Abstract

Background: RCVS is characterized by severe thunderclap headaches often triggered by medications or sexual activity. Convexity subarachnoid hemorrhage, and less commonly, ischemic stroke and intracerebral hemorrhage can occur. There are few studies of the clinical-imaging features of RCVS (Singhal, 2011; Ducros, 2007). The pathogenesis is yet to be understood, and further studies looking into various triggers and outcomes, such as our present one, is essential to characterize the condition and develop targeted treatments. Methods: We performed an EMR search (2012-2023) for patients over 18Y of age with a diagnosis of RCVS in inpatient and outpatient settings. We retrospectively analyzed clinical (demographics, triggers, and discharge mRS)) and imaging features (CTA, MRA, DSA, and transcranial doppler (TCD)) to confirm RCVS diagnosis. RCVS score was calculated. Discharge mRS was inferred from EMR. Diagnosis adjudication was performed by RSM. Student t-test and Chi-square test were used to analyze outcomes. Results: Seventy-nine patients were identified (mean age 40±11.5Y, 91% women). The most common triggers were medications (39%), sexual activity (11.4%), and marijuana use (5%). One-third (35%) had chronic headache history (migraines 22%). Twenty-nine (37%) were postpartum (n=19) or pregnant (n=10). Fourteen had concurrent pre-eclampsia (17%). Twelve (15%) also had imaging features of posterior reversible encephalopathy syndrome (PRES). Vasoconstriction was seen on DSA (n=15), CTA (n=25), and MRA (n=23); In 14 patients, vasoconstriction was evident only on TCD. SAH occurred in 26 (33%), ischemic stroke in 8, and ICH in 9. Median RCVS score was 7 (IQR 6-9, range 4-10). CSF profile (n=19) was normal, aside from mildly elevated lymphocyte count (n=2, range 10-13 cells) or protein (n=5, range 50-80 mg/dL). Discharge mRS was 0-1 in 83%. Patients without RCVS trigger (n= 40) had higher ischemic stroke or hemorrhage occurrence ( χ2 = 3.8, P=0.05 ). RCVS scores in pregnant/postpartum patients were similar to others ( t = 2.01, P = 0.06). Conclusion: In this case series, absence of an RCVS trigger was associated with occurrence of stroke or hemorrhage. Most patients had favorable prognosis. These findings need to be confirmed in prospective studies.

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