The Spectrum of Nontraumatic Convexity Subarachnoid Hemorrhage

Background: Nontraumatic convexity subarachnoid hemorrhage (cSAH) is a nonaneurysmal variant that is associated with diverse etiologies. Methods: With IRB approval, we retrospectively reviewed consecutive nontraumatic cSAH from July 1, 2006 to July 1, 2016. Data were abstracted on demographics, medical history, neuroimaging, etiology, and clinical presentation. Results: We identified 94 cases of cSAH. The cases were classified according to the following etiologies: reversible cerebral vasoconstriction syndrome (RCVS) 17 (18%), cerebral amyloid angiopathy (CAA) 15 (16%), posterior reversible encephalopathy syndrome 16 (17%), cerebral venous thrombosis 10 (11%), large artery occlusion 7 (7%), endocarditis 6 (6%), and cryptogenic 25 (27%). Early rebleeding occurred in 9 (10%) patients. Time from initial imaging to CT rebleeding was 40 hours (range, 5-74). CAA was associated with the highest mean age at 75.8 and RCVS the lowest at 47.6 years (P< .0001). Among patients with RCVS, initial vascular imaging was negative in 6 (35%), and repeat imaging documented vasoconstriction at a mean delay of 5 days (range, 3-16). Conclusion: There were significant differences among the subgroups in cSAH, with CAA presenting as older men with transient neurological deficits, and RCVS presenting as younger women with thunderclap headache. Rebleeding was seen in 10% of cSAH patients. One-third of RCVS patients with cSAH required repeat vascular imaging to diagnose vasoconstriction.     

Reversible cerebral vasoconstriction syndrome combined with posterior reversible encephalopathy syndrome after heart transplantation

Reversible cerebral vasoconstriction syndrome (RCVS) combined with posterior reversible encephalopathy syndrome (PRES) is a rare complication in patients treated with immunosuppressants. A 52-year-old male patient presented with seizures after heart transplantation. The patient was suspected of having PRES on brain images. Despite the strict blood pressure control, the patient presented with altered mentality and the brain images showed a newly developed large acute infarction. Digital subtraction angiography (DSA) revealed the classic “sausage on a string” appearance of the cerebral arteries – potential feature of RCVS. To our knowledge, this is the first case report to describe RCVS combined with PRES after heart transplantation.     

A pediatric case of reversible cerebral vasoconstriction syndrome with cortical subarachnoid hemorrhage

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare disorder characterized by acute onset, severe headache, with reversible vasoconstriction of cerebral arteries often accompanied by additional neurological symptoms. This syndrome is seen mainly in middle-aged adults, predominantly women. Herein, we report on a pediatric case of RCVS with cortical subarachnoid hemorrhage (SAH). A 12-year-old boy developed acute, severe headache with paralysis of lower extremities causing gait disturbance after administration of eletriptan. Brain magnetic resonance angiography (MRA) revealed multifocal narrowing of the cerebral arteries, whereas magnetic resonance imaging (MRI) demonstrated sulcal hyperintensity on fluid-attenuated inversion recovery, consistent with cortical SAH. The patient's clinical symptoms resolved spontaneously after a few days and the MRI and MRA findings disappeared 3months later, suggesting a diagnosis of RCVS. Eletriptan might cause vasoconstriction of cerebral arteries. Although most patients with RCVS are adults and pediatric cases are rare, RCVS should be considered in a child complaining of severe headache.     

PRES and RCVS: Two Distinct Entities or a Spectrum of the Same Disease?

ObjectivesTo report a case of a patient with overlapping posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS), and review the existing literature emphasizing the pathophysiological overlap of these two entities.Materials and MethodsWe conducted a literature search in electronic database PubMed identifying studies reporting the overlap of PRES and RCVS.ResultsPRES and RCVS are two increasingly recognized entities that share similar clinical and imaging features. PRES is characterized by vasogenic edema predominantly in the parieto-occipital regions, associated with acute onset of neurological symptoms including encephalopathy, seizures, headaches, and visual disturbances. RCVS is characterized by reversible segmental and multifocal vasoconstriction of the cerebral arteries and classically presents with thunderclap headache, with or without associated focal neurological deficits and seizures. PRES is frequently associated with uncontrolled hypertension but can also be seen in the setting of renal failure, exposure to cytotoxic agents, or pre-eclampsia. RCVS is often triggered by exposure to vasoactive agents, postpartum state, or immunosuppression. We report a case of a patient presenting with vision changes and hemiparesis, and found to have extensive cytotoxic and vasogenic edema involving the cortex and subcortical white matter on brain imaging. These changes were primarily noted in the parieto-occipital and brainstem regions, along with features of reversible vasculopathy on vascular imaging suggestive of coexisting PRES and RCVS.ConclusionsPRES and RCVS share precipitating factors, clinical and radiological features, and frequently co-exist, suggesting a common pathophysiological mechanism related to reversible dysregulation of cerebral vasculature, endothelial dysfunction, and breakdown of the blood-brain barrier.     

可复性后部脑病综合征的影像学诊断

目的探讨可复性后部脑病综合征（PRES）的影像学表现.方法回顾性分析了12例PRES病人的临床和影像学资料,其中9例为子痫/先兆子痫,2例为高血压脑病,1例为环孢菌素A（CSA）的神经毒性.12例均行MRI检查,其中7例同时行钆喷替酸葡甲胺（Gd-DTPA）增强扫描,4例行磁共振血管造影（3D-TOF MRA）检查,1例行弥散加权成像（DWI）.7例行CT平扫检查,2例行脑血管造影（DSA）检查.结果MRI显示病灶基本上呈双侧对称性分布,多数病灶位于顶、枕叶脑实质内,T1WI呈等或略低信号,T2WI呈高信号,FLAIR像显示皮层和皮层下白质明显高信号影,较T1WI、T2WI更加清楚.注射Gd-DTPA后多无明显异常对比增强.1例DWI显示双侧顶、枕叶及额叶皮层内弥散受限呈高信号,ADC图显示邻近的皮层下白质呈高信号.4例CT显示双侧顶、枕叶及额叶对称性斑片状低密度影,3例CT未见异常.经对症处理后复查示所有病灶几乎完全吸收消失.结论PRES的影像学表现具有特征性.MRI应作为诊断本病的首选手段.     

Is hypertension predictive of clinical recurrence in posterior reversible encephalopathy syndrome?

Posterior reversible encephalopathy syndrome (PRES) has a distinctive clinical presentation and typical neuroimaging findings. However, data on its clinical course and recurrence are scarce. This study aims to investigate its clinical profile and factors that predict recurrence. We included patients diagnosed with PRES between 2005 and 2010 and collected data on demographics, presenting symptoms, co-morbidities, risk factors, clinical parameters, MRI findings, complications and recurrence. Patients were categorized into two groups: PRES due to primary hypertension and PRES due to secondary causes. Correlation with presenting symptoms, radiological features, and recurrence were analyzed. PRES was identified in 28 patients. Fourteen (50%) had primary hypertension. Secondary causes included immunosuppression-related (39%), preeclampsia/eclampsia (7%), and marijuana-intake-related (4%) causes. Patients presented with altered mental status (79%), headache (75%), seizure (68%), visual disturbance (39%) and hemiparesis (21%). On MRI 93% had the typical parietal–occipital involvement. The frontal lobe was affected in 64%, cerebellum in 29%, brainstem in 21%, and basal ganglia in 11%. About 36% had cortical involvement; 21% had diffusion-restricted lesions. Non-aneurysmal subarachnoid haemorrhage was found in 18% of patients and intracerebral hemorrhage in 14% of patients. No significant difference existed in presenting symptoms and the MRI distribution of vasogenic edema between the primary hypertension group and the secondary causes group. Recurrence occurred in four patients (14.3%, 95% confidence interval 4.2–33.7) and was significantly associated (p = 0.05) with primary hypertension as the etiology. Intensive monitoring and treatment of hypertension is recommended for reducing morbidity.     

Reversible cerebral vasoconstriction syndrome in association with fingolimod use

Background: Reversible cerebral vasoconstriction syndrome (RCVS), also known as Call-Fleming syndrome, is characterized by thunderclap headaches, non-aneurysmal segmental cerebral vasoconstriction seen on arteriogram, and spontaneously resolves within 12 weeks. Fingolimod has been reported to cause posterior reversible encephalopathy syndrome (PRES) and one case of RCVS. Objective: We report a case of RCVS possibly related to fingolimod use, and compare to cases of adverse outcomes in fingolimod use. Methods: This is a single observational study without controls; therefore, level of evidence is IV. A literature review in pubmed with keywords, fingolimod, vasospasm, RCVS, Call-Fleming, stroke, PRES and hemorrhage. Results: One case of RCVS on fingolimod in the post-partum period. Two other cases in the literature were found one with hemorrhagic encephalitis and the other critical vasospasm in the upper extremity associated with fingolimod 1.25 mg daily in the FREEDOMS II trial and TRANSFORMS study, respectively. Additionally, Novartis reports nine cases of PRES related to fingolimod use. Conclusion: Fingolimod has the potential to cause vasoconstriction however appears to be rare and more likely on doses higher than 0.5 mg daily. Fingolimod may be associated in RCVS and should be considered in patients with severe headache on fingolimod.     

可逆性脑血管收缩综合征临床及影像学特点

目的探讨可逆性脑血管收缩综合征(RCVS)的临床、影像学特点,以提高对该病的认识。方法总结3例RCVS患者的临床及影像学资料并复习相关文献。结果本组3例患者均以剧烈头痛为首发症状,其中1例伴有双下肢无力,1例伴视物模糊、右侧肢体轻偏瘫。TCD检查均提示不同血管不同程度的血流速度增快,头颅MRI及MRA检查提示部分血管痉挛,典型者呈"串珠样"改变;2例脑内有病灶。脑脊液等相关检查无特异性,经钙离子拮抗剂等治疗后临床症状逐渐消失,1~7个月超声影像学复查完全恢复正常,临床呈单相病程,预后良好。结论临床表现为"雷击样、撕裂样"头痛的患者伴或不伴有神经系统定位体征,影像学检查有血管痉挛表现,在排除其他疾病后应考虑RCVS的可能。     

Posterior reversible encephalopathy syndrome

Posterior reversible encephalopathy syndrome (PRES) is a clinico-neuroradiological entity with typical symptoms and symmetric high-signal intensity lesions in the bilateral parieto-occipital lobes on T2-weighted or fluid-attenuated inversion recovery (FLAIR) MRI. We described three patients with PRES of varied etiologies. Patient 1 was a young man with severe hypertension who presented with headache and visual disturbance. Patient 2 had leukemia and was receiving umbilical cord blood cell transplantation with immunosuppressant, and developed PRES with convulsions. Patient 3 was a pregnant woman with renal failure, who repeatedly developed PRES with convulsions. FLAIR and apparent diffusion coefficient mapping were useful in detecting PRES lesions in our patients, although diffusion-weighted imaging and CT scans had limited use in the diagnosis. Adequate and prompt treatment with antihypertensive medication immediately ameliorated the symptoms, with improvement of abnormal MRI findings. In previous reports, delayed diagnosis might have affected the prognosis. Further work on the clinical manifestations of PRES and its therapy is required.     

Cortical subarachnoid hemorrhage associated with reversible cerebral vasoconstriction syndrome after elective triplet cesarean delivery

Reversible cerebral vasoconstriction syndromes (RCVS) comprise a group of disorders characterized by prolonged, but reversible vasoconstriction of the cerebral arteries, usually associated with acute-onset, severe, recurrent headaches, with or without additional neurological signs and symptoms. Various complications of this condition have been observed, such as cortical subarachnoid hemorrhages (cSAH), intracerebral hemorrhages, reversible posterior leukoencephalopathy, ischaemic strokes and transient ischaemic attacks. It is important to include RCVS in thunderclap headache differential diagnosis and among non-aneurismatic subarachnoid hemorrhage causes. In the past years, thanks to the major diffusion of new diagnostic tools such as magnetic resonance, computed tomography and digital subtraction angiography, RCVS have been demonstrated to be more frequent than previously thought. We report an illustrative case of a woman affected by a small cSAH, associated to RCVS, after elective triplet cesarean delivery. To our knowledge, this is the first case of cSAH associated to RCVS after a triplet pregnancy.     

Reversible Cerebral Vasoconstriction Syndrome in Patients with Coronavirus Disease: A Multicenter Case Series

Background and objectivesRCVS (Reversible Cerebral Vasoconstrictive Syndrome) is a condition associated with vasoactive agents that alter endothelial function. There is growing evidence that endothelial inflammation contributes to cerebrovascular disease in patients with coronavirus disease 2019 (COVID-19). In our study, we describe the clinical features, risk factors, and outcomes of RCVS in a multicenter case series of patients with COVID-19.Materials and methodsMulticenter retrospective case series. We collected clinical characteristics, imaging, and outcomes of patients with RCVS and COVID-19 identified at each participating site.ResultsTen patients were identified, 7 women, ages 21 – 62 years. Risk factors included use of vasoconstrictive agents in 7 and history of migraine in 2. Presenting symptoms included thunderclap headache in 5 patients with recurrent headaches in 4. Eight were hypertensive on arrival to the hospital. Symptoms of COVID-19 included fever in 2, respiratory symptoms in 8, and gastrointestinal symptoms in 1. One patient did not have systemic COVID-19 symptoms. MRI showed subarachnoid hemorrhage in 3 cases, intraparenchymal hemorrhage in 2, acute ischemic stroke in 4, FLAIR hyperintensities in 2, and no abnormalities in 1 case. Neurovascular imaging showed focal segment irregularity and narrowing concerning for vasospasm of the left MCA in 4 cases and diffuse, multifocal narrowing of the intracranial vasculature in 6 cases. Outcomes varied, with 2 deaths, 2 remaining in the ICU, and 6 surviving to discharge with modified Rankin scale (mRS) scores of 0 (n=3), 2 (n=2), and 3 (n=1).ConclusionsOur series suggests that patients with COVID-19 may be at risk for RCVS, particularly in the setting of additional risk factors such as exposure to vasoactive agents. There was variability in the symptoms and severity of COVID-19, clinical characteristics, abnormalities on imaging, and mRS scores. However, a larger study is needed to validate a causal relationship between RCVS and COVID-19.     

Reversible cerebral vasoconstriction syndromes and primary angiitis of the central nervous system: clinical,imaging, and angiographic comparison

Reversible cerebral vasoconstriction syndromes (RCVS) and primary angiitis of the central nervous system (PACNS) are invariably considered in the differential diagnosis of new cerebral arteriopathies. However, prompt and accurate diagnosis remains challenging. Here we compared the features of 159 RCVS to 47 PACNS patients and developed criteria for prompt bedside diagnosis. Recurrent thunderclap headache (TCH), and single TCH combined with either normal neuroimaging, border zone infarcts, or vasogenic edema, have 100% positive predictive value for diagnosing RCVS or RCVS‐spectrum disorders. In patients without TCH and positive angiography, neuroimaging can discriminate RCVS (no lesion) from PACNS (deep/brainstem infarcts). Ann Neurol 2016;79:882–894     

Seizure outcomes of posterior reversible encephalopathy syndrome and correlations with electroencephalographic changes

RationaleSeizures are among the most common clinical presentations of posterior reversible encephalopathy syndrome (PRES). This syndrome has rarely been reported to cause chronic epilepsy or persistent cortical dysfunction. The prognostic value of EEG findings during PRES is unknown. We retrospectively evaluated EEG characteristics in patients with PRES in a single medical center. We also evaluated the long-term outcome regarding seizure occurrence beyond the acute phase in these patients.MethodsWe searched a radiology database at the University of Minnesota from 1997 to 2012 to identify patients with clinically and radiologically diagnosed PRES. Among the patients with PRES, we reviewed MRI images, EEG findings, clinical manifestations including seizure occurrences, and clinical outcomes beyond the acute phase.ResultsSeventy-five patients were included in the study. Fifty-eight out of seventy-five (77.3%) patients with PRES had seizures. A total of 48 EEG studies were performed in 38 patients. Generalized slowing was the most common EEG pattern. Among the 38 patients who had EEGs, 37 (97.3%) patients had diffuse or focal slowing of the background, and 11 (28.9%) patients had IEDs. Four out of seventy-five (5.3%) patients had seizures later than one month beyond their hospitalization for PRES. None of these 4 patients had seizures before the episode of PRES. Two patients developed chronic epilepsy, with seizures occurring later than one year after the PRES.ConclusionMost patients who had seizures or who had epileptiform activities in EEG during PRES did not subsequently develop chronic epilepsy. No patient developed chronic epilepsy in the absence of clinical seizures during PRES. Posterior reversible encephalopathy syndrome may infrequently be associated with subsequent development of symptomatic epilepsy.     

Posterior reversible encephalopathy syndrome induced by intracranial hypotension in a postpartum patient

Abstract

Objective: A number of hormonal, physiologic, immunologic, and hemodynamic changes can cause a series of central nervous system-related problems in pregnant and postpartum women. Posterior reversible encephalopathy syndrome (PRES) is commonly seen in these conditions. However, PRES during pregnancy and the postpartum period are not always due to pregnancy.

Methods: We describe a patient who presented with headache followed by an epileptic seizure after cesarean section and whose computed tomography (CT) showed bilateral low-density lesions in the frontal lobe. To explore the pathogenesis, we further examined the patient with brain magnetic resonance imaging (MRI) and lumbar puncture.

Results: Brain MRI revealed vasogenic edema in the frontal lobe and temporal-occipital regions of both hemispheres. MRI of the brain with contrast showed diffuse enhancement of the supratentorial dura mater and decreased of bilateral lateral ventricles. There was no abnormality in brain magnetic resonance angiography and magnetic resonance venography. Bloody cerebrospinal fluid flowed very slowly during lumbar puncture.

Conclusion: These findings suggest that, although rare, intracranial hypotension in postpartum patients may be a cause of PRES.     

Primary intraventricular hemorrhage in adults: etiological causes and prognostic factors in Chinese population

Primary intraventricular hemorrhage (PIVH) is a rare type of hemorrhagic stroke that is poorly understood. We aimed to explore the features of this disease in Chinese population via an institutional prospective study. Adult patients diagnosed with PIVH from January 2013 to January 2016 were enrolled in this study. Data, including clinical variables, radiological features, and yield of angiography, were collected to evaluate the clinical features, etiological causes, and prognostic factors of this disease. A total of 67 patients (73.6%) were diagnosed with PIVH which constituted 3.2% of contemporary patients with hemorrhagic stroke in our hospital. Thirty-four patients (52.3%) were diagnosed with vascular structural abnormality (VSA)-related PIVH, and the etiologies included Moyamoya disease (22.4%), arteriovenous malformations (17.9%), aneurysms (7.5%), bilateral internal carotid artery dissection (1.5%), and tumor (1.5%). Idiopathic PIVH was diagnosed in 31 patients (47.7%), including coagulopathy in 3 (4.5%). Patients with VSA-related PIVH were younger than idiopathic PIVH patients, with a mean age of 37.1 ± 14.6 years, and idiopathic PIVH patients were more commonly hypertensive. The overall mortality rate was 11.9%, and 21 patients (31.3%) had a poor outcome at the 6-month follow-up. Patients with younger age, lower Graeb score, and a known etiology of arteriovenous malformation might be associated with a favorable outcome. We recommended routine thin-slice computed tomography (CT) scan, computed tomographic angiography (CTA), and digital subtraction angiography (DSA) for patients with PIVH. The etiological causes and prognostic factors of PIVH in Chinese patients were associated with distinctive features.     

Transcranial Doppler findings in a population with clinical probable reversible cerebral vasoconstriction syndrome

ObjectiveTo describe transcranial Doppler (TCD) findings in a population with clinical probable RCVS. Exploratory objectives included the study of clinical characteristics of probable RCVS patients with and without spasm detected by TCD.MethodsCross-sectional cohort study of patients with thunderclap headache (TCH) without subarachnoid hemorrhage (SAH) of our neurology and headache center between 2010 and 2019, selecting patients with clinical diagnosis of probable RCVS (negative angiography study) by ICHD-3 criteria and with at least two TCD studies.ResultsFrom 114 TCH patients, 36/114 had probable RCVS by ICHD-3 criteria and had at least two TCD studies available. The mean age at RCVS onset was 42.9 years (21–72 years); 29/36 (80.6%) were female, 7/28 (25%) had cardiovascular risk factors and 20/36 (55.6%) had history of migraine. Most common triggers were stressful emotion, drugs, valsalva maneuvers and sexual activity. Five/36 (13.9%) had complications and 3/36 (8.3%) had late recurrence. Initial TCD was performed on average of 16 (6–26) days after headache onset. Twenty-nine had vasospasm on TCD, presenting mean flow velocity of MCA (VMCA) of 135.7 ± 17.0 cm/s and mean maximum VMCA of 138.3 ± 17.2. Vasospasm was mild in 21/29 patients (72.4%) and moderate in 8/29 (27.6%). Complete VMCA normalization occurred on average 41 (30–70) days after headache onset and 24 (11–47) days after initial TCD. The group of patients with vasospasm detected by TCD had more female patients (26/29, 89.7% vs. 3/7, 42.8%, P = 0.016), and more TCH attacks (mean of 3.6 vs. 2.14, P = 0.049).ConclusionTCD may be a useful tool in the identification of vasospasm in patients with probable RCVS, supporting the diagnosis of RCVS in patients presenting with recurrent TCH without SAH.     

A case of reversible cerebral vasoconstriction syndrome (RCVS) triggered by a Chinese herbal medicine]

A 51-year-old woman started taking Chinese medicine containing ephedara herba as a nasal decongestant. One week later, she had three episodes of thunderclap headache, one during defecation and the others while taking a bath. She then had a convulsive seizure upon resolution of the second headache. A cranial CT did not show subarachnoid hemorrhage. Repeated CSF examinations showed neither xanthochromia nor inflammation. Brain diffusion-weighted and FLAIR MR images revealed high intensity lesions in bilateral hemispheres. A cerebral angiography showed multifocal segmental stenosis of bilateral cerebral arteries. Four months later, follow-up angiography showed normalized flow in all cerebral arteries and we gave a diagnosis of reversible vasoconstriction syndrome (RCVS). She has had no symptoms and signs since the third attack of headache. RCVS is an important disease in the differential diagnosis of thunderclap headache without neurological deficit. This is the first report of RCVS triggered by Chinese herbal medicine.     

产后脑血管病一例报道和文献复习

目的 探讨产后脑血管病的病因、临床特征、诊断及治疗.方法 对一例产后脑血管病患者的临床症状、体征、辅助检查、治疗和预后进行报道,结合38例文献报道的病例进行复习.结果 均急性发病,雷击样头痛是共同的临床症状,可伴有短阵性血压升高.MRI检查大多数患者表现双侧大脑半球皮质、皮质下白质多发斑片状T2WI、Flair高信号,DWI高信号、ADC图呈高信号或低信号.脑血管造影或TCD检查存在颅内大动脉及其分支多发节段性、可逆性痉挛.病理检查无血管炎性改变.绝大部分患者预后良好,复查MRI异常信号影消失.结论 (1)产后脑血管病病因未明,可能与免疫机制异常有关,雷击样头痛是主要的临床表现.(2)MRI检查多表现为可逆性后部白质脑病综合症改变,可伴有皮质、皮质下或分水岭区腔隙性脑梗塞、少量脑出血和蛛网膜下腔出血.(3)颅内大动脉及其分支可逆性痉挛是产后脑血管病的病理生理改变.(4)解除脑血管痉挛是治疗成功的关键.     

脑静脉窦血栓形成的诊断与血管内治疗

目的 探讨脑静脉窦血栓形成的诊断和血管内溶栓治疗。方法 对8例以顽固性头痛、呕吐伴或不伴局灶性神经功能缺损或癫发作为主要表现的患者，采用颅脑MRI和脑血管造影检查确定诊断，并施行经颈内动脉、静脉窦内接触性溶栓或静脉窦内支架成形术协同抗凝治疗。结果 MRI显示相应脑静脉窦区出现长T2WI，短T1WI信号，DSA显示脑静脉窦闭塞、静脉侧支循环建立和脑动静脉循环时间延长。治疗后静脉窦完全或部分再通，脑循环时间接近正常，临床病情显著缓解。结论 血管内介入治疗可能是脑静脉窦血栓形成最有效的治疗方法之一，有待大样本研究证实。     

Isolated thunderclap headache during sex: Orgasmic headache or reversible cerebral vasoconstriction syndrome?

Orgasmic headache (OH) is an “explosive” headache that occurs at orgasm. Historically, it was considered benign with no treatment needed. Reversible cerebral vasoconstriction syndrome (RCVS) refers to a group of disorders characterized by recurrent thunderclap headache (TCH) and multifocal vasoconstriction. Patients who have RCVS often recover completely, but some may have persistent neurological deficits. We report a 34-year-old woman who presented with isolated and recurrent TCH at orgasm, which fulfilled the diagnosis of OH. However, she was post-partum and had recent exposure to ecstasy, making her symptoms highly suggestive of RCVS. Brain magnetic resonance angiography showed segmental vasoconstriction. We concluded that she could be considered to have either OH or RCVS. This patient suggests the theory that OH could be a presentation of RCVS. Given that RCVS is potentially treatable, early recognition by clinicians is vital in order to prevent devastating complications.