Reversible cerebral vasoconstriction syndrome: the importance of follow-up imaging within 2 weeks

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Abstract Background: In patients with thunderclap headache, reversible cerebral vasoconstriction syndrome (RCVS) should be considered as a differential diagnosis. However, RCVS diagnosis in the emergency department (ED) remains challenging. This study described the clinical features of RCVS, determined the factors related to RCVS diagnosis, and suggested treatment strategies for its management. Methods: We retrospectively identified eight patients diagnosed with RCVS and reviewed their medical records. From January 2010 to March 2019, eight patients with RCVS (ages 18-69 years, 5 females) were identified. Results: The median duration from the ED visit to RCVS diagnosis was 6 days (range, 1-11 days). Of the eight patients, seven were middle-aged, six had apparent triggers, six had subarachnoid haemorrhage (SAH), five had high systolic blood pressure, and none had any specific abnormality observed upon physical examination. At the ED visit, RCVS was diagnosed in only one patient with a history of RCVS. In other patients, SAH was diagnosed in two patients, and primary headache was diagnosed in four patients with negative computed tomography (CT) findings. Based on the follow-up imaging, seven of eight patients with convexal SAH were diagnosed as having RCVS (as the cause of SAH) using angiography (e.g., magnetic resonance angiography). Conclusions: RCVS with negative CT findings at the ED visit was likely to be misdiagnosed as primary headache. In patients with thunderclap headache and negative CT findings, physicians should consider RCVS as a differential diagnosis, inform patients of the risk of RCVS and the likelihood of a negative image evaluation early in the course of the disease, and carry out follow-up imaging within 2-weeks of the visit.
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Reversible cerebral vasoconstriction syndrome: the importance of follow-up imaging within 2 weeks | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Original research Reversible cerebral vasoconstriction syndrome: the importance of follow-up imaging within 2 weeks Kiyomitsu Fukaguchi, Tadahiro Goto, Hiroyuki Fukui, Ichiro Sekine, and 1 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-28609/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 01 Jan, 2020 Read the published version in Acute Medicine & Surgery → Version 1 posted You are reading this latest preprint version Abstract Background: In patients with thunderclap headache, reversible cerebral vasoconstriction syndrome (RCVS) should be considered as a differential diagnosis. However, RCVS diagnosis in the emergency department (ED) remains challenging. This study described the clinical features of RCVS, determined the factors related to RCVS diagnosis, and suggested treatment strategies for its management. Methods: We retrospectively identified eight patients diagnosed with RCVS and reviewed their medical records. From January 2010 to March 2019, eight patients with RCVS (ages 18-69 years, 5 females) were identified. Results: The median duration from the ED visit to RCVS diagnosis was 6 days (range, 1-11 days). Of the eight patients, seven were middle-aged, six had apparent triggers, six had subarachnoid haemorrhage (SAH), five had high systolic blood pressure, and none had any specific abnormality observed upon physical examination. At the ED visit, RCVS was diagnosed in only one patient with a history of RCVS. In other patients, SAH was diagnosed in two patients, and primary headache was diagnosed in four patients with negative computed tomography (CT) findings. Based on the follow-up imaging, seven of eight patients with convexal SAH were diagnosed as having RCVS (as the cause of SAH) using angiography (e.g., magnetic resonance angiography). Conclusions: RCVS with negative CT findings at the ED visit was likely to be misdiagnosed as primary headache. In patients with thunderclap headache and negative CT findings, physicians should consider RCVS as a differential diagnosis, inform patients of the risk of RCVS and the likelihood of a negative image evaluation early in the course of the disease, and carry out follow-up imaging within 2-weeks of the visit. Critical Care & Emergency Medicine thunderclap headache reversible cerebral vasoconstriction syndrome emergency department follow-up imaging Introduction Headache is one of the most frequent symptoms among patients presenting at the emergency department (ED). In particular, the sudden intense headache called “thunderclap headache,” is widely known as an important symptom suggesting several critical conditions including aneurysmal subarachnoid haemorrhage (aSAH). While aSAH is a most critical condition that should be differentiated, recent studies have underscored the importance and prevalence of reversible cerebral vasoconstriction syndrome (RCVS) among patients with thunderclap headache [1–3]. RCVS is a relatively new disease concept, defined by Calabrese et al. in 2007 [4–6] and included in the International Classification of Diseases, 10th revisio n (ICD-10). Representative symptoms of RCVS include thunderclap headache, sometimes involving minor bleeding (like SAH), and focal neurological symptoms caused by reversible segmental spasms in the cerebral blood vessel [6]. While RCVS was thought to be a common, benign condition, recent studies have reported that RCVS causes premature stroke and recurrent headache, which could be responsive to interventions [7]. Despite the clinical importance, there are few ED-based clinical studies and no optimal strategy to manage patients with suspected RCVS in the ED [8]. To address the knowledge gap in the literature, we described the clinical features and course of RCVS among eight patients presenting at the ED who were ultimately diagnosed with RCVS. We also determined factors related to RCVS diagnosis, and reported the strategy to manage patients suspected of having RCVS at the ED. Methods This is a case-series using hospital data at one of the largest tertiary care hospital in Japan, with 42,000 annual ED visits and 14,000 emergency transportations. The observation period was from January 2010 to March 2019. We retrospectively identified patients who were diagnosed as RCVS using the ICD-10 code. We then reviewed their medical records to report patient characteristics, medical history (e.g., triggers of headache), clinical features, diagnostic tests performed at the first ED visit, imaging findings at the ED visits and the time of diagnosis, the number of days from the onset of headache to diagnosis, and the number of days from confirmation of vasospasm. This study was approved by the hospital's Institutional Review Board, and the requirement of written informed consent was waived. Results Patient characteristics and clinical features at the ED visit The patient characteristics and clinical features of the eight RCVS cases are summarised in Table 1 . The median age was 55 years (range, 18–69 years), and 5 patients were female. Six patients visited the ED in the summer or autumn. Seven patients visited the ED for thunderclap headache, while one patient visited for headache with syncope. Based on their medical history, varying causes were thought to be triggers of RCVS, including sexual intercourse, beating of drums at a festival, physical or emotional stress (e.g., dietary restriction and school examinations, and the death of a daughter), exertion, and urinary tract infection. Three patients had comorbidity with migraine or chronic headache, and one patient had a history of RCVS. Table 1 Clinical features of eight patients with reversible cerebral vasoconstriction syndrome in the emergency department Case number Age, sex Visit season Chief complaints Potential trigger Comorbidities Pulse rate, per min Blood pressure, mmHg Physical examinations, including neck stiffness Initial imaging findings Initial ED diagnosis Disposition 1 60 F Autumn Thunderclap headache Sexual intercourse Chronic headache 97 159/104 No abnormality No specific CT findings Primary headache (RCVS was not suspected) Home 2 66 F Summer Thunderclap headache, vomiting Beating drums None 75 160/95 No abnormality Finding of cSAH by CT cSAH Hospitalization 3 18 M Autumn Thunderclap headache, vomiting Physical and emotional stress (dietary restriction and school exam) RCVS (2 years ago), migraine 99 95/57 No abnormality No specific CT findings. Peripheral cerebral vasospasm of the left MCA by MRA RCVS Hospitalization 4 51 F Spring Thunderclap headache Unknown Hypertension, cerebral infarction 92 132/84 No abnormality No specific CT and MRI findings Primary headache (RCVS was not suspected) Home 5 58 F Summer Thunderclap headache Emotional stress (death of her daughter) Migraine, insomnia 56 148/76 No abnormality No specific CT findings Primary headache (RCVS was not suspected) Home 6 51 F Winter Thunderclap headache, nausea Exertion (ran in a hurry) Menopausal disorder 72 159/97 No abnormality No specific CT findings Primary headache (RCVS was not suspected) Home 7 65 M Summer Headache, syncope Unknown Diabetes 85 162/89 No abnormality Finding of cSAH by CT cSAH Hospitalization 8 69 M Autumn Thunderclap headache, fever Urinary tract infection Diabetes, chronic kidney disease 92 112/81 No abnormality No specific CT and MRI findings UTI, Primary headache (RCVS was not suspected) Hospitalization Abbreviations: ED, emergency department; F, female; CT, computed tomography; RCVS, reversible cerebral vasoconstriction syndrome; cSAH, convexal subarachnoid haemorrhage; M, male; MRI, magnetic resonance imaging; DSA, digital subtraction angiography; MRA, magnetic resonance angiography; CTA, computed tomography angiography; MCA, middle cerebral artery; PCA, posterior cerebral artery; UTI, urinary tract infection; The mean pulse rate was 84 beats per minute (range, 56–99), the mean systolic blood pressure was 141 mmHg (range, 95–162 mmHg), and the mean diastolic blood pressure was 85 mmHg (range, 57–104 mmHg). All patients had no specific findings (including neck stiffness) at physical and neurological examinations. In all cases, head computed tomography (CT) was taken at the ED visit, but there were no abnormalities observed except in two patients with SAH. In three patients, magnetic resonance imaging (MRI) was further performed, with no specific findings. The initial diagnosis at the ED was that of primary headache in five out of eight patients. In these five patients, there were no medical records suggestive of RCVS, and four patients were discharged home without further instructions on RCVS. In the remaining three patients who were not diagnosed with primary headache, SAH was diagnosed in two, and RCVS was diagnosed in one. The RCVS patient diagnosed at the ED visit was the patient with a history of RCVS; this patient showed vasospasm on MRI. Follow-up and RCVS diagnosis Among the seven patients who were not diagnosed with RCVS at the initial ED visit, follow-up imaging was performed within 2 weeks (range, 2–11 days; Table 2 ). Based on the follow-up imaging, all seven undiagnosed cases revealed SAH with a slight pericortical hematoma as a complication of RCVS. These seven patients were diagnosed with RCVS following further assessment of SAH using angiography. The diagnostic devices for vasospasm were magnetic resonance angiography (MRA), CT angiography (CTA), and digital subtraction angiography (DSA) in five, one, and two patients, respectively. The sites of vasospasm were the middle cerebral artery and the diffuse and multiple vessels in three and five patients, respectively. Improvement in vasospasm was later confirmed in seven patients. One patient died of a reason other than an intracranial disease. Table 2 Clinical courses of eight cases of reversible cerebral vasoconstriction syndrome (RCVS) Case number Days from the initial ED visit to RCVS diagnosis (Days from onset of headache) Diagnostic device Imaging findings and the site of spasm RCVS-related complications Prognosis 1 2 (7) MRA Spasm of the right MCA (M1) Frontal lobe cSAH (MRI) Confirmed the improvement of spasm 3 months later by CTA 2 5 (10) DSA Spasm of the segmental diffuse cerebral artery Parietal lobe cSAH (CT) Confirmed the improvement of spasm 3 months later by MRA 3 1 (1) MRA Spasm of the peripheral cerebral artery of the left MCA (M2) None Confirmed the improvement of spasm 1 month later by MRA 4 9 (9) MRA Spasm of the cerebral artery of the right MCA (M2) Frontal lobe cSAH (MRI) Confirmed the improvement of spasm 6 months later by MRA 5 7 (10) DSA Spasm of both sides of the PCA and right MCA Occipital lobe cSAH (MRI) Confirmed the improvement of spasm 3 months later by MRA 6 6 (6) MRA Spasm of the peripheral cerebral arteries of the fornix and posterior circulation Parietal lobe cSAH (MRI) Confirmed the improvement of spasm 3 months later by MRA 7 11 (13) MRA Spasm of both sides of MCA Left temporal lobe cSAH (CT) Confirmed the improvement of spasm 6 months later by MRA 8 6 (6) CTA Spasm of both sides of PCA Occipital lobe cSAH (CT) Not checked Death Abbreviations: ED, emergency department; cSAH, convexal subarachnoid haemorrhage; CT, computed tomography, MRI, magnetic resonance imaging; DSA, digital subtraction angiography; MRA, magnetic resonance angiography; CTA, computed tomography angiography; MCA, middle cerebral artery; PCA, posterior cerebral artery; UTI, urinary tract infection; M1, M1 segment (Horizontal / Sphenoidal part) of MCA; M2, M2 segment (insular part) of MCA; Discussion From the eight patients in our study, we found two important points for managing patients with suspected RCVS. First, RCVS may likely be misdiagnosed as primary headache in patients with no complications such as SAH. Second, in thunderclap headache with negative CT findings, RCVS should be considered as a differential diagnosis and the patient should be followed-up with imaging (e.g., MRI, MRA) within 2 weeks of the visit. At our hospital, in seven out of eight patients, the condition was not diagnosed as RCVS at the ED visit. Although RCVS diagnosis is obviously difficult at the ED, the ED management of these patients should be an important basis for emergency care. Characteristics and management at the initial ED visit RCVS characteristics in in-hospital or outpatient settings have been reported in several studies [6–7,9]. For example, RCVS typically occurs in the middle-aged population along with thunderclap headache involving nausea/vomiting [7]. Similar to other cardio- or cerebrovascular diseases, a key history may be the presence of triggers. RCVS triggers that have been previously reported include the prescription of vasoactive drugs such as triptans and selective serotonin reuptake inhibitors [9–11], bathing [12], and sexual activity [13]. In our study, the reported characteristics of the eight patients with RCVS are consistent with those described in previous reports and are very similar to those of aSAH – middle-aged patients (n = 7/8) that visited the ED for thunderclap headache with nausea/vomiting (n = 7/8), apparent triggers (n = 6/8), SAH (hypertension, diabetes, migraine) risk (n = 6/8), high systolic blood pressure (≥ 140 mmHg) at the ED visit (n = 5/8), and no specific abnormality during the physical examination (n = 8/8). However, in contrast to the characteristics of patients with aSAH, there were no specific CT findings in six out of eight patients with RCVS at the ED visit. These results indicate the difficulty in diagnosing RCVS based on the present illness, vital signs, and physical findings, although the apparent triggers may be somewhat beneficial. Therefore, in the case of thunderclap headache with negative CT findings, physicians should consider RCVS as a differential diagnosis, inform the patient of the potential risk of RCVS, and record the potential diagnosis in the patients’ medical chart for further review at a follow-up visit. Indeed, four patients who were discharged to their homes did not receive appropriate follow-up instructions. The timing of imaging and the diagnosis of RCVS The most important point that makes RCVS diagnosis difficult is the inconsistency in the duration between the onset of headache and the vasospasm imaging findings. RCVS imaging features are often normal in the early stages of severe headache (e.g., at the ED visit). Consistent with observations reported in previous studies [7–10], an average of 8 days was required to confirm vasospasm in this study. Previous studies revealed that vasospasm occurred over time from the peripheral side to the central side of the cerebral artery [7, 14–16], and the appropriate timing of the image evaluation is considered to be 1–2 weeks after the onset of headache [8]. However, by this time, the headache tends to improve [7], and thereafter, the patients no longer visited the hospitals. Complications and prognosis of RCVS While studies have reported that the prognosis of RCVS was generally good, recent studies have demonstrated the risk concurrent with SAH, as well as other complications in patients with RCVS [6–7,9]. Reported complications of RCVS include SAH in 22–34%, cerebral haemorrhage in 6–12%, and cerebral infarction in 4–10% [9,17–18]. These cerebrovascular complications are likely to occur at an early stage [9]. Due to no RCVS-specific findings for the presenting illnesses, or upon physical examination, laboratory testing, and CT imaging, these cerebrovascular complications may be the key to RCVS diagnosis at an early stage. Indeed, in our cases, the SAH complication led to RCVS diagnosis in seven out of eight patients. Conversely, because cerebrovascular complications are reported in 20–30% of RCVS, approximately 70–80% of RCVS patients without complications are likely to be misdiagnosed as having primary headache or other conditions. In our study, there were four patients with slight hematoma that was not detected at CT, and required further imaging using MRI. Although MRI is an expensive test, it may be helpful to distinguish SAH and RCVS from other common, non-life-threatening conditions at the ED, if available. An accurate RCVS diagnosis can improve the patients’ outcomes. Triggers have been reported in about 50% [19] of patients with RCVS, and some of these triggers may be avoidable in the patients’ daily lives. In addition, the recurrence rate of RCVS has been reported at 5% within 5 years [20]. Patients with undiagnosed RCVS are at risk of frequent ED visits and of receiving inappropriate therapy (e.g., triptan for migraine). Therefore, the accurate diagnosis of RCVS may reduce unnecessary ED visits and tests and lead to appropriate therapy. Conclusion Based on eight cases of RCVS presenting at an initial ED visit, there were no specific findings including the presenting illness, vital signs, physical examination, and CT imaging that would allow for diagnosis of RCVS at the ED. RCVS without specific CT findings at the ED visit was likely to be misdiagnosed as primary headache. Since prompt diagnosis of RCVS is difficult at the ED, for patients with thunderclap headache and negative CT findings, physicians should consider RCVS as a differential diagnosis, inform patients of the risk of RCVS, and perform the follow-up imaging (e.g., MRA) within 2-weeks of the ED visit. This practice could facilitate early diagnosis of RCVS, resulting in improved patient outcomes and the reduction of unnecessary ED visits and related resources. Abbreviations aSAH, aneurysmal subarachnoid haemorrhage; cSAH, convexal subarachnoid haemorrhage; CT, computed tomography; CTA, computed tomography angiography; DSA, digital subtraction angiography; ED, emergency department; MCA, middle cerebral artery; MRA, magnetic resonance angiography; MRI, magnetic resonance imaging; PCA, posterior cerebral artery; RCVS, reversible cerebral vasoconstriction syndrome; SAH, subarachnoid haemorrhage; SSRI, selective serotonin reuptake inhibitors; UTI, urinary tract infection Declarations Ethics approval and consent to participate This study was approved by the hospital's Institutional Review Board, and the requirement of written informed consent was waived. Consent for publication Not applicable, as the requirement of written informed consent was waived by the hospital’s Institutional Review Board. Availability of data and materials The datasets used during the current study are available from the corresponding author on reasonable request. Competing interests The authors declare that they have no competing interests. Funding Not applicable. Authors' contributions Kiyomitsu Fukaguchi takes responsibility for the paper as a whole. Kiyomitsu Fukaguchi, Hiroyuki Fukui, Ichiro Sekine, and Hiroshi Yamagami conceived the study. Tadahiro Goto, Hiroyuki Fukui, Ichiro Sekine, and Hiroshi Yamagami supervised the conduct of the study. Kiyomitsu Fukaguchi and Tadahiro Goto drafted the manuscript, and all authors contributed substantially to its revision. Acknowledgements Not applicable. References 〔1〕Edlow JA. Managing Patients With Nontraumatic, Severe, Rapid-Onset Headache. Ann Emerg Med. 2018;71(3):400. 〔2〕Ducros A, Bousser MG. Thunderclap headache. BMJ. 2013;346:e8557. 〔3〕Schwedt TJ. Clinical spectrum of thunderclap headache. Expert Rev Neurother. 2007 Sep;7(9):1135-44. 〔4〕Call GK, Fleming MC, Sealfon S, et al. Reversible cerebral segmental vasoconstriction. Stroke. 1988 ;19:1159–70. 〔5〕Singhal AB, Caviness VS, Begleiter AF, et al. Cerebral vasoconstriction and stroke after use of serotonergic drugs. Neurology. 2002;58(1):130. 〔6〕Calabrese LH, Dodick DW, Schwedt TJ, et al. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med. 2007;146(1):34-44. 〔7〕Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol. 2012;11(10):906-17. 〔8〕Chen SP, Fuh JL, Wang SJ, et al. : Magnetic resonance angiography in reversible cerebral vasoconstriction syndromes. Ann Neurol. 2010;67(5):648-56. 〔9〕Ducros A, Boukobza M, Porcher R, et al. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain. 2007;130:3091–101. 〔10〕Chen SP, Fuh JL, Chang FC, et al. Transcranial color doppler study for reversible cerebral vasoconstriction syndromes. Ann Neurol. 2008;63:751–57. 〔11〕Chen SP, Fuh JL, Wang SJ. Reversible cerebral vasoconstriction syndrome: an under-recognized clinical emergency. Ther Adv Neurol Disord. 2010;3:161–71. 〔12〕Wang SJ, Fuh JL, Wu ZA, et al. Bath-related thunderclap headache: a study of 21 consecutive patients. Cephalalgia. 2008;28:524–30. 〔13〕Hu CM, Lin YJ, Fan YK, et al. Isolated thunderclap headache during sex: Orgasmic headache or reversible cerebral vasoconstriction syndrome? J Clin Neurosci. 2010;17:1349–51. 〔14〕Miller TR, Shivashankar R, Mossa-Basha M, et al. Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical Course. AJNR Am J Neuroradiol. 2015;36:1392-9. 〔15〕Miller TR, Shivashankar R, Mossa-Basha M, et al. Reversible Cerebral Vasoconstriction Syndrome, Part 2: Diagnostic Work-Up, Imaging Evaluation, and Differential Diagnosis. AJNR Am J Neuroradiol. 2015 ;36:1580-8. 〔16〕Shimoda M, Oda S, Hirayama A, et al. Centripetal Propagation of Vasoconstriction at the Time of Headache Resolution in Patients with Reversible Cerebral Vasoconstriction Syndrome. AJNR Am J Neuroradiol. 2016;37:1594-8. 〔17〕Sattar A, Manousakis G, Jensen MB. Systematic review of reversible cerebral vasoconstriction syndrome. Expert Rev Cardiovasc Ther. 2010 ;8:1417–21. 〔18〕Singhal AB, Hajj-Ali RA, Topcuoglu MA, et al. Reversible cerebral vasoconstriction syndromes : analysis of 139 cases. Arch Neuro1. 2011;68:1005-12. 〔19〕Ducros A, Wolff V. The Typical Thunderclap Headache of Reversible Cerebral Vasoconstriction Syndrome and its Various Triggers. Headache 2016;56:657-73. 〔20〕Chen SP, Fuh JL, Lirng JF, et al. Recurrence of reversible cerebral vasoconstriction syndrome : a long-term follow-up study. Neurology. 2015;84:1552-8. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-28609","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Original research","associatedPublications":[],"authors":[{"id":573392,"identity":"dfe355c5-926e-4e38-a1bc-7a4bdd06a39e","order_by":1,"name":"Kiyomitsu Fukaguchi","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA1klEQVRIiWNgGAWjYBAC9gY2IHmAIYEfxEsoIEILzwGoFskGkBYDUrQYHABxidIikZb48ceZ2jzj86sTPzwwYJDnFztAUMthaZ4bx4vNbrzdLAF0mOHM2Qn4tdhLpDdIM3w4lrjtxtkNIC0JBrcJaOGRSG/++QOoZfOMs5t/EKkl7ZgEz42axA38vduItIXnWZo1z5kDiTNu8G6zSDCQIOwXHvY045s/jtUl9vef3XzzR4WNPL80AS1QcJiBQQKsUoIo5SBQx8DAf4Bo1aNgFIyCUTDCAACjc0u2F0Fb3wAAAABJRU5ErkJggg==","orcid":"https://orcid.org/0000-0003-2262-1898","institution":"Shonan Kamakura General Hospital","correspondingAuthor":true,"submittingAuthor":false,"prefix":"","firstName":"Kiyomitsu","middleName":"","lastName":"Fukaguchi","suffix":""},{"id":573393,"identity":"05a0aef7-6b51-4928-b830-b3ca5e60c69e","order_by":2,"name":"Tadahiro Goto","email":"","orcid":"","institution":"Tokyo University","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Tadahiro","middleName":"","lastName":"Goto","suffix":""},{"id":573394,"identity":"0ea3ffc1-3c2d-461b-a7e7-2ab017391ed5","order_by":3,"name":"Hiroyuki Fukui","email":"","orcid":"","institution":"Shonan Kamakura General Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Hiroyuki","middleName":"","lastName":"Fukui","suffix":""},{"id":573395,"identity":"979c887c-bb3a-4a47-9ff2-97525bd9ae78","order_by":4,"name":"Ichiro Sekine","email":"","orcid":"","institution":"Shonan Kamakura General Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Ichiro","middleName":"","lastName":"Sekine","suffix":""},{"id":573396,"identity":"5102a6dd-c6a2-4b07-9b26-23c73b9076f1","order_by":5,"name":"Hiroshi Yamagami","email":"","orcid":"","institution":"Shonam Kamakura General Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Hiroshi","middleName":"","lastName":"Yamagami","suffix":""}],"badges":[],"createdAt":"2020-05-12 12:53:33","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-28609/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-28609/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1002/ams2.559","type":"published","date":"2020-01-01T18:48:42+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":13504762,"identity":"0a9e0b98-245f-4225-9809-02c79faaf16b","added_by":"auto","created_at":"2021-09-16 23:24:28","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":367398,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-28609/v1/c1b8c5b1-7b89-44ae-a037-ffacf6470059.pdf"}],"financialInterests":"","formattedTitle":"Reversible cerebral vasoconstriction syndrome: the importance of follow-up imaging within 2 weeks","fulltext":[{"header":"Introduction","content":" \u003cp\u003eHeadache is one of the most frequent symptoms among patients presenting at the emergency department (ED). In particular, the sudden intense headache called \u0026ldquo;thunderclap headache,\u0026rdquo; is widely known as an important symptom suggesting several critical conditions including aneurysmal subarachnoid haemorrhage (aSAH). While aSAH is a most critical condition that should be differentiated, recent studies have underscored the importance and prevalence of reversible cerebral vasoconstriction syndrome (RCVS) among patients with thunderclap headache [1\u0026ndash;3].\u003c/p\u003e \u003cp\u003eRCVS is a relatively new disease concept, defined by Calabrese et al. in 2007 [4\u0026ndash;6] and included in the \u003cem\u003eInternational Classification of Diseases, 10th revisio\u003c/em\u003en (ICD-10). Representative symptoms of RCVS include thunderclap headache, sometimes involving minor bleeding (like SAH), and focal neurological symptoms caused by reversible segmental spasms in the cerebral blood vessel [6]. While RCVS was thought to be a common, benign condition, recent studies have reported that RCVS causes premature stroke and recurrent headache, which could be responsive to interventions [7]. Despite the clinical importance, there are few ED-based clinical studies and no optimal strategy to manage patients with suspected RCVS in the ED [8].\u003c/p\u003e \u003cp\u003eTo address the knowledge gap in the literature, we described the clinical features and course of RCVS among eight patients presenting at the ED who were ultimately diagnosed with RCVS. We also determined factors related to RCVS diagnosis, and reported the strategy to manage patients suspected of having RCVS at the ED.\u003c/p\u003e "},{"header":"Methods","content":" \u003cp\u003eThis is a case-series using hospital data at one of the largest tertiary care hospital in Japan, with 42,000 annual ED visits and 14,000 emergency transportations. The observation period was from January 2010 to March 2019. We retrospectively identified patients who were diagnosed as RCVS using the ICD-10 code. We then reviewed their medical records to report patient characteristics, medical history (e.g., triggers of headache), clinical features, diagnostic tests performed at the first ED visit, imaging findings at the ED visits and the time of diagnosis, the number of days from the onset of headache to diagnosis, and the number of days from confirmation of vasospasm. This study was approved by the hospital's Institutional Review Board, and the requirement of written informed consent was waived.\u003c/p\u003e "},{"header":"Results","content":" \u003cdiv id=\"Sec4\" class=\"Section2\"\u003e \u003ch2\u003ePatient characteristics and clinical features at the ED visit\u003c/h2\u003e \u003cp\u003eThe patient characteristics and clinical features of the eight RCVS cases are summarised in Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e. The median age was 55\u0026nbsp;years (range, 18\u0026ndash;69\u0026nbsp;years), and 5 patients were female. Six patients visited the ED in the summer or autumn. Seven patients visited the ED for thunderclap headache, while one patient visited for headache with syncope. Based on their medical history, varying causes were thought to be triggers of RCVS, including sexual intercourse, beating of drums at a festival, physical or emotional stress (e.g., dietary restriction and school examinations, and the death of a daughter), exertion, and urinary tract infection. Three patients had comorbidity with migraine or chronic headache, and one patient had a history of RCVS.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eClinical features of eight patients with reversible cerebral vasoconstriction syndrome in the emergency department\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"12\"\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCase number\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eAge, sex\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eVisit season\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eChief complaints\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003ePotential trigger\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c6\"\u003e \u003cp\u003eComorbidities\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c7\"\u003e \u003cp\u003ePulse rate, per min\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c8\"\u003e \u003cp\u003eBlood pressure, mmHg\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c9\"\u003e \u003cp\u003ePhysical examinations, including neck stiffness\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c10\"\u003e \u003cp\u003eInitial imaging findings\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c11\"\u003e \u003cp\u003eInitial ED diagnosis\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c12\"\u003e \u003cp\u003eDisposition\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e60\u003c/p\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eAutumn\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eThunderclap headache\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eSexual intercourse\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eChronic headache\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c7\"\u003e \u003cp\u003e97\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e159/104\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003eNo abnormality\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eNo specific CT findings\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003ePrimary headache\u003c/p\u003e \u003cp\u003e(RCVS was not suspected)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eHome\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e66\u003c/p\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eSummer\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eThunderclap headache, vomiting\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eBeating drums\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eNone\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c7\"\u003e \u003cp\u003e75\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e160/95\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003eNo abnormality\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eFinding of cSAH by CT\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003ecSAH\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eHospitalization\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e18\u0026nbsp;M\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eAutumn\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eThunderclap headache, vomiting\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003ePhysical and emotional stress\u003c/p\u003e \u003cp\u003e(dietary restriction and school exam)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eRCVS\u003c/p\u003e \u003cp\u003e(2\u0026nbsp;years ago), migraine\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c7\"\u003e \u003cp\u003e99\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e95/57\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003eNo abnormality\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eNo specific CT findings. Peripheral cerebral vasospasm of the left MCA by MRA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003eRCVS\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eHospitalization\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e51\u003c/p\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eSpring\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eThunderclap headache\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eUnknown\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eHypertension,\u003c/p\u003e \u003cp\u003ecerebral infarction\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c7\"\u003e \u003cp\u003e92\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e132/84\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003eNo abnormality\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eNo specific CT and MRI findings\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003ePrimary headache\u003c/p\u003e \u003cp\u003e(RCVS was not suspected)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eHome\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e58\u003c/p\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eSummer\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eThunderclap headache\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eEmotional stress\u003c/p\u003e \u003cp\u003e(death of her daughter)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eMigraine,\u003c/p\u003e \u003cp\u003einsomnia\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c7\"\u003e \u003cp\u003e56\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e148/76\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003eNo abnormality\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eNo specific CT findings\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003ePrimary headache\u003c/p\u003e \u003cp\u003e(RCVS was not suspected)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eHome\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e51\u003c/p\u003e \u003cp\u003eF\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eWinter\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eThunderclap headache, nausea\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eExertion (ran in a hurry)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eMenopausal disorder\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c7\"\u003e \u003cp\u003e72\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e159/97\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003eNo abnormality\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eNo specific CT findings\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003ePrimary headache\u003c/p\u003e \u003cp\u003e(RCVS was not suspected)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eHome\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e65\u003c/p\u003e \u003cp\u003eM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eSummer\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eHeadache,\u003c/p\u003e \u003cp\u003esyncope\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eUnknown\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eDiabetes\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c7\"\u003e \u003cp\u003e85\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e162/89\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003eNo abnormality\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eFinding of cSAH by CT\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003ecSAH\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eHospitalization\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e69\u003c/p\u003e \u003cp\u003eM\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eAutumn\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eThunderclap headache, fever\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eUrinary tract infection\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eDiabetes,\u003c/p\u003e \u003cp\u003echronic kidney disease\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"char\" char=\".\" colname=\"c7\"\u003e \u003cp\u003e92\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c8\"\u003e \u003cp\u003e112/81\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c9\"\u003e \u003cp\u003eNo abnormality\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c10\"\u003e \u003cp\u003eNo specific CT and MRI findings\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c11\"\u003e \u003cp\u003eUTI, Primary headache\u003c/p\u003e \u003cp\u003e(RCVS was not suspected)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c12\"\u003e \u003cp\u003eHospitalization\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"12\"\u003eAbbreviations: ED, emergency department; F, female; CT, computed tomography; RCVS, reversible cerebral vasoconstriction syndrome; cSAH, convexal subarachnoid haemorrhage; M, male; MRI, magnetic resonance imaging; DSA, digital subtraction angiography; MRA, magnetic resonance angiography; CTA, computed tomography angiography; MCA, middle cerebral artery; PCA, posterior cerebral artery;\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"12\"\u003eUTI, urinary tract infection;\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003cp\u003eThe mean pulse rate was 84 beats per minute (range, 56\u0026ndash;99), the mean systolic blood pressure was 141\u0026nbsp;mmHg (range, 95\u0026ndash;162\u0026nbsp;mmHg), and the mean diastolic blood pressure was 85\u0026nbsp;mmHg (range, 57\u0026ndash;104\u0026nbsp;mmHg). All patients had no specific findings (including neck stiffness) at physical and neurological examinations. In all cases, head computed tomography (CT) was taken at the ED visit, but there were no abnormalities observed except in two patients with SAH. In three patients, magnetic resonance imaging (MRI) was further performed, with no specific findings.\u003c/p\u003e \u003cp\u003eThe initial diagnosis at the ED was that of primary headache in five out of eight patients. In these five patients, there were no medical records suggestive of RCVS, and four patients were discharged home without further instructions on RCVS. In the remaining three patients who were not diagnosed with primary headache, SAH was diagnosed in two, and RCVS was diagnosed in one. The RCVS patient diagnosed at the ED visit was the patient with a history of RCVS; this patient showed vasospasm on MRI.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec5\" class=\"Section2\"\u003e \u003ch2\u003eFollow-up and RCVS diagnosis\u003c/h2\u003e \u003cp\u003eAmong the seven patients who were not diagnosed with RCVS at the initial ED visit, follow-up imaging was performed within 2 weeks (range, 2\u0026ndash;11 days; Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). Based on the follow-up imaging, all seven undiagnosed cases revealed SAH with a slight pericortical hematoma as a complication of RCVS. These seven patients were diagnosed with RCVS following further assessment of SAH using angiography. The diagnostic devices for vasospasm were magnetic resonance angiography (MRA), CT angiography (CTA), and digital subtraction angiography (DSA) in five, one, and two patients, respectively. The sites of vasospasm were the middle cerebral artery and the diffuse and multiple vessels in three and five patients, respectively. Improvement in vasospasm was later confirmed in seven patients. One patient died of a reason other than an intracranial disease.\u003c/p\u003e \u003cp\u003e \u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab2\" border=\"1\"\u003e \u003ccaption language=\"En\"\u003e \u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e \u003cdiv class=\"CaptionContent\"\u003e \u003cp\u003eClinical courses of eight cases of reversible cerebral vasoconstriction syndrome (RCVS)\u003c/p\u003e \u003c/div\u003e \u003c/caption\u003e \u003ccolgroup cols=\"6\"\u003e \u003cthead\u003e \u003ctr\u003e \u003cth align=\"left\" colname=\"c1\"\u003e \u003cp\u003eCase number\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c2\"\u003e \u003cp\u003eDays from the initial ED visit to RCVS diagnosis\u003c/p\u003e \u003cp\u003e(Days from onset of headache)\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c3\"\u003e \u003cp\u003eDiagnostic device\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c4\"\u003e \u003cp\u003eImaging findings and the site of spasm\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c5\"\u003e \u003cp\u003eRCVS-related complications\u003c/p\u003e \u003c/th\u003e \u003cth align=\"left\" colname=\"c6\"\u003e \u003cp\u003ePrognosis\u003c/p\u003e \u003c/th\u003e \u003c/tr\u003e \u003c/thead\u003e \u003ctbody\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e1\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e2 (7)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eMRA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eSpasm of the right MCA (M1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eFrontal lobe cSAH (MRI)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eConfirmed the improvement of spasm 3 months later by CTA\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e2\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e5 (10)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eDSA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eSpasm of the segmental diffuse cerebral artery\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eParietal lobe cSAH (CT)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eConfirmed the improvement of spasm 3 months later by MRA\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e3\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e1 (1)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eMRA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eSpasm of the peripheral cerebral artery of the left MCA (M2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eNone\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eConfirmed the improvement of spasm 1 month later by MRA\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e4\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e9 (9)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eMRA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eSpasm of the cerebral artery of the right MCA (M2)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eFrontal lobe cSAH (MRI)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eConfirmed the improvement of spasm 6 months later by MRA\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e5\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e7 (10)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eDSA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eSpasm of both sides of the PCA and right MCA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eOccipital lobe\u003c/p\u003e \u003cp\u003ecSAH (MRI)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eConfirmed the improvement of spasm 3 months later by MRA\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e6\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6 (6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eMRA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eSpasm of the peripheral cerebral arteries of the fornix and posterior circulation\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eParietal lobe cSAH (MRI)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eConfirmed the improvement of spasm 3 months later by MRA\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e7\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e11 (13)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eMRA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eSpasm of both sides of MCA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eLeft temporal lobe cSAH (CT)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eConfirmed the improvement of spasm 6 months later by MRA\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003ctr\u003e \u003ctd align=\"left\" colname=\"c1\"\u003e \u003cp\u003e8\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c2\"\u003e \u003cp\u003e6 (6)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c3\"\u003e \u003cp\u003eCTA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c4\"\u003e \u003cp\u003eSpasm of both sides of PCA\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c5\"\u003e \u003cp\u003eOccipital lobe cSAH (CT)\u003c/p\u003e \u003c/td\u003e \u003ctd align=\"left\" colname=\"c6\"\u003e \u003cp\u003eNot checked\u003c/p\u003e \u003cp\u003eDeath\u003c/p\u003e \u003c/td\u003e \u003c/tr\u003e \u003c/tbody\u003e \u003c/colgroup\u003e \u003ctfoot\u003e \u003ctr\u003e\u003ctd colspan=\"6\"\u003eAbbreviations: ED, emergency department; cSAH, convexal subarachnoid haemorrhage; CT, computed tomography, MRI, magnetic resonance imaging; DSA, digital subtraction angiography; MRA, magnetic resonance angiography; CTA, computed tomography angiography; MCA, middle cerebral artery; PCA, posterior cerebral artery;\u003c/td\u003e\u003c/tr\u003e \u003ctr\u003e\u003ctd colspan=\"6\"\u003eUTI, urinary tract infection; M1, M1 segment (Horizontal / Sphenoidal part) of MCA; M2, M2 segment (insular part) of MCA;\u003c/td\u003e\u003c/tr\u003e \u003c/tfoot\u003e \u003c/table\u003e\u003c/div\u003e \u003c/p\u003e \u003c/div\u003e "},{"header":"Discussion","content":" \u003cp\u003eFrom the eight patients in our study, we found two important points for managing patients with suspected RCVS. First, RCVS may likely be misdiagnosed as primary headache in patients with no complications such as SAH. Second, in thunderclap headache with negative CT findings, RCVS should be considered as a differential diagnosis and the patient should be followed-up with imaging (e.g., MRI, MRA) within 2 weeks of the visit. At our hospital, in seven out of eight patients, the condition was not diagnosed as RCVS at the ED visit. Although RCVS diagnosis is obviously difficult at the ED, the ED management of these patients should be an important basis for emergency care.\u003c/p\u003e \u003cdiv id=\"Sec7\" class=\"Section2\"\u003e \u003ch2\u003eCharacteristics and management at the initial ED visit\u003c/h2\u003e \u003cp\u003eRCVS characteristics in in-hospital or outpatient settings have been reported in several studies [6\u0026ndash;7,9]. For example, RCVS typically occurs in the middle-aged population along with thunderclap headache involving nausea/vomiting [7]. Similar to other cardio- or cerebrovascular diseases, a key history may be the presence of triggers. RCVS triggers that have been previously reported include the prescription of vasoactive drugs such as triptans and selective serotonin reuptake inhibitors [9\u0026ndash;11], bathing [12], and sexual activity [13]. In our study, the reported characteristics of the eight patients with RCVS are consistent with those described in previous reports and are very similar to those of aSAH \u0026ndash; middle-aged patients (n\u0026thinsp;=\u0026thinsp;7/8) that visited the ED for thunderclap headache with nausea/vomiting (n\u0026thinsp;=\u0026thinsp;7/8), apparent triggers (n\u0026thinsp;=\u0026thinsp;6/8), SAH (hypertension, diabetes, migraine) risk (n\u0026thinsp;=\u0026thinsp;6/8), high systolic blood pressure (\u0026ge;\u0026thinsp;140\u0026nbsp;mmHg) at the ED visit (n\u0026thinsp;=\u0026thinsp;5/8), and no specific abnormality during the physical examination (n\u0026thinsp;=\u0026thinsp;8/8). However, in contrast to the characteristics of patients with aSAH, there were no specific CT findings in six out of eight patients with RCVS at the ED visit. These results indicate the difficulty in diagnosing RCVS based on the present illness, vital signs, and physical findings, although the apparent triggers may be somewhat beneficial. Therefore, in the case of thunderclap headache with negative CT findings, physicians should consider RCVS as a differential diagnosis, inform the patient of the potential risk of RCVS, and record the potential diagnosis in the patients\u0026rsquo; medical chart for further review at a follow-up visit. Indeed, four patients who were discharged to their homes did not receive appropriate follow-up instructions.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec8\" class=\"Section2\"\u003e \u003ch2\u003eThe timing of imaging and the diagnosis of RCVS\u003c/h2\u003e \u003cp\u003eThe most important point that makes RCVS diagnosis difficult is the inconsistency in the duration between the onset of headache and the vasospasm imaging findings. RCVS imaging features are often normal in the early stages of severe headache (e.g., at the ED visit). Consistent with observations reported in previous studies [7\u0026ndash;10], an average of 8 days was required to confirm vasospasm in this study. Previous studies revealed that vasospasm occurred over time from the peripheral side to the central side of the cerebral artery [7, 14\u0026ndash;16], and the appropriate timing of the image evaluation is considered to be 1\u0026ndash;2 weeks after the onset of headache [8]. However, by this time, the headache tends to improve [7], and thereafter, the patients no longer visited the hospitals.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec9\" class=\"Section2\"\u003e \u003ch2\u003eComplications and prognosis of RCVS\u003c/h2\u003e \u003cp\u003eWhile studies have reported that the prognosis of RCVS was generally good, recent studies have demonstrated the risk concurrent with SAH, as well as other complications in patients with RCVS [6\u0026ndash;7,9]. Reported complications of RCVS include SAH in 22\u0026ndash;34%, cerebral haemorrhage in 6\u0026ndash;12%, and cerebral infarction in 4\u0026ndash;10% [9,17\u0026ndash;18]. These cerebrovascular complications are likely to occur at an early stage [9]. Due to no RCVS-specific findings for the presenting illnesses, or upon physical examination, laboratory testing, and CT imaging, these cerebrovascular complications may be the key to RCVS diagnosis at an early stage. Indeed, in our cases, the SAH complication led to RCVS diagnosis in seven out of eight patients. Conversely, because cerebrovascular complications are reported in 20\u0026ndash;30% of RCVS, approximately 70\u0026ndash;80% of RCVS patients without complications are likely to be misdiagnosed as having primary headache or other conditions.\u003c/p\u003e \u003cp\u003eIn our study, there were four patients with slight hematoma that was not detected at CT, and required further imaging using MRI. Although MRI is an expensive test, it may be helpful to distinguish SAH and RCVS from other common, non-life-threatening conditions at the ED, if available.\u003c/p\u003e \u003cp\u003eAn accurate RCVS diagnosis can improve the patients\u0026rsquo; outcomes. Triggers have been reported in about 50% [19] of patients with RCVS, and some of these triggers may be avoidable in the patients\u0026rsquo; daily lives. In addition, the recurrence rate of RCVS has been reported at 5% within 5\u0026nbsp;years [20]. Patients with undiagnosed RCVS are at risk of frequent ED visits and of receiving inappropriate therapy (e.g., triptan for migraine). Therefore, the accurate diagnosis of RCVS may reduce unnecessary ED visits and tests and lead to appropriate therapy.\u003c/p\u003e \u003c/div\u003e "},{"header":"Conclusion","content":"\u003cp\u003eBased on eight cases of RCVS presenting at an initial ED visit, there were no specific findings including the presenting illness, vital signs, physical examination, and CT imaging that would allow for diagnosis of RCVS at the ED. RCVS without specific CT findings at the ED visit was likely to be misdiagnosed as primary headache. Since prompt diagnosis of RCVS is difficult at the ED, for patients with thunderclap headache and negative CT findings, physicians should consider RCVS as a differential diagnosis, inform patients of the risk of RCVS, and perform the follow-up imaging (e.g., MRA) within 2-weeks of the ED visit. This practice could facilitate early diagnosis of RCVS, resulting in improved patient outcomes and the reduction of unnecessary ED visits and related resources.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cp\u003eaSAH, aneurysmal subarachnoid haemorrhage; cSAH, convexal subarachnoid haemorrhage; CT, computed tomography; CTA, computed tomography angiography; DSA, digital subtraction angiography; ED, emergency department; MCA, middle cerebral artery; MRA, magnetic resonance angiography; MRI, magnetic resonance imaging; PCA, posterior cerebral artery; RCVS, reversible cerebral vasoconstriction syndrome; SAH, subarachnoid haemorrhage; SSRI, selective serotonin reuptake inhibitors; UTI, urinary tract infection\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study was approved by the hospital's Institutional Review Board, and the requirement of written informed consent was waived.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable, as the requirement of written informed consent was waived by the hospital\u0026rsquo;s Institutional Review Board.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe datasets used during the current study are available from the corresponding author on reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that they have no competing interests.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors' contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eKiyomitsu Fukaguchi takes responsibility for the paper as a whole. Kiyomitsu Fukaguchi, Hiroyuki Fukui, Ichiro Sekine, and Hiroshi Yamagami conceived the study. Tadahiro Goto, Hiroyuki Fukui, Ichiro Sekine, and Hiroshi Yamagami supervised the conduct of the study. Kiyomitsu Fukaguchi and Tadahiro Goto drafted the manuscript, and all authors contributed substantially to its revision.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgements\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e"},{"header":"References","content":"\u003cp\u003e〔1〕Edlow JA. Managing Patients With Nontraumatic, Severe, Rapid-Onset Headache. Ann Emerg Med. 2018;71(3):400.\u003c/p\u003e\n\u003cp\u003e〔2〕Ducros A, Bousser MG. Thunderclap headache. BMJ. 2013;346:e8557.\u003c/p\u003e\n\u003cp\u003e〔3〕Schwedt TJ. Clinical spectrum of thunderclap headache. Expert Rev Neurother. 2007 Sep;7(9):1135-44.\u003c/p\u003e\n\u003cp\u003e〔4〕Call GK, Fleming MC, Sealfon S, et al. Reversible cerebral segmental vasoconstriction. Stroke. 1988 ;19:1159\u0026ndash;70.\u003c/p\u003e\n\u003cp\u003e〔5〕Singhal AB, Caviness VS, Begleiter AF, et al. Cerebral vasoconstriction and stroke after use of serotonergic drugs. Neurology. 2002;58(1):130.\u003c/p\u003e\n\u003cp\u003e〔6〕Calabrese LH, Dodick DW, Schwedt TJ, et al. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med. 2007;146(1):34-44.\u003c/p\u003e\n\u003cp\u003e〔7〕Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol. 2012;11(10):906-17.\u003c/p\u003e\n\u003cp\u003e〔8〕Chen SP, Fuh JL, Wang SJ, et al. : Magnetic resonance angiography in reversible cerebral vasoconstriction syndromes. Ann Neurol. 2010;67(5):648-56.\u003c/p\u003e\n\u003cp\u003e〔9〕Ducros A, Boukobza M, Porcher R, et al. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. 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AJNR Am J Neuroradiol. 2015;36:1392-9.\u003c/p\u003e\n\u003cp\u003e〔15〕Miller TR, Shivashankar R, Mossa-Basha M, et al. Reversible Cerebral Vasoconstriction Syndrome, Part 2: Diagnostic Work-Up, Imaging Evaluation, and Differential Diagnosis. AJNR Am J Neuroradiol. 2015 ;36:1580-8.\u003c/p\u003e\n\u003cp\u003e〔16〕Shimoda M, Oda S, Hirayama A, et al. Centripetal Propagation of Vasoconstriction at the Time of Headache Resolution in Patients with Reversible Cerebral Vasoconstriction Syndrome. AJNR Am J Neuroradiol. 2016;37:1594-8.\u003c/p\u003e\n\u003cp\u003e〔17〕Sattar A, Manousakis G, Jensen MB. Systematic review of reversible cerebral vasoconstriction syndrome. Expert Rev Cardiovasc Ther. 2010 ;8:1417\u0026ndash;21.\u003c/p\u003e\n\u003cp\u003e〔18〕Singhal AB, Hajj-Ali RA, Topcuoglu MA, et al. Reversible cerebral vasoconstriction syndromes : analysis of 139 cases. Arch Neuro1. 2011;68:1005-12.\u003c/p\u003e\n\u003cp\u003e〔19〕Ducros A, Wolff V. The Typical Thunderclap Headache of Reversible Cerebral Vasoconstriction Syndrome and its Various Triggers. Headache 2016;56:657-73.\u003c/p\u003e\n\u003cp\u003e〔20〕Chen SP, Fuh JL, Lirng JF, et al. Recurrence of reversible cerebral vasoconstriction syndrome : a long-term follow-up study. Neurology. 2015;84:1552-8.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"thunderclap headache, reversible cerebral vasoconstriction syndrome, emergency department, follow-up imaging","lastPublishedDoi":"10.21203/rs.3.rs-28609/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-28609/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e Background: In patients with thunderclap headache, reversible cerebral vasoconstriction syndrome (RCVS) should be considered as a differential diagnosis. However, RCVS diagnosis in the emergency department (ED) remains challenging. This study described the clinical features of RCVS, determined the factors related to RCVS diagnosis, and suggested treatment strategies for its management. \u003c/p\u003e\u003cp\u003eMethods: We retrospectively identified eight patients diagnosed with RCVS and reviewed their medical records. From January 2010 to March 2019, eight patients with RCVS (ages 18-69 years, 5 females) were identified. \u003c/p\u003e\u003cp\u003eResults: The median duration from the ED visit to RCVS diagnosis was 6 days (range, 1-11 days). Of the eight patients, seven were middle-aged, six had apparent triggers, six had subarachnoid haemorrhage (SAH), five had high systolic blood pressure, and none had any specific abnormality observed upon physical examination. At the ED visit, RCVS was diagnosed in only one patient with a history of RCVS. In other patients, SAH was diagnosed in two patients, and primary headache was diagnosed in four patients with negative computed tomography (CT) findings. Based on the follow-up imaging, seven of eight patients with convexal SAH were diagnosed as having RCVS (as the cause of SAH) using angiography (e.g., magnetic resonance angiography). \u003c/p\u003e\u003cp\u003eConclusions: RCVS with negative CT findings at the ED visit was likely to be misdiagnosed as primary headache. In patients with thunderclap headache and negative CT findings, physicians should consider RCVS as a differential diagnosis, inform patients of the risk of RCVS and the likelihood of a negative image evaluation early in the course of the disease, and carry out follow-up imaging within 2-weeks of the visit.\u003c/p\u003e","manuscriptTitle":"Reversible cerebral vasoconstriction syndrome: the importance of follow-up imaging within 2 weeks","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2020-05-17 20:41:39","doi":"10.21203/rs.3.rs-28609/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"3bd9d33b-3dfe-48e9-bbfe-67dbfadd9ea8","owner":[],"postedDate":"May 17th, 2020","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[{"id":101587,"name":"Critical Care \u0026 Emergency Medicine"}],"tags":[],"updatedAt":"2021-07-22T18:48:42+00:00","versionOfRecord":{"articleIdentity":"rs-28609","link":"https://doi.org/10.1002/ams2.559","journal":{"identity":"acute-medicine-and-surgery","isVorOnly":true,"title":"Acute Medicine \u0026 Surgery"},"publishedOn":"2020-01-01 18:48:42","publishedOnDateReadable":"January 1st, 2020"},"versionCreatedAt":"2020-05-17 20:41:39","video":"","vorDoi":"10.1002/ams2.559","vorDoiUrl":"https://doi.org/10.1002/ams2.559","workflowStages":[]},"version":"v1","identity":"rs-28609","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-28609","identity":"rs-28609","version":["v1"]},"buildId":"-HB7Z8yhvgn0wM9Nzuekk","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}

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