapies in children. Early diagnosis, multidisciplinary assessment, and prompt endovascular therapy will prevent morbidity and enable resumption of potentially life‐extending oncologic therapy. Additional pharmacovigilance and further study are necessary to better clarify the incidence, pathogenesis, and treatment of this new complication.

Objectives To report demographics and characteristics of reversible cerebral vasoconstriction syndrome (RCVS) in the Korean cohort. Methods We prospectively recruited patients with definite (imaging-proven) RCVS and probable (imaging-negative) RCVS who visited Samsung Medical Center between June 2012 and September 2016. Clinical manifestations, neuroimaging, treatment, and clinical outcomes were evaluated in all patients. Characteristics of RCVS without typical causes ("idiopathic RCVS") were compared with those of RCVS with identifiable causes ("secondary RCVS"). International Classification of Headache Disorders (ICHD)-3 beta criteria for 6.7.3 RCVS and 6.7.3.1 probable RCVS were tested. Results A total of 138 patients (104 definite and 34 probable RCVS) were included in this study. Patients with definite RCVS were predominantly female (85.6%) and middle-aged (mean, 50.7 [range, 23-82] years). Probable RCVS was associated with less female predominance (70.6%, p = 0.049), more typical manifestations ( p < 0.001), and none of neurological complications. One-hundred and one (97.1%) patients with definite RCVS had headache, but the typical "recurrent and/or triggered" thunderclap headache was reported in only 83 (82.2%). In most patients with definite RCVS (84.6%), RCVS was idiopathic, while only 16 (15.4%) had secondary causes. Compared to those with secondary RCVS, patients with idiopathic RCVS were older (52.8 ± 11.42 vs. 39.1 ± 9.55 years, p < 0.001). Patients with secondary RCVS had more complications than those with idiopathic RCVS (40.5% vs. 12.5%, p = 0.018). Among idiopathic RCVS patients, 33 (37.5%) reported a preceding event or a change in lifestyle, environment, health, or medication within one month before onset. Conclusion In our cohort, RCVS was benign and idiopathic in most patients, and occurred frequently in middle-aged women. Manifestations of RCVS were more diverse than previously recognized, and forms without any headache existed. Different genetic, social, and environmental factors should be taken into account to unveil the spectrum and pathophysiology of RCVS.

Syndrome de vasoconstriction cérébrale réversible : une cause de céphalée en coup de tonnerre peu connue chez l’enfant

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

112. Reversible cerebral vasoconstriction syndrome associated with pregnancy in peripartum period