allow a broad range of systolic blood pressure, from 90 to 180 mmHg. We treat hypotension (systolic <90 mmHg) with intravenous fluids, although the threshold of 90 mmHg may be too low if vasoconstriction is severe. High blood pressure (systolic >180 mmHg) can be treated with labetalol or nicardipine. Theoretically, pharmacologically induced hypertension can induce further cerebral vasoconstriction or result in brain hemorrhage and, in the setting of cerebral vasoconstriction, even mild hypotension can trigger ischemic stroke [85]. Pain The pain of RCVS-associated headache is extreme and frequently warrants the use of opioid analgesics in addition to nonsteroidal anti-inflammatory drugs (NSAIDs). In our experience, oral treatment with hydromorphone or acetaminophen-codeine is usually sufficient to manage pain. Thunderclap headaches typically decrease in intensity and frequency over a span of days to weeks. Triptans and the ergot derivatives are contraindicated because of their vasoconstrictive actions [4,78]. Seizures Acute seizures warrant treatment with antiseizure medications, though seizures are usually present only upon presentation and do not recur. Therefore, long-term seizure prophylaxis is probably unnecessary. No seizure prophylaxis is needed for patients who do https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 12/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate not have a seizure. (See "Evaluation and management of the first seizure in adults", section on 'Acute symptomatic seizures' and "Evaluation and management of the first seizure in adults", section on 'When to start antiseizure medication therapy'.) Avoid empiric glucocorticoids We suggest not using empiric glucocorticoid therapy for possible primary angiitis of the central nervous system (PACNS) when RCVS is suspected. However, glucocorticoids are often administered to minimize the risk of delaying treatment in patients who may actually have PACNS, a condition that shares certain features with RCVS (see 'Differential diagnosis' above) and is believed to be progressive and potentially fatal without prompt immunosuppressive therapy. Unfortunately, many patients remain on glucocorticoids for prolonged durations and incur the risk of serious steroid-related adverse effects. There are several reasons to avoid glucocorticoid therapy: Distinguishing RCVS and PACNS in the acute setting is generally straightforward ( table 6 and table 4 and table 5). (See 'Angiographic differential' above.) There is little evidence that a therapeutic delay of a few days would increase the risk for worse outcome in PACNS; even with diagnostically challenging cases, the diagnosis usually becomes apparent after a brief period of observation. Glucocorticoids are associated with worse outcome in RCVS [22,86]. Bedside efforts should focus on distinguishing RCVS from PACNS on the basis of the initial clinical and imaging features and reserve empiric glucocorticoid therapy for the rare patient with a rapidly worsening clinical course while the diagnosis remains uncertain. Vasoconstriction Because clinical and angiographic resolution occur spontaneously without any medical intervention in approximately 90 percent of patients with RCVS, we generally do not use any agent to treat vasoconstriction. In the absence of controlled trials, management of vasoconstriction is guided by observational data and expert opinion. Empiric therapy is not justified for patients who present with thunderclap headache but have not yet undergone vascular imaging. Even when cerebral vasoconstriction has been documented, specific treatment remains undefined. While the literature is replete with various treatment approaches associated with good outcome, these reports probably reflect publication bias. Pharmacologic treatment Calcium channel blockers such as nimodipine and verapamil [87] and brief courses of magnesium sulfate [27,88], serotonin antagonists, and dantrolene https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 13/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate [89] have been administered in an effort to relieve the vasoconstriction. Data from two prospective case series suggest that nimodipine does not affect the time course of cerebral vasoconstriction [16,17]. However, nimodipine might relieve the number and intensity of headaches and has documented effects on the smaller vasculature not easily imaged by angiography. Calcium channel blockers can be discontinued after resolution of symptoms or angiographic abnormalities if they are used. Intra-arterial vasodilation We reserve intra-arterial measures for patients exhibiting clear signs of clinical progression, particularly since over 90 to 95 percent of RCVS patients have a benign, self-limited syndrome despite the presence of severe angiographic vasoconstriction and ischemic or hemorrhagic brain lesions. Unfortunately, no known clinical or imaging features reliably predict disease progression. Balloon angioplasty and direct intra-arterial administration of nicardipine, papaverine, milrinone, and nimodipine have been used with variable success [90-92]. In patients with RCVS, intra-arterial infusion of vasodilators into a single constricted artery can promptly reverse vasoconstriction in that artery and often in multiple brain arteries, including the contralateral arteries. A similar but milder response has rarely been observed in RCVS mimics such as PACNS and intracranial atherosclerosis. On this basis, the demonstration of arterial dilatation using intra-arterial vasodilator infusions has been proposed as a "diagnostic test" for RCVS [93]. However, intra-arterial interventions carry a risk for reperfusion injury. Prevention and counseling In the acute setting, it is logical to avoid further exposure to any potential precipitating factors, such as marijuana, cocaine, exercise stimulants, amphetamines and triptans, serotonergic antidepressants, or other vasoconstrictive medications that can worsen the clinical course. Patients should avoid physical exertion, sexual activity, the Valsalva maneuver, and other known triggers of recurrent headaches for a few weeks. Laxatives and stool softeners should be used to avoid constipation (which can trigger the Valsalva maneuver), especially in patients receiving opioids for head pain. The risk of recurrent RCVS is low; hence, reexposure to the potential precipitating factor (eg, antidepressants) can be considered if clinically necessary and after other therapeutic options are exhausted. Usual secondary stroke preventive medications, such as antiplatelet agents, anticoagulants, and cholesterol-lowering agents, are probably not indicated. There are no known genetic implications of RCVS. https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 14/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate CLINICAL COURSE AND PROGNOSIS The resolution of the different components of RCVS, including headaches, focal deficits, and angiographic narrowing, does not always follow the same time course. The thunderclap headaches typically resolve over days to weeks. Similarly, most patients show resolution of visual and other focal neurologic signs and symptoms within days to weeks. Less than 15 to 20 percent are left with residual deficits from stroke, and in most cases the deficits are relatively minor or moderate (ie, 90 to 95 percent have a modified Rankin scale score ( table 9) of 0 to 2 at discharge) [94]. Progressive cerebral arterial vasoconstriction culminating in massive strokes, brain edema, severe morbidity, or death occurs in less than five percent of cases, and these fulminant cases have been more commonly reported in postpartum patients [58,95-97]. Predictors of poor outcome in retrospective studies include baseline clinical factors (advanced age, comorbid medical conditions), severe imaging findings (infarction, intracerebral hemorrhage, cerebral edema), and exposure to glucocorticoid therapy [39,86]. Some patients go on to have intractable chronic migraine-like headaches or depression [94]. The time course of vasoconstriction is variable, but most patients show resolution within three months. Note that "reversible" in the term RCVS refers to the dynamic and reversible nature of vasoconstriction; clinical deficits from brain damage might persist, and the vasoconstriction (particularly if severe and prolonged) may not fully reverse in rare cases. Recurrence of an episode of RCVS after resolution of the initial symptomatic period is uncommon, approximately 5 to 6 percent in two studies [98,99], and usually manifests as an isolated thunderclap headache without complications such as stroke [98,100]. SUMMARY AND RECOMMENDATIONS Definition and epidemiology Reversible cerebral vasoconstriction syndrome (RCVS) represents a group of conditions (including Call-Fleming syndrome, benign angiopathy of the central nervous system, and postpartum angiopathy) characterized by reversible narrowing and dilatation of the cerebral arteries. The mean age of onset of RCVS is approximately 42 years. In adults, RCVS affects females more often than males. (See 'Terminology' above and 'Epidemiology' above.) Clinical presentation and risk factors The clinical presentation of RCVS is usually dramatic with sudden, severe thunderclap headaches that often recur over a span of days https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 15/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate to weeks. Some patients develop seizures or focal neurologic deficits. (See 'Clinical presentation and course' above.) Many patients have triggering factors, such as orgasm, physical exertion, acute stressful or emotional situations, Valsalva maneuvers, bathing, or swimming. A variety of conditions have been associated with RCVS including exposure to vasoconstrictive drugs and medications, other headache disorders, and recent pregnancy ( table 1). (See 'Risk factors and associated conditions' above.) Neuroimaging features Cerebral angiographic abnormalities of RCVS are dynamic and progress proximally, resulting in a "sausage on a string" appearance of the circle of Willis arteries and their branches. These abnormalities resolve spontaneously over a few weeks. (See 'Neurovascular imaging' above.) Magnetic resonance imaging (MRI) of the brain is normal in over 50 percent of patients with RCVS. In the ensuing days, many patients may develop complications such as ischemic stroke, convexity (nonaneurysmal) subarachnoid hemorrhage, lobar hemorrhage, and reversible brain edema, alone or in combination. (See 'Brain imaging' above.) Diagnostic evaluation Patients who present with thunderclap headache must be evaluated as a medical emergency, beginning with cranial computed tomography (CT) or brain MRI and head and neck CT angiography (CTA) or magnetic resonance angiography (MRA). If imaging is normal, lumbar puncture and cerebrospinal fluid analysis is appropriate to exclude other causes such as subarachnoid hemorrhage. (See 'Urgent evaluation' above.) The diagnosis of RCVS is based upon the characteristic clinical, brain imaging, and angiographic features, as summarized in the tables ( table 3 and table 6 and table 4 and table 5). (See 'Diagnosis' above.) Differential diagnosis The individual clinical and imaging features if RCVS carry a wide range of differential diagnoses, particularly aneurysmal subarachnoid hemorrhage, other conditions associated with thunderclap headache, and intracranial arteriopathies including intracranial atherosclerosis, primary angiitis of the central nervous system, moyamoya disease, and fibromuscular dysplasia. (See 'Differential diagnosis' above.) Management There is no proven therapy for RCVS. Supportive care is directed toward managing blood pressure, severe headaches, and other complications such as seizures. We generally do not use calcium channel blockers or other agents to treat vasoconstriction, as https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 16/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate evidence for this strategy is lacking. Intra-arterial vasodilator therapy has been attempted in fulminant cases with variable success. (See 'Management' above.) Prognosis The clinical outcome is benign in 90 to 95 percent of patients. Rare patients develop severe irreversible deficits or death from progressive strokes or cerebral edema. Recurrence of an episode of RCVS is rare. (See 'Clinical course and prognosis' above.) Use of UpToDate is subject to the Terms of Use. REFERENCES 1. Serdaru M, Chiras J, Cujas M, Lhermitte F. Isolated benign cerebral vasculitis or migrainous vasospasm? J Neurol Neurosurg Psychiatry 1984; 47:73. 2. Jackson M, Lennox G, Jaspan T, Jefferson D. 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Topic 14075 Version 19.0 https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 23/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate GRAPHICS Risk factors, triggers, and other conditions associated with reversible cerebral vasoconstriction syndrome Changes in estrogen-progesterone levels Pregnancy Eclampsia-preeclampsia Ovarian stimulation Oral contraceptive medications Headache disorders Primary thunderclap headache Primary cough headache Primary headache associated with sexual activity Exercise (exertional) headache Migraine Vasoconstrictive agents Antimigraine agents (triptans, isometheptene, ergotamine tartrate) Blood products (red blood cell transfusions, erythropoietin) Cough and cold suppressants (phenylpropanolamine, pseudoephedrine) Diet pills and energy-enhancing agents (amphetamine derivatives, Hydroxycut) Antidepressants (selective serotonin reuptake inhibitors and serotonin-noradrenaline reuptake inhibitors) Adrenergic agents (epinephrine, bromocriptine, lisuride) Illicit drugs (cocaine, ecstasy, marijuana, lysergic acid diethylamide) Chemotherapeutic agents (tacrolimus, cyclophosphamide) Other (indomethacin, interferon alpha, intravenous immune globulin, licorice, ma huang [ephedra], methylergonovine, nicotine patches) Tumors Carcinoid, carotid paraganglioma, pheochromocytoma Metabolic Hypercalcemia, porphyria https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 24/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate Environmental exposure or trauma High altitude Cold water exposure Swimming Head trauma Vascular Cerebral venous thrombosis Cervical artery dissection Postcarotid endarterectomy Posterior reversible encephalopathy syndrome (PRES) Neurosurgical manipulation of intracerebral arteries Spinal subdural hematoma Unruptured saccular cerebral aneurysm Adapted from: Singhal AB, Bernstein RA. Postpartum angiopathy and other cerebral vasoconstriction syndromes. Neurocrit Care 2005; 3:91. Graphic 101277 Version 4.0 https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 25/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate Etiologies of thunderclap headache Most common causes of thunderclap headache: Subarachnoid hemorrhage Reversible cerebral vasoconstriction syndromes (RCVS) Conditions that less commonly cause thunderclap headache: Cerebral infection (eg, meningitis, acute complicated sinusitis) Cerebral venous thrombosis Cervical artery dissection Spontaneous intracranial hypotension Acute hypertensive crisis Posterior reversible leukoencephalopathy syndrome (PRES) Intracerebral hemorrhage Ischemic stroke Conditions that uncommonly or rarely cause thunderclap headache: Pituitary apoplexy Colloid cyst of the third ventricle Aortic arch dissection Aqueductal stenosis Brain tumor Giant cell arteritis Pheochromocytoma Pneumocephalus Retroclival hematoma Spinal epidural hematoma Varicella zoster virus vasculopathy Vogt-Koyanagi-Harada syndrome Disputed causes of thunderclap headache: Sentinel headache (unruptured intracranial aneurysm)* Primary thunderclap headache Sentinel headache due to an unruptured intracranial aneurysm is a possible cause of thunderclap headache, but supporting data are weak. https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 26/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate There is controversy as to whether thunderclap headache can occur as a benign and potentially recurrent headache disorder in the absence of underlying organic intracranial pathology. Graphic 81710 Version 8.0 https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 27/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate Typical neuroimaging features of reversible cerebral vasoconstriction syndrome (A) Head CT angiogram: sagittal, maximum-intensity projection image showing the classic "sausage on a string" appearance of both anterior cerebral arteries. (B) Head CT: axial image showing subarachnoid hemorrhage overlying the right frontal lobe (arrow). (C) Brain MRI: axial FLAIR image in the same patient showing the right frontal subarachnoid hemorrhage (arrow) as well as multiple dot-shaped hyperintensities (arrowhead) within the sulci of both hemispheres, suggesting the presence of dilated cortical surface arteries. (D) Brain MRI: axial FLAIR image showing the posterior-predominant crescentic hyperintense signal in the cortical-subcortical regions (dashed arrow). Corresponding DWI and SWI (not shown) were normal. These findings suggest the presence of brain edema as described in the posterior reversible leukoencephalopathy syndrome. (E) Brain MRI: axial DWI showing ischemic lesions (short arrows) in the bilateral "watershed" regions of the middle and posterior cerebral arteries. (F) Head CT scan: axial image showing a left frontal parenchymal hemorrhage. https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 28/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate RCVS: reversible cerebral vasoconstriction syndrome; FLAIR: fluid-attenuated inversion recovery; DWI: diffusion-weighted image; SWI: susceptibility-weighted image. Reproduced with permission from: Singhal AB, Hajj-Ali RA, Topcuoglu MA, et al. Reversible cerebral vasoconstriction syndromes: Analysis of 139 cases. Arch Neurol 2011; 68:1005. Copyright 2011 American Medical Association. All rights reserved. Graphic 101799 Version 9.0 https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 29/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate Brain lesions in reversible cerebral vasoconstriction syndrome Representative brain images from patients with RCVS are shown to highlight different lesion patterns. The numbers in parenthesis show the percentages of the lesion patterns; totals exceed 100% due to lesion combinations. (A) No acute parenchymal lesion (24%). Normal axial DWI, GRE, and FLAIR images are shown. The hyperintense dot sign is present on FLAIR (far right, arrow). (B) Border zone/watershed infarcts (25%). On the far left, DWI shows typical symmetric, posterior infarcts that spare the cortical ribbon. In https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 30/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate the middle and on the far right, DWI shows widespread watershed infarcts. (C) Vasogenic edema (28%). Subcortical crescent-shaped T2- hyperintense lesions consistent with the posterior reversible encephalopathy syndrome are seen on FLAIR. (D) Hemorrhagic lesions (42%). The two images on the left (axial GRE) show simultaneous lobar and deep intraparenchymal hemorrhages. The two images on the right show convexal subarachnoid hemorrhages on CT and axial GRE. (E) Lesion combinations (28%). The two images on the left show bilateral watershed infarcts on DWI and the two images on the right show lobar as well as convexal subarachnoid hemorrhages on axial FLAIR and CT, all in the same patient. RCVS: reversible cerebral vasoconstriction syndrome; DWI: diffusion- weighted images; GRE: gradient-echo; FLAIR: fluid-attenuated inversion recovery; CT: computed tomography. From: Singhal AB, Topcuoglu MA, Fok JW, et al. Reversible cerebral vasoconstriction syndromes and primary angiitis of the central nervous system: clinical, imaging, and angiographic comparison. Ann Neurol 2016; 79:882. http://onlinelibrary.wiley.com/wol1/doi/10.1002/ana.24652/abstract. Copyright 2016 American Neurological Association. Reproduced with permission of John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's permissions department either via email: permissions@wiley.com or use the RightsLink service by clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (http://onlinelibrary.wiley.com). Graphic 109962 Version 2.0 https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 31/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate Convexal subarachnoid hemorrhage in a patient with reversible cerebral vasoconstriction syndrome (A) CT scan of a 56-year-old man with severe headaches showing presence of superficial convexal SAH along bilateral posterior frontal lobes (arrows). (B) Conventional angiogram performed a day later shows multiple vessel narrowings (arrows) involving the branches of the posterior and anterior cerebral arteries. A repeat angiogram performed four weeks later showed restitution of normal arterial caliber (not shown). SAH: subarachnoid hemorrhage. From: Kumar S, Goddeau RP Jr, Selim MH, et al. Atraumatic convexal subarachnoid hemorrhage: Clinical presentation, imaging patterns, and etiologies. Neurology 2010; 74:893. Copyright 2010 American Academy of Neurology. Reproduced with permission from Wolters Kluwer Health. Unauthorized reproduction of this material is prohibited. Graphic 101281 Version 5.0 https://www.uptodate.com/contents/reversible-cerebral-vasoconstriction-syndrome/print 32/46 7/6/23, 12:35 PM Reversible cerebral vasoconstriction syndrome - UpToDate Resolution of vasoconstriction in a patient with reversible cerebral vasoconstriction syndrome