RARE DISEASE
Reversible cerebral vasoconstriction syndrome
Reversible cerebral vasoconstriction syndrome
Reversible cerebral vasoconstriction syndrome
Synonyms: RCVS
Synonyms: RCVS
Synonyms: RCVS
Drug discovery
0
drugs
With orphan designations
Overview
Reversible cerebral vasoconstriction syndrome (RCVS) is a neurovascular disorder characterized by thunderclap headaches and reversible multifocal cerebral arterial narrowing, often presenting as "string of beads" on angiography. Most cases resolve spontaneously within 1-3 months, though complications like ischemic/hemorrhagic strokes (17-32% of cases) or posterior reversible encephalopathy syndrome may occur. Diagnosis requires excluding mimics such as aneurysmal subarachnoid hemorrhage or CNS vasculitis [1][2][6][12].
Therapies
First-line: Oral calcium channel blockers (nimodipine 30-60 mg q4h) for headache control [1][3][11]
Critical interventions: Trigger avoidance (e.g., vasoconstrictive drugs), blood pressure management, and seizure prophylaxis [2][8][12]
Rescue therapy: Intra-arterial vasodilators/angioplasty for refractory vasoconstriction [2][4][11]
Categories: rare neurological diseases
Research Papers
168 drug discovery papers about Reversible cerebral vasoconstriction syndrome. Recent publications:
168 drug discovery papers about Reversible cerebral vasoconstriction syndrome. Recent publications:
categories:
Small molecules
small molecules
2026-08-01 | Reversible Cerebral Vasoconstriction Syndrome: A Medical Challenge in the Peripartum Period Requiring a Multimodal Approach—A Case Report
Reversible cerebral vasoconstriction syndrome is a rare but potentially life-threatening neurological disorder that may occur during pregnancy and the postpartum period and represents an important diagnostic challenge because of its overlap with other obstetric neurological emergencies. We report the case of a 35-year-old woman with a dichorionic diamniotic twin pregnancy who developed severe preeclampsia requiring cesarean delivery at 30 weeks of gestation. On the fifth postpartum day, she experienced thunderclap headache, generalized seizures, and refractory cerebral vasospasm complicated by subarachnoid hemorrhage, cerebral ischemia, and intracranial hypertension. She was managed in a specialized maternal neurocritical care unit using a multimodal strategy that included continuous neuromonitoring with transcranial Doppler ultrasonography, cerebral oxygenation and metabolic monitoring, electroencephalography, intracranial pressure monitoring, serial cerebral angiography with selective intra-arterial milrinone infusion, and, ultimately, ultrasound-guided stellate ganglion block as rescue therapy. Progressive neurological recovery followed, allowing liberation from mechanical ventilation, initiation of rehabilitation, and hospital discharge with a favorable functional outcome. This case highlights the importance of considering reversible cerebral vasoconstriction syndrome in postpartum women presenting with acute neurological deterioration. Early diagnosis, multidisciplinary management, and multimodal neuromonitoring may facilitate individualized treatment and timely therapeutic escalation in patients with refractory cerebral vasospasm. Further prospective studies are needed to better define the role of advanced neuromonitoring and rescue therapies in this rare condition.
2026-07-03 | Reversible cerebral vasoconstriction syndrome with concomitant recurrent subarachnoid hemorrhage and coronary vasospastic angina: A manifestation of systemic vasoconstriction syndrome
Background: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by thunderclap headache and reversible segmental cerebral arterial constriction. Although neurological complications are well recognized, concurrent extracerebral vasoconstriction remains under appreciated. Case Description: A 53-year-old woman presented with sudden chest tightness immediately followed by a thunderclap headache. Initial brain computed tomography and magnetic resonance imaging showed no hemorrhage or infarction; however, magnetic resonance angiography demonstrated multifocal cerebral arterial narrowing, consistent with RCVS. During hospitalization, she developed recurrent nonaneurysmal subarachnoid hemorrhages (SAHs) on days 2 and 4. No bleeding source was identified on serial angiography. Recurrent episodes of chest tightness prompted coronary angiography with acetylcholine provocation, which revealed severe coronary vasospasm, establishing the diagnosis of vasospastic angina (VSA). Treatment with benidipine and nicorandil was initiated thereafter, and no recurrence of either cardiac or neurological symptoms was observed, with complete resolution of cerebral vasoconstriction on follow-up imaging. Conclusion: This case highlights a rare but clinically important association between RCVS, recurrent nonaneurysmal SAH, and coronary VSA confirmed by pharmacologic provocation. Although each condition is potentially reversible, their coexistence supports the concept of systemic vasoconstriction syndrome and underscores the need for vigilance regarding extracranial vascular involvement in patients with RCVS. Early recognition and targeted vasodilator therapy may be essential to prevent serious neurological and cardiovascular complications.
2026-07-01 | E-292 Diagnosis and management of reversible cerebral vasoconstriction syndrome with a systemic rheumatological disorder: a case and updated review of the literature
Reversible cerebral vasoconstriction syndrome(RCVS) is commonly encountered. Clinical scales, such as RCVS2, and non-invasive cerebrovascular imaging are used to differentiate it from primary CNS angiitis and secondary vasospasm. Patients with inflammatory disorders may have higher exposure to vasoactive triggers and other factors that can precipitate RCVS, which may lead to more complicated clinical courses. We present a patient with a complicated course of RCVS with notable history of a rheumatological disorder. Further, we systematically review the literature for RCVS cases with comorbid rheumatological diagnoses to understand the clinical course and treatment heterogeneity for this population. A 51-year-old female with systemic lupus erythematosus vs undifferentiated connective tissue disorder on mycophenolate mofetil and hydroxychloroquine, presented with worst headache of life accompanied with blurry vision, vomiting and unsteady gait. She experienced two episodes of thunderclap headache the week prior. She also had a history of mechanical aortic valve requiring aspirin and warfarin, implantable cardioverter-defibrillator, hypertension, depression on fluoxetine, and chronic pain on cannabidiol. Examination revealed mild right upper extremity drift. She had supratherapeutic INR (4.58). CT head demonstrated right parafalcine SAH with small bilateral frontoparietal intraparenchymal hemorrhages. CT angiography showed multifocal distal arterial narrowing without aneurysm. CSF and blood analyses were noninflammatory. The RCVS2 score was 10, and a diagnosis of RCVS was favored. Despite normalizing the INR, discontinuation of potential offending agents such as MMF, fluoxetine, and hydroxychloroquine, and treatment with oral nimodipine, she developed a new left hemiparesis with worsening vasoconstriction on repeat imaging. Subsequent digital subtraction angiography confirmed the diffuse distal vasoconstriction. Verapamil 10mg was administered in each ICA resulting in angiographic improvement. She had resolution of weakness and returned to her functional baseline within 2 weeks. Systematic review of the literature identified 16 prior cases (table 1). Rheumatologic conditions included SLE, APLA, lupus nephritis, and others. Presentations included infarcts, PRES, and hemorrhage. Most patients had triggers including steroids, immunosuppressants and vasoactive agents. Patients often got serial imaging and increasing doses of calcium channel blockers for variable durations, alongside removal of triggers. However, IA vasodilator therapy was used infrequently. RCVS is a clinicoradiological syndrome of vasoconstriction, and complicated courses have been described with comorbid rheumatologic disease. While the mainstay treatment of RCVS is removal of offending agents and oral calcium channel blockers, we propose that angiography and intra-arterial vasodilator therapy can offer dual diagnostic as well as therapeutic benefits in severe or refractory cases. F. Kanakhara: None. M. Nahhas: None. R. Regenhardt: None. S. Sheth: None.
2026-06-09 | Idiopathic Intracranial Hypertension Following Non-aneurysmal Spontaneous Subarachnoid Hemorrhage in a Sickle Cell Disease Patient with Suspected Reversible Cerebral Vasoconstriction Syndrome (P5-8.009)
To describe a pediatric patient with sickle cell disease presenting with non-aneurysmal spontaneous subarachnoid hemorrhage (SAH) suspected secondary to Reversible Cerebral Vasoconstriction Syndrome (RCVS), later complicated by Idiopathic Intracranial Hypertension (IIH).
2026-05-01 | A54-28 Toxic Clouds: Methamphetamine Induced Reversible Cerebral Vasoconstriction Syndrome (RCVS), Cardiomyopathy and ARDS Following Laced-Hookah Use
Abstract Introduction Amphetamine use can cause diffuse vasospasm, leading to pulmonary hemorrhage, ARDS, and RCVCS. Early diagnosis and supportive care are crucial. Case Report A 30-year-old male with schizophrenia presented to the ED with cough and dyspnea after smoking flavored tobacco (hookah). He was hypoxic, tachycardic, and in respiratory distress with bibasilar crackles. He was intubated, with blood noted in the endotracheal tube. CT chest showed diffuse bilateral ground-glass opacities without PE. Echo revealed new severe LV hypokinesis (EF 10%), and ECGs showed sinus tachycardia. Drug screening confirmed amphetamines and THC. Ventilation was initiated per ARDS protocol along with nebulized tranexamic acid for hemoptysis. He suffered a brief PEA arrest post-intubation and underwent 24 hours of targeted temperature management (TTM). Neuroimaging revealed a small subarachnoid hemorrhage, cerebellar infarct, and multifocal diffuse intracranial vasospasm. Cerebral angiography confirmed reversible cerebral vasoconstriction syndrome (RCVS), treated with intra-arterial then oral verapamil. His condition improved, and he was eventually extubated and discharged to psychiatry. At 1-year follow-up, he remains functional and asymptomatic. Discussion Amphetamines can induce excessive catecholamine release, leading to diffuse vasospasm, endothelial injury, vascular permeability and tissue hypoxia across multiple organ systems; The lungs (ARDS, pulmonary hypertension and DAH), heart (arrhythmias, stress cardiomyopathy, vasospastic angina), eyes (transient monocular blindness, retinal occlusion), kidneys (renal tubular injury), and brain (RCVCS, ischemic and hemorrhagic stroke). The condition is rare but increasingly reported in males under 45 with polysubstance use (as in our case). ARDS from Amphetamine induced pulmonary hemorrhage is uncommon, with few reported cases. Reversible Cerebral Vasoconstriction Syndrome (RCVCS) is exceedingly rare (0.2 cases per million annually), characterized by transient narrowing of cerebral arteries. Though more common in females, amphetamine-associated cases are increasingly recognized amongst males. Diagnostic criteria includes severe (thunderclap-type) headaches, seizures, and/or focal deficits following drug exposure, with imaging showing ischemic or hemorrhagic changes. Digital Subtraction Angiography (DSA) is the gold standard, revealing alternating segments of arterial narrowing and dilation (string of beads appearance). The RCVS2 score incorporates clinical and imaging findings to help rule out other intracranial vasculopathies; a score of ≥ 5 is both highly specific and sensitive. Treatment includes calcium channel blockers such as verapamil, with 90% reported recovery and reversibility demonstrated on angiography within 1-3 months (a key feature). Our patient’s outcome underscores the importance of early recognition of drug-induced vasospasm, management within a multidisciplinary team with interventional capability, and the implementation of supportive care to maintain systemic perfusion. This abstract is funded by: None
antibodies
2025-04-30 | Persistent Headache Attributed to Past Reversible Cerebral Vasoconstriction Syndrome Demonstrating Significant Improvement with Fremanezumab
A 45-year-old woman with a history of migraine experienced thunderclap headaches, after which cerebral vasospasm was confirmed by brain magnetic resonance angiography. Nimodipine treatment initially improved the headache, but moderate daily headaches persisted despite vasospasm resolution. Conventional headache medications including topiramate, propranolol and amitriptyline proved ineffective. Treatment with fremanezumab, a calcitonin gene-related peptide (CGRP)-targeted therapy, resulted in significant reduction of headache frequency and intensity after 3 months. This case suggests the potential effectiveness of CGRP inhibition for persistent headaches following reversible cerebral vasoconstriction syndrome.
2025-04-29 | Reversible cerebral vasoconstriction syndrome in idiopathic multicentric Castleman disease under treatment with tocilizumab.
Idiopathic multicentric Castleman disease (iMCD) is a rare polyclonal lymphoproliferative disorder characterised by systemic inflammation resulting from overproduction of interleukin 6 (IL-6). While iMCD primarily affects the lymph nodes and related tissues, it can also rarely involve the central nervous system. We report the case of a 58-year-old female patient with at least a 3-year history of iMCD, who experienced acute thunderclap headaches due to reversible cerebral vasoconstriction syndrome (RCVS). RCVS occurred 3 months after initiating treatment with tocilizumab, a humanised anti-IL-6 receptor monoclonal antibody, and was accompanied by focal cortical subarachnoid haemorrhage (SAH). Elevated IL-6 levels were found in both serum and cerebrospinal fluid. MR angiography revealed multiple diffuse stenotic lesions in the bilateral middle and posterior cerebral arteries, which, along with bilateral cerebral oedema, resolved within 3 months. The diffuse nature of the cerebral vasospasm and the presence of bilateral brain oedema suggested that cerebral vasospasm was due to RCVS rather than SAH. In patients with Castleman disease, RCVS may occur due to IL-6-dependent chronic cerebral vascular inflammation, either as a primary condition or as a complication of tocilizumab treatment.
2025-02-10 | Beyond Septic Encephalopathy: A Case Report of Severe RCVS and PRES in a Patient With HLH due to Appendicitis.
Background and Objectives: We report a rare case of severe posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) in an adult patient with hemophagocytic lymphohistiocytosis (HLH), and speculate that these three diagnoses are related by similar mechanisms of vascular endothelial dysfunction. Methods: Informed consent for this case report was obtained from the patient's legally authorized surrogate decision maker. Discussion and Practical Implications: Our patient initially presented with HLH secondary to intra-abdominal sepsis, and was later found to have severe PRES and RCVS resulting in extensive border-zone cortex infarction. Improvement of the severe systemic inflammatory syndrome characteristic of HLH and arrest of PRES and RCVS progression occurred only after HLH-specific treatment was initiated. In addition to illustrating the potential of HLH to manifest as PRES and RCVS, this case emphasizes the importance of prompt recognition and treatment of HLH and the role the neurologist can play in this process. This case also sheds light on the pathophysiological links between PRES, RCVS, and HLH. These three diagnoses may be related by similar mechanisms of vascular endothelial dysfunction caused by uncontrolled and severe systemic inflammation.
2024-04-01 | E035 Tocilizumab and the reversible cerebral vasoconstriction syndrome
Abstract Background/Aims Reversible cerebral vasoconstriction syndrome (RCVS) describes a group of conditions characterised by thunderclap headache and radiographic evidence of multifocal cerebral artery vasoconstriction. While typically following a benign course to resolution within a period of days to weeks, a small proportion of patients experience complications such as stroke, seizure or cerebral oedema. While a wide range of aetiological factors have been implicated in the clinical literature, there is, to our knowledge, only one prior publication reporting an association between tocilizumab and RCVS. Methods Here, we describe the case of a patient who developed RCVS following treatment with tocilizumab for rheumatoid arthritis. Results A 41 year old lady presented to her local emergency department after waking from sleep with sudden onset thunderclap headache with associated photophobia and vomiting. There was no prior history of headache. Her past medical history included rheumatoid arthritis and depression. At time of admission, her prescribed medications included folic acid, sertraline, methotrexate and tocilizumab. For the past 5 years, her rheumatoid arthritis had been controlled with a combination of methotrexate and etanercept, but, with her disease flaring, the etanercept was switched for tocilizumab six weeks prior to admission. Initial evaluation with CT head, CT venogram and lumbar puncture did not reveal any underlying abnormalities. A subsequent CT angiogram of the head and neck revealed multiple smooth stenoses bilaterally within the anterior cerebral arteries (ACA), middle cerebral arteries (MCA) and posterior cerebral arteries (PCA). A diagnosis of RCVS was made and tocilizumab was discontinued. Conclusion In patients taking tocilizumab, particular consideration should be given to the possibility of RCVS when performing a diagnostic evaluation of thunderclap headache. Tocilizumab may be an aetiological factor in this condition and its discontinuation may prevent the development of more serious complications. Disclosure B. Corney: None. C. Rhys-Dillon: None.
2021-09-14 | A Case of Reversible Cerebral Vasoconstriction Syndrome: Postpartum Cerebral Angiopathy in a 32-Year-Old Primigravid Treated with Intravenous Immunoglobulin
This is a case of a 32-year-old primigravid who developed sudden severe headache on the 7th day postpartum associated with focal neurologic deficits and altered sensorium. She had a GCS score of 6, anisocoric pupils and an NIHSS score of 31. Cranial MRI with MRA showed multifocal hyperacute to acute infarcts on the left occipital lobe, left thalamus, and midbrain which was more prominent on the right. Due to clinical deterioration, a repeat Cranial MRI with MRA was done and showed progression of infarcts involving both thalami and right pons with interval appearance of contour irregularities in the proximal anterior cerebral, posterior cerebral, basilar and internal carotid arteries. Serial transcranial Doppler showed significant distal right middle cerebral artery vasospasm. She was managed as a case of reversible cerebral vasoconstriction syndrome, associated with postpartum cerebral angiopathy. Intravenous pulse methylprednisolone was started subsequently IVIG was initiated. Intravenous immunoglobulin was given for 5 days. The patient gradually improved, underwent rehabilitation therapy, and was discharged stable after 6 weeks.
cell therapies
2025-11-01 | Abstract 206: A Case of Medically Refractory Reversible Cerebral Vasoconstriction Syndrome Successfully Treated with Plasmapheresis
Objective To describe a case of a treatment refractory cerebral medium vessel arteriopathy, likely representing a severe case of reversible cerebral vasoconstriction syndrome (RCVS). Background RCVS is a cerebrovascular dysregulation syndrome typically characterized by acute, segmental cerebral arterial narrowing. The most common presenting symptom is acute onset thunderclap headache. The term “reversible” refers to reversibility of angiopathic abnormalities; however, the cerebral vasoconstriction is not always reversed. This case highlights a severe case of RCVS with unclear etiology and novel treatment approach. Design/Methods N/A Results Patient is a 55‐year old female who presented to the ED with acute onset of left‐sided numbness. Her initial workup included CT angiography of the neck which demonstrated an unstable thrombus in the distal right common carotid artery and high‐grade stenosis of the right carotid bulb. She was initiated on intravenous heparin for the unstable thrombus in addition to aspirin 81 mg daily. The patient underwent carotid endarterectomy (CEA) with vascular surgery 2 days after admission. She was discharged the following day on aspirin 81 mg daily as antiplatelet monotherapy. She presented again 11 days following discharge with acute onset of left leg weakness and recurrent, acute onset thunderclap headache. CT angiogram head and neck demonstrated multifocal high‐grade stenosis and occlusions along the right MCA and bilateral ACAs, suspicious for RCVS. Pertinent medications at the time of admission included tramadol (recently initiated for new headache) and nicotine patch; the patient denied any additional changes in medications including use of serotonergic agents. The patient was initiated on a non‐dihydropyridine calcium channel blocker with verapamil 40 mg three times daily and discharged 5 days after admission with resolution of her symptoms. The patient presented again to the ED the following day with acute onset of post‐coital left leg weakness. Initial imaging was significant for interval worsening of previously demonstrated vasospasms of the right MCA and bilateral ACAs. The patient was ultimately admitted for over one month, with multiple treatments attempted based on the refractory nature of her disease. With concern for an autoimmune component to her focal arteriopathy, considered potentially secondary to a hypersensitivity reaction to the bovine graft in CEA, she was initiated on intravenous methylprednisolone. Intravenous or oral corticosteroids were continued throughout admission. The patient also underwent multiple angiographies with intraarterial administration of verapamil, milrinone, and nicardipine, with only transient improvement in vasoconstriction following each treatment. Extensive serum and cerebrospinal fluid testing failed to demonstrate a causative etiology for her refractory symptoms. 3 weeks into admission, the patient was initiated on plasmapheresis for five days. Her exam stabilized, and she was successfully discharged to a long term acute care facility. Conclusions Although typically a self‐limited condition, RCVS can be treatment‐refractory and result in irreversible changes in cerebral function. Our case demonstrates a severe, refractory multifocal cerebral vasoconstriction syndrome, resistant to multiple therapies, which ultimately responded to treatment with plasmapheresis.
2019-12-01 | Reversible cerebral vasoconstriction with thunderclap headache
Abstract Rationale: Reversible cerebral vasoconstriction syndrome (RCVS) is often accompanied by thunderclap headaches. Although symptoms usually resolve spontaneously within 2 months, it can cause fatal complications, such as cerebral hemorrhage, and is difficult to differentiate from a migraine and other headaches on the basis of symptoms and Imaging study. In this case report, we explore clinical findings and appropriate treatment methods for RCVS through the case study of a female patient who experienced severe headache upon defecation Patient concerns: A 42-year-old female patient complained of a severe throbbing headache with a Numeric Rating Scale (NRS) score of 10 after defecation. The pain subsided temporarily after treatment with diclofenac 75 mg and Tridol 50 mg propacetamol 1 g, but the headache returned upon defecation; soon after, the patient complained again of regular headaches at 4 to 6-hour intervals irrespective of defecation. Diagnosis: Brain computed tomography (CT) and head and neck magnetic resonance angiography, performed during a headache episode, revealed no specific neurological findings. Blood analysis was also normal. Head and neck CT angiography, performed one month after the start of the headaches, revealed RCVS. Interventions: Treatment commenced with pregabalin (150 mg), oxycodone HCl/naloxone (10/5 mg), Alpram (0.5 mg), milnacipran (25 mg), and frovatriptan 25 mg, but there was no improvement in the headaches. The patient received bilateral trigger point injections (TPI) in the temporal muscles on four occasions at the pain clinic. Outcomes: Medication showed no effect, but after the patient received four sessions of bilateral TPI in the temporal muscles her NRS score eventually decreased from 10 to 2. The patient is currently continuing medication while still experiencing headaches at reduced intensities. Lessons: RCVS is difficult to diagnose; moreover, it is difficult differentiate RCVS from other headaches. However, as it can cause fatal complications, it should not be overlooked. It is essential to consider diagnostic treatment for all types of headaches because RCVS can be accompanied by headaches originating from other causes.
2018-11-16 | Upshaw-Schulman syndrome diagnosed during pregnancy complicated by reversible cerebral vasoconstriction syndrome
Upshaw-Schulman syndrome (USS) is an inherited type of thrombotic thrombocytopenic purpura (TTP) that is extremely rare, but often diagnosed during pregnancy. Reversible cerebral vasoconstriction syndrome (RCVS) is the transient stenosis of several cerebral arteries that is frequently diagnosed post-partum. We describe a 28-year-old woman with USS complicated by RCVS after delivery that was treated by plasma exchange with a good outcome. She was referred to our hospital with thunderclap headache, anemia and thrombocytopenia that occurred immediately postpartum. She was diagnosed with TTP and multiple cerebral infarctions. Plasma exchange promptly improved her symptoms on hospital day 3. Moreover, multiple stenoses of cerebral arteries indicating RCVS were resolved. Since her sister also had an episode of thrombocytopenia during pregnancy, inherited TTP was suspected and genetic analyses confirmed USS. Pregnancy is a risk for not only TTP, but also RCVS. Endothelial damage might be an underlining cause and vasospasm after delivery is a trigger of RCVS. Plasma exchange was effective against both TTP and RCVS.
small molecules
2026-08-01 | Reversible Cerebral Vasoconstriction Syndrome: A Medical Challenge in the Peripartum Period Requiring a Multimodal Approach—A Case Report
Reversible cerebral vasoconstriction syndrome is a rare but potentially life-threatening neurological disorder that may occur during pregnancy and the postpartum period and represents an important diagnostic challenge because of its overlap with other obstetric neurological emergencies. We report the case of a 35-year-old woman with a dichorionic diamniotic twin pregnancy who developed severe preeclampsia requiring cesarean delivery at 30 weeks of gestation. On the fifth postpartum day, she experienced thunderclap headache, generalized seizures, and refractory cerebral vasospasm complicated by subarachnoid hemorrhage, cerebral ischemia, and intracranial hypertension. She was managed in a specialized maternal neurocritical care unit using a multimodal strategy that included continuous neuromonitoring with transcranial Doppler ultrasonography, cerebral oxygenation and metabolic monitoring, electroencephalography, intracranial pressure monitoring, serial cerebral angiography with selective intra-arterial milrinone infusion, and, ultimately, ultrasound-guided stellate ganglion block as rescue therapy. Progressive neurological recovery followed, allowing liberation from mechanical ventilation, initiation of rehabilitation, and hospital discharge with a favorable functional outcome. This case highlights the importance of considering reversible cerebral vasoconstriction syndrome in postpartum women presenting with acute neurological deterioration. Early diagnosis, multidisciplinary management, and multimodal neuromonitoring may facilitate individualized treatment and timely therapeutic escalation in patients with refractory cerebral vasospasm. Further prospective studies are needed to better define the role of advanced neuromonitoring and rescue therapies in this rare condition.
2026-07-03 | Reversible cerebral vasoconstriction syndrome with concomitant recurrent subarachnoid hemorrhage and coronary vasospastic angina: A manifestation of systemic vasoconstriction syndrome
Background: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by thunderclap headache and reversible segmental cerebral arterial constriction. Although neurological complications are well recognized, concurrent extracerebral vasoconstriction remains under appreciated. Case Description: A 53-year-old woman presented with sudden chest tightness immediately followed by a thunderclap headache. Initial brain computed tomography and magnetic resonance imaging showed no hemorrhage or infarction; however, magnetic resonance angiography demonstrated multifocal cerebral arterial narrowing, consistent with RCVS. During hospitalization, she developed recurrent nonaneurysmal subarachnoid hemorrhages (SAHs) on days 2 and 4. No bleeding source was identified on serial angiography. Recurrent episodes of chest tightness prompted coronary angiography with acetylcholine provocation, which revealed severe coronary vasospasm, establishing the diagnosis of vasospastic angina (VSA). Treatment with benidipine and nicorandil was initiated thereafter, and no recurrence of either cardiac or neurological symptoms was observed, with complete resolution of cerebral vasoconstriction on follow-up imaging. Conclusion: This case highlights a rare but clinically important association between RCVS, recurrent nonaneurysmal SAH, and coronary VSA confirmed by pharmacologic provocation. Although each condition is potentially reversible, their coexistence supports the concept of systemic vasoconstriction syndrome and underscores the need for vigilance regarding extracranial vascular involvement in patients with RCVS. Early recognition and targeted vasodilator therapy may be essential to prevent serious neurological and cardiovascular complications.
2026-07-01 | E-292 Diagnosis and management of reversible cerebral vasoconstriction syndrome with a systemic rheumatological disorder: a case and updated review of the literature
Reversible cerebral vasoconstriction syndrome(RCVS) is commonly encountered. Clinical scales, such as RCVS2, and non-invasive cerebrovascular imaging are used to differentiate it from primary CNS angiitis and secondary vasospasm. Patients with inflammatory disorders may have higher exposure to vasoactive triggers and other factors that can precipitate RCVS, which may lead to more complicated clinical courses. We present a patient with a complicated course of RCVS with notable history of a rheumatological disorder. Further, we systematically review the literature for RCVS cases with comorbid rheumatological diagnoses to understand the clinical course and treatment heterogeneity for this population. A 51-year-old female with systemic lupus erythematosus vs undifferentiated connective tissue disorder on mycophenolate mofetil and hydroxychloroquine, presented with worst headache of life accompanied with blurry vision, vomiting and unsteady gait. She experienced two episodes of thunderclap headache the week prior. She also had a history of mechanical aortic valve requiring aspirin and warfarin, implantable cardioverter-defibrillator, hypertension, depression on fluoxetine, and chronic pain on cannabidiol. Examination revealed mild right upper extremity drift. She had supratherapeutic INR (4.58). CT head demonstrated right parafalcine SAH with small bilateral frontoparietal intraparenchymal hemorrhages. CT angiography showed multifocal distal arterial narrowing without aneurysm. CSF and blood analyses were noninflammatory. The RCVS2 score was 10, and a diagnosis of RCVS was favored. Despite normalizing the INR, discontinuation of potential offending agents such as MMF, fluoxetine, and hydroxychloroquine, and treatment with oral nimodipine, she developed a new left hemiparesis with worsening vasoconstriction on repeat imaging. Subsequent digital subtraction angiography confirmed the diffuse distal vasoconstriction. Verapamil 10mg was administered in each ICA resulting in angiographic improvement. She had resolution of weakness and returned to her functional baseline within 2 weeks. Systematic review of the literature identified 16 prior cases (table 1). Rheumatologic conditions included SLE, APLA, lupus nephritis, and others. Presentations included infarcts, PRES, and hemorrhage. Most patients had triggers including steroids, immunosuppressants and vasoactive agents. Patients often got serial imaging and increasing doses of calcium channel blockers for variable durations, alongside removal of triggers. However, IA vasodilator therapy was used infrequently. RCVS is a clinicoradiological syndrome of vasoconstriction, and complicated courses have been described with comorbid rheumatologic disease. While the mainstay treatment of RCVS is removal of offending agents and oral calcium channel blockers, we propose that angiography and intra-arterial vasodilator therapy can offer dual diagnostic as well as therapeutic benefits in severe or refractory cases. F. Kanakhara: None. M. Nahhas: None. R. Regenhardt: None. S. Sheth: None.
2026-06-09 | Idiopathic Intracranial Hypertension Following Non-aneurysmal Spontaneous Subarachnoid Hemorrhage in a Sickle Cell Disease Patient with Suspected Reversible Cerebral Vasoconstriction Syndrome (P5-8.009)
To describe a pediatric patient with sickle cell disease presenting with non-aneurysmal spontaneous subarachnoid hemorrhage (SAH) suspected secondary to Reversible Cerebral Vasoconstriction Syndrome (RCVS), later complicated by Idiopathic Intracranial Hypertension (IIH).
2026-05-01 | A54-28 Toxic Clouds: Methamphetamine Induced Reversible Cerebral Vasoconstriction Syndrome (RCVS), Cardiomyopathy and ARDS Following Laced-Hookah Use
Abstract Introduction Amphetamine use can cause diffuse vasospasm, leading to pulmonary hemorrhage, ARDS, and RCVCS. Early diagnosis and supportive care are crucial. Case Report A 30-year-old male with schizophrenia presented to the ED with cough and dyspnea after smoking flavored tobacco (hookah). He was hypoxic, tachycardic, and in respiratory distress with bibasilar crackles. He was intubated, with blood noted in the endotracheal tube. CT chest showed diffuse bilateral ground-glass opacities without PE. Echo revealed new severe LV hypokinesis (EF 10%), and ECGs showed sinus tachycardia. Drug screening confirmed amphetamines and THC. Ventilation was initiated per ARDS protocol along with nebulized tranexamic acid for hemoptysis. He suffered a brief PEA arrest post-intubation and underwent 24 hours of targeted temperature management (TTM). Neuroimaging revealed a small subarachnoid hemorrhage, cerebellar infarct, and multifocal diffuse intracranial vasospasm. Cerebral angiography confirmed reversible cerebral vasoconstriction syndrome (RCVS), treated with intra-arterial then oral verapamil. His condition improved, and he was eventually extubated and discharged to psychiatry. At 1-year follow-up, he remains functional and asymptomatic. Discussion Amphetamines can induce excessive catecholamine release, leading to diffuse vasospasm, endothelial injury, vascular permeability and tissue hypoxia across multiple organ systems; The lungs (ARDS, pulmonary hypertension and DAH), heart (arrhythmias, stress cardiomyopathy, vasospastic angina), eyes (transient monocular blindness, retinal occlusion), kidneys (renal tubular injury), and brain (RCVCS, ischemic and hemorrhagic stroke). The condition is rare but increasingly reported in males under 45 with polysubstance use (as in our case). ARDS from Amphetamine induced pulmonary hemorrhage is uncommon, with few reported cases. Reversible Cerebral Vasoconstriction Syndrome (RCVCS) is exceedingly rare (0.2 cases per million annually), characterized by transient narrowing of cerebral arteries. Though more common in females, amphetamine-associated cases are increasingly recognized amongst males. Diagnostic criteria includes severe (thunderclap-type) headaches, seizures, and/or focal deficits following drug exposure, with imaging showing ischemic or hemorrhagic changes. Digital Subtraction Angiography (DSA) is the gold standard, revealing alternating segments of arterial narrowing and dilation (string of beads appearance). The RCVS2 score incorporates clinical and imaging findings to help rule out other intracranial vasculopathies; a score of ≥ 5 is both highly specific and sensitive. Treatment includes calcium channel blockers such as verapamil, with 90% reported recovery and reversibility demonstrated on angiography within 1-3 months (a key feature). Our patient’s outcome underscores the importance of early recognition of drug-induced vasospasm, management within a multidisciplinary team with interventional capability, and the implementation of supportive care to maintain systemic perfusion. This abstract is funded by: None
antibodies
2025-04-30 | Persistent Headache Attributed to Past Reversible Cerebral Vasoconstriction Syndrome Demonstrating Significant Improvement with Fremanezumab
A 45-year-old woman with a history of migraine experienced thunderclap headaches, after which cerebral vasospasm was confirmed by brain magnetic resonance angiography. Nimodipine treatment initially improved the headache, but moderate daily headaches persisted despite vasospasm resolution. Conventional headache medications including topiramate, propranolol and amitriptyline proved ineffective. Treatment with fremanezumab, a calcitonin gene-related peptide (CGRP)-targeted therapy, resulted in significant reduction of headache frequency and intensity after 3 months. This case suggests the potential effectiveness of CGRP inhibition for persistent headaches following reversible cerebral vasoconstriction syndrome.
2025-04-29 | Reversible cerebral vasoconstriction syndrome in idiopathic multicentric Castleman disease under treatment with tocilizumab.
Idiopathic multicentric Castleman disease (iMCD) is a rare polyclonal lymphoproliferative disorder characterised by systemic inflammation resulting from overproduction of interleukin 6 (IL-6). While iMCD primarily affects the lymph nodes and related tissues, it can also rarely involve the central nervous system. We report the case of a 58-year-old female patient with at least a 3-year history of iMCD, who experienced acute thunderclap headaches due to reversible cerebral vasoconstriction syndrome (RCVS). RCVS occurred 3 months after initiating treatment with tocilizumab, a humanised anti-IL-6 receptor monoclonal antibody, and was accompanied by focal cortical subarachnoid haemorrhage (SAH). Elevated IL-6 levels were found in both serum and cerebrospinal fluid. MR angiography revealed multiple diffuse stenotic lesions in the bilateral middle and posterior cerebral arteries, which, along with bilateral cerebral oedema, resolved within 3 months. The diffuse nature of the cerebral vasospasm and the presence of bilateral brain oedema suggested that cerebral vasospasm was due to RCVS rather than SAH. In patients with Castleman disease, RCVS may occur due to IL-6-dependent chronic cerebral vascular inflammation, either as a primary condition or as a complication of tocilizumab treatment.
2025-02-10 | Beyond Septic Encephalopathy: A Case Report of Severe RCVS and PRES in a Patient With HLH due to Appendicitis.
Background and Objectives: We report a rare case of severe posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) in an adult patient with hemophagocytic lymphohistiocytosis (HLH), and speculate that these three diagnoses are related by similar mechanisms of vascular endothelial dysfunction. Methods: Informed consent for this case report was obtained from the patient's legally authorized surrogate decision maker. Discussion and Practical Implications: Our patient initially presented with HLH secondary to intra-abdominal sepsis, and was later found to have severe PRES and RCVS resulting in extensive border-zone cortex infarction. Improvement of the severe systemic inflammatory syndrome characteristic of HLH and arrest of PRES and RCVS progression occurred only after HLH-specific treatment was initiated. In addition to illustrating the potential of HLH to manifest as PRES and RCVS, this case emphasizes the importance of prompt recognition and treatment of HLH and the role the neurologist can play in this process. This case also sheds light on the pathophysiological links between PRES, RCVS, and HLH. These three diagnoses may be related by similar mechanisms of vascular endothelial dysfunction caused by uncontrolled and severe systemic inflammation.
2024-04-01 | E035 Tocilizumab and the reversible cerebral vasoconstriction syndrome
Abstract Background/Aims Reversible cerebral vasoconstriction syndrome (RCVS) describes a group of conditions characterised by thunderclap headache and radiographic evidence of multifocal cerebral artery vasoconstriction. While typically following a benign course to resolution within a period of days to weeks, a small proportion of patients experience complications such as stroke, seizure or cerebral oedema. While a wide range of aetiological factors have been implicated in the clinical literature, there is, to our knowledge, only one prior publication reporting an association between tocilizumab and RCVS. Methods Here, we describe the case of a patient who developed RCVS following treatment with tocilizumab for rheumatoid arthritis. Results A 41 year old lady presented to her local emergency department after waking from sleep with sudden onset thunderclap headache with associated photophobia and vomiting. There was no prior history of headache. Her past medical history included rheumatoid arthritis and depression. At time of admission, her prescribed medications included folic acid, sertraline, methotrexate and tocilizumab. For the past 5 years, her rheumatoid arthritis had been controlled with a combination of methotrexate and etanercept, but, with her disease flaring, the etanercept was switched for tocilizumab six weeks prior to admission. Initial evaluation with CT head, CT venogram and lumbar puncture did not reveal any underlying abnormalities. A subsequent CT angiogram of the head and neck revealed multiple smooth stenoses bilaterally within the anterior cerebral arteries (ACA), middle cerebral arteries (MCA) and posterior cerebral arteries (PCA). A diagnosis of RCVS was made and tocilizumab was discontinued. Conclusion In patients taking tocilizumab, particular consideration should be given to the possibility of RCVS when performing a diagnostic evaluation of thunderclap headache. Tocilizumab may be an aetiological factor in this condition and its discontinuation may prevent the development of more serious complications. Disclosure B. Corney: None. C. Rhys-Dillon: None.
2021-09-14 | A Case of Reversible Cerebral Vasoconstriction Syndrome: Postpartum Cerebral Angiopathy in a 32-Year-Old Primigravid Treated with Intravenous Immunoglobulin
This is a case of a 32-year-old primigravid who developed sudden severe headache on the 7th day postpartum associated with focal neurologic deficits and altered sensorium. She had a GCS score of 6, anisocoric pupils and an NIHSS score of 31. Cranial MRI with MRA showed multifocal hyperacute to acute infarcts on the left occipital lobe, left thalamus, and midbrain which was more prominent on the right. Due to clinical deterioration, a repeat Cranial MRI with MRA was done and showed progression of infarcts involving both thalami and right pons with interval appearance of contour irregularities in the proximal anterior cerebral, posterior cerebral, basilar and internal carotid arteries. Serial transcranial Doppler showed significant distal right middle cerebral artery vasospasm. She was managed as a case of reversible cerebral vasoconstriction syndrome, associated with postpartum cerebral angiopathy. Intravenous pulse methylprednisolone was started subsequently IVIG was initiated. Intravenous immunoglobulin was given for 5 days. The patient gradually improved, underwent rehabilitation therapy, and was discharged stable after 6 weeks.
cell therapies
2025-11-01 | Abstract 206: A Case of Medically Refractory Reversible Cerebral Vasoconstriction Syndrome Successfully Treated with Plasmapheresis
Objective To describe a case of a treatment refractory cerebral medium vessel arteriopathy, likely representing a severe case of reversible cerebral vasoconstriction syndrome (RCVS). Background RCVS is a cerebrovascular dysregulation syndrome typically characterized by acute, segmental cerebral arterial narrowing. The most common presenting symptom is acute onset thunderclap headache. The term “reversible” refers to reversibility of angiopathic abnormalities; however, the cerebral vasoconstriction is not always reversed. This case highlights a severe case of RCVS with unclear etiology and novel treatment approach. Design/Methods N/A Results Patient is a 55‐year old female who presented to the ED with acute onset of left‐sided numbness. Her initial workup included CT angiography of the neck which demonstrated an unstable thrombus in the distal right common carotid artery and high‐grade stenosis of the right carotid bulb. She was initiated on intravenous heparin for the unstable thrombus in addition to aspirin 81 mg daily. The patient underwent carotid endarterectomy (CEA) with vascular surgery 2 days after admission. She was discharged the following day on aspirin 81 mg daily as antiplatelet monotherapy. She presented again 11 days following discharge with acute onset of left leg weakness and recurrent, acute onset thunderclap headache. CT angiogram head and neck demonstrated multifocal high‐grade stenosis and occlusions along the right MCA and bilateral ACAs, suspicious for RCVS. Pertinent medications at the time of admission included tramadol (recently initiated for new headache) and nicotine patch; the patient denied any additional changes in medications including use of serotonergic agents. The patient was initiated on a non‐dihydropyridine calcium channel blocker with verapamil 40 mg three times daily and discharged 5 days after admission with resolution of her symptoms. The patient presented again to the ED the following day with acute onset of post‐coital left leg weakness. Initial imaging was significant for interval worsening of previously demonstrated vasospasms of the right MCA and bilateral ACAs. The patient was ultimately admitted for over one month, with multiple treatments attempted based on the refractory nature of her disease. With concern for an autoimmune component to her focal arteriopathy, considered potentially secondary to a hypersensitivity reaction to the bovine graft in CEA, she was initiated on intravenous methylprednisolone. Intravenous or oral corticosteroids were continued throughout admission. The patient also underwent multiple angiographies with intraarterial administration of verapamil, milrinone, and nicardipine, with only transient improvement in vasoconstriction following each treatment. Extensive serum and cerebrospinal fluid testing failed to demonstrate a causative etiology for her refractory symptoms. 3 weeks into admission, the patient was initiated on plasmapheresis for five days. Her exam stabilized, and she was successfully discharged to a long term acute care facility. Conclusions Although typically a self‐limited condition, RCVS can be treatment‐refractory and result in irreversible changes in cerebral function. Our case demonstrates a severe, refractory multifocal cerebral vasoconstriction syndrome, resistant to multiple therapies, which ultimately responded to treatment with plasmapheresis.
2019-12-01 | Reversible cerebral vasoconstriction with thunderclap headache
Abstract Rationale: Reversible cerebral vasoconstriction syndrome (RCVS) is often accompanied by thunderclap headaches. Although symptoms usually resolve spontaneously within 2 months, it can cause fatal complications, such as cerebral hemorrhage, and is difficult to differentiate from a migraine and other headaches on the basis of symptoms and Imaging study. In this case report, we explore clinical findings and appropriate treatment methods for RCVS through the case study of a female patient who experienced severe headache upon defecation Patient concerns: A 42-year-old female patient complained of a severe throbbing headache with a Numeric Rating Scale (NRS) score of 10 after defecation. The pain subsided temporarily after treatment with diclofenac 75 mg and Tridol 50 mg propacetamol 1 g, but the headache returned upon defecation; soon after, the patient complained again of regular headaches at 4 to 6-hour intervals irrespective of defecation. Diagnosis: Brain computed tomography (CT) and head and neck magnetic resonance angiography, performed during a headache episode, revealed no specific neurological findings. Blood analysis was also normal. Head and neck CT angiography, performed one month after the start of the headaches, revealed RCVS. Interventions: Treatment commenced with pregabalin (150 mg), oxycodone HCl/naloxone (10/5 mg), Alpram (0.5 mg), milnacipran (25 mg), and frovatriptan 25 mg, but there was no improvement in the headaches. The patient received bilateral trigger point injections (TPI) in the temporal muscles on four occasions at the pain clinic. Outcomes: Medication showed no effect, but after the patient received four sessions of bilateral TPI in the temporal muscles her NRS score eventually decreased from 10 to 2. The patient is currently continuing medication while still experiencing headaches at reduced intensities. Lessons: RCVS is difficult to diagnose; moreover, it is difficult differentiate RCVS from other headaches. However, as it can cause fatal complications, it should not be overlooked. It is essential to consider diagnostic treatment for all types of headaches because RCVS can be accompanied by headaches originating from other causes.
2018-11-16 | Upshaw-Schulman syndrome diagnosed during pregnancy complicated by reversible cerebral vasoconstriction syndrome
Upshaw-Schulman syndrome (USS) is an inherited type of thrombotic thrombocytopenic purpura (TTP) that is extremely rare, but often diagnosed during pregnancy. Reversible cerebral vasoconstriction syndrome (RCVS) is the transient stenosis of several cerebral arteries that is frequently diagnosed post-partum. We describe a 28-year-old woman with USS complicated by RCVS after delivery that was treated by plasma exchange with a good outcome. She was referred to our hospital with thunderclap headache, anemia and thrombocytopenia that occurred immediately postpartum. She was diagnosed with TTP and multiple cerebral infarctions. Plasma exchange promptly improved her symptoms on hospital day 3. Moreover, multiple stenoses of cerebral arteries indicating RCVS were resolved. Since her sister also had an episode of thrombocytopenia during pregnancy, inherited TTP was suspected and genetic analyses confirmed USS. Pregnancy is a risk for not only TTP, but also RCVS. Endothelial damage might be an underlining cause and vasospasm after delivery is a trigger of RCVS. Plasma exchange was effective against both TTP and RCVS.
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