Le syndrome de vasoconstriction cérébrale réversible

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by severe headaches, as well as vasoconstriction of cerebral arteries, which resolves spontaneously in one to three months. This condition has a moderate female preponderance. The mean age of onset is around 45 years. About 60% of the cases are secondary, mainly occurring during postpartum and/or after exposure to vasoactive substances. The main clinical presentation includes multiple recurrent thunderclap headaches over one to three weeks. The major complications of RCVS are localized cortical subarachnoid hemorrhages (cSAH) (20-25%) and parenchymal strokes (5-10%). Complications occur with different time courses: hemorrhages (cSAH and intracerebral hemorrhages), and posterior reversible encephalopathy syndrome are early events occurring during the first week, while ischemic events including TIAs and cerebral infarcts occur significantly later, during the second week. Diagnosis requires the demonstration of the “string and beads” aspect of cerebral arteries by a cerebral angiogram (MRA, CTA or conventional) and the demonstration of the complete or marked normalisation of arteries by a repeat angiogram performed within 12 weeks of onset. Treatment is based on nimodipine that seems to reduce thunderclap headaches within 48 h. However, nimodipine has not proven any efficacy against the hemorrhagic and ischemic complications of RCVS. Relapses are possible but rare and have not been reported yet in prospective series. It seems appropriate to advise the patients to avoid sympathomimetic and serotoninergic substances.     

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.     

Progressive Manifestations of Reversible Cerebral Vasoconstriction Syndrome Presenting with Subarachnoid Hemorrhage,Intracerebral Hemorrhage,and Cerebral Infarction

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden-onset headache with focal neurologic deficit and prolonged but reversible multifocal narrowing of the distal cerebral arteries. Stroke, either hemorrhagic or ischemic, is a relatively frequent presentation in RCVS, but progressive manifestations of subarachnoid hemorrhage, intracerebral hemorrhage, cerebral infarction in a patient is seldom described. We report a rare case of a 56-year-old woman with reversible cerebral vasoconstriction syndrome consecutively presenting as cortical subarachnoid hemorrhage, intracerebral hemorrhage, and cerebral infarction. When she complained of severe headache with subtle cortical subarachnoid hemorrhage, her angiography was non-specific. But, computed tomographic angiography showed typical angiographic features of this syndrome after four days. Day 12, she suffered mental deterioration and hemiplegia due to contralateral intracerebral hematoma, and she was surgically treated. For recurrent attacks of headache, medical management with calcium channel blockers has been instituted. Normalized angiographic features were documented after 8 weeks. Reversible cerebral vasoconstriction syndrome should be considered as differential diagnosis of non-aneurysmal subarachnoid hemorrhage, and repeated angiography is recommended for the diagnosis of this under-recognized syndrome.     

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.     

Reversible cerebral vasoconstriction syndrome: rare or underrecognized in children?

Reversible cerebral vasoconstriction syndrome (RCVS) is a clinicoradiological diagnosis comprising ‘thunderclap’ headaches and reversible segmental vasoconstriction of cerebral arteries, occasionally complicated by ischaemic or haemorrhagic stroke. We report a case of RCVS in a 13‐year‐old male with severe thunderclap headaches and no focal neurological signs. Brain imaging showed multiple posterior circulation infarcts; cerebral computed tomography, magnetic resonance imaging, and catheter angiography showed multifocal irregularity and narrowing, but in different arterial segments. Laboratory studies did not support a diagnosis of vasculitis. Symptoms resolved over 3 weeks; magnetic resonance angiography 3 months later was normal and remained so after 2 years. We highlight the typical clinical features of RCVS in this case and suggest that the diagnosis should be considered in children with thunderclap headaches or stroke syndromes where headache is a prominent feature, especially if cerebrovascular imaging studies appear to be evolving or discrepant.     

Reversible cerebral vasoconstriction syndrome and dissection in the setting of COVID-19 infection

The current COVID-19 pandemic has recently brought to attention the myriad of neuro- logic sequelae associated with Coronavirus infection including the predilection for stroke, particularly in young patients. Reversible cerebral vasoconstriction syndrome (RCVS) is a well-described clinical syndrome leading to vasoconstriction in the intracra- nial vessels, and has been associated with convexity subarachnoid hemorrhage and oc- casionally cervical artery dissection. It is usually reported in the context of a trigger such as medications, recreational drugs, or the postpartum state; however, it has not been described in COVID-19 infection. We report a case of both cervical vertebral ar- tery dissection as well as convexity subarachnoid hemorrhage due to RCVS, in a pa- tient with COVID-19 infection and no other triggers.     

Pronóstico al año de la hemorragia subaracnoidea cortical no traumática: Serie prospectiva de 34 pacientes

IntroductionCortical subarachnoid haemorrhage (cSAH) has multiple aetiologies. No prospective study has reported the long-term progression of the condition. The objective of this study is to describe the clinical and aetiological characteristics of patients with cSAH and to gain insight into prognosis.MethodsWe performed a prospective, observational, multi-centre study. Data on clinical and radiological variables were collected; during a one-year follow-up period, we recorded data on mortality, dependence, rebleeding, and the appearance of dementia.ResultsThe study included 34 patients (mean age, 68.3 years; range, 27-89). The most frequent symptoms were headache and focal neurological deficits, which were frequently transient and recurrent. CT scans returned pathological findings in 28 patients (85%). Brain MRI scans were performed in 30 patients (88%), revealing acute ischaemia in 10 (29%), old haemorrhage in 7 (21%), and superficial siderosis in 2 (6%). Aetiology was identified in 26 patients (76.5%): causes were cerebral amyloid angiopathy in 8, ischaemic stroke in 5, vasculitis in 4, reversible posterior encephalopathy in 2, venous thrombosis in 2, reversible cerebral vasoconstriction syndrome in 2, carotid occlusion in 1, Marfan syndrome in 1, and meningeal carcinomatosis in 1. Three patients died during follow-up (2 due to causes related to the cause of cSAH). Three patients developed dementia, 3 had lobar haemorrhages, and one had a second cSAH.ConclusionsThe most frequent causes of cSAH in our series were cerebral amyloid angiopathy, ischaemic stroke, and vasculitis. This type of haemorrhage has a worse prognosis than other non-aneurysmal cSAH. There are numerous possible causes, and prognosis depends on the aetiology. In elderly patients, intracranial haemorrhage is frequently associated with cognitive impairment.     

Reversible cerebral vasoconstriction syndrome or primary angiitis of the central nervous system?

BACKGROUND: Reversible Cerebral Vasoconstriction Syndrome (RCVS) may present as thunderclap headache (TCH), accompanied by reversible cerebral vasospasm and focal neurological deficits, often without a clear precipitant. RCVS may be mistaken for Primary Angiitis of the Central Nervous System (PACNS) due to the presence of similar angiographic features of segmental narrowing of cerebral arteries. We discuss the clinical features of a young female migraine patient who developed TCH and was found to have RCVS following initial treatment with corticosteroids for PACNS, in the context of a systematic review of the available medical literature. METHODS: A Medline search was performed to identify all case reports since 1966 describing RCVS and PACNS that provide sufficient clinical detail to permit diagnostic classification according to published criteria. RCVS included case studies in which there was angiographic or transcranial Doppler ultrasound evidence of near-to-complete resolution of cerebral vasoconstriction in the absence of a well-recognized secondary cause. PACNS included reports of histologically confirmed PACNS either through biopsy or necropsy. RESULTS: Reversible Cerebral Vasoconstriction Syndrome occurs primarily in females and is characterized by sudden, severe headache at onset, normal CSF analysis, vasoconstriction involving the Circle of Willis and its immediate branches, and angiographic or TCD ultrasound evidence of near-to-complete vasospastic resolution within 1-4 weeks. It occurs typically in the context of vasoconstrictive drug use, the peripartum period, bathing, and physical exertion. CONCLUSION: Initial and follow-up (within 4 weeks) non-invasive angiographic studies are indicated in patients who present with TCH or who have clinical presentations that could be consistent with RCVS or PACNS in the absence of a well-recognized secondary cause, such as subarachnoid haemorrhage. Early reversibility of cerebral vasospasm is the key neuroradiological feature that supports the clinical diagnosis of RCVS.     

Reversible Vasoconstriction Syndrome with Bilateral Basal Ganglia Hemorrhages

Reversible cerebral vasoconstriction syndrome (RCVS) is an increasingly recognized acute cerebrovascular condition that may produce myriad transient and sustained neurologic deficits as well as a host of radiologic features. We report the case of a woman with RCVS and a severe clinical syndrome with bilateral basal ganglia hemorrhages, cerebral infarctions, and marked vascular abnormalities. The patient made a near complete clinical recovery, representing an extreme and illustrative form of RCVS.     

Severe reversible cerebral vasoconstriction syndrome mimicking aneurysmal rupture and vasospasm

Introduction  

Presenting symptoms of aneurysmal subarachnoid hemorrhage (SAH) and reversible cerebral vasoconstriction syndrome (RCVS) may overlap. Patients with RCVS often harbor unruptured aneurysms.     

Subarachnoid and intra-cerebral hemorrhage in young adults: Rare and underdiagnosed

Objectives

Convexity subarachnoid and intra-cerebral hemorrhages, in patients aged < 50 years, are always a diagnostic challenge. This condition is characterized by acute headaches with or without neurological symptoms and/or seizures, and by the radiological demonstration of subarachnoid and/or intra-cerebral hemorrhages and, more rarely, by the association of ischemic events.

Patients and methods

In a prospective series of 30 consecutive patients (median age 31 years; 22 women) with a subarachnoid and intra-cerebral hemorrhages, 19 were diagnosed with reversible cerebral vasoconstriction syndrome (RCVS), 7 with cerebral venous sinus thrombosis (CVST), and 4 with a bleeding mycotic aneurysm (MA).

Results

RCVS appeared spontaneously in 16 patients and was related to the postpartum period in three cases. Subarachnoid hemorrhage (SAH) was demonstrated in 24 patients as follows: 18 cases were in cortical areas, 4 were in the polygon of Willis, one was inter-hemispheric, and one was inter-hemispheric/intra-cerebral. A convexity pure intra-cerebral hemorrhage (ICH) was recorded in 6 cases. Among the 7 patients suffering from CVST, the superior sagittal sinus was involved in 4 cases, the transverse sinuses (TS) in 2, and the TS plus sigmoid sinus (SS) in one.

Conclusion

The three most common causes in this series were RCVS, followed by CVST and bleeding from MA. Because of atypical clinical or radiological presentations, this large spectrum of etiologies can cause diagnostic difficulties. Therefore, careful analysis is needed to ensure correct and prompt diagnosis and to avoid any dangerous delays in management.     

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.     

非外伤性皮质凸面蛛网膜下腔出血237例汇集分析

目的探讨非外伤性大脑皮质凸面蛛网膜下腔出血(cSAH)的临床特征,以提高对该病的认识。方法以PubMed和"中国生物医学文献数据库"为主要检索数据库,对2004至2014年发表的中、英文文献26篇中237例CSAH的临床特征进行汇集分析,内容包括病因、临床表现、影像学特征、治疗及预后。结果 cSAH各年龄段均有发病,绝大多数急性起病。脑淀粉样血管病、可逆性脑血管收缩综合征、脑静脉血栓形成、颈内动脉狭窄/闭塞、可逆性后部脑病综合征占全部病因66.67%。常见症状为不同程度的头痛、类似短暂性脑缺血发作的神经功能缺损症状、局限性神经功能缺损以及癫发作。头颅CT阳性检出率87.91%,主要表现大脑皮质凸面条索状出血。大多数cSAH患者预后良好,再出血发生率低。结论 cSAH是蛛网膜下腔出血(SAH)的少见类型,其病因、临床表现及影像学特点与动脉瘤破裂导致SAH不同。对于初次发病的cSAH,要进行全面的脑血管筛查。     

Atraumatic nonaneurysmal sulcal subarachnoid hemorrhages: a diagnostic workup based on a case series

Introduction: Atraumatic and nonaneurysmal sulcal subarachnoid hemorrhage (sSAH) is a rare type of cerebrovascular disease with various etiologies previously reported in small case reports. In this study, we propose to analyze clinical presentations, imaging patterns and etiologies in a large case series of such patients in order to propose a diagnostic workup. Methods: We retrospectively analyzed clinical and radiological data of consecutive patients with a diagnosis of atraumatic and nonaneurysmal sSAH, admitted to our institution between 2008 and 2011. All patients had both computed tomography (CT) and magnetic resonance imaging (MRI) as a part of their initial evaluation. Results: 30 patients (18 women and 12 men, mean age: 60 years) were identified. The main clinical symptoms at presentation were focal and transient neurological deficit (n = 22) and thunderclap headache (n = 10). Four patients had progressive headache and 4 other had partial or generalized epileptic seizures. MRI abnormalities associated with sSAH were prior hemorrhages, microbleeds, severe leukoencephalopathy and hemosiderosis suggesting cerebral amyloid angiopathy (CAA; n = 9), vasogenic edema in parieto-occipital areas compatible with a posterior reversible encephalopathy syndrome (PRES; n = 3), cortical venous thrombosis (n = 2) and concomitant acute cortical stroke (n = 3). Other underlying causes of sSAH, not diagnosed on MRI, were reversible cerebral vasoconstriction syndrome (RCVS) based on clinical criteria and conventional angiography (n = 4), angiitis diagnosed by skin biopsy (n = 1), vascular malformation diagnosed on CT and digital subtraction angiographies (n = 3), and overanticoagulation (n = 1). Four cases remained unresolved. Conclusion: This study confirmed that sSAH is a rare condition related to a wide spectrum of etiologies. Combination of brain MRI and magnetic resonance angiography and eventually digital subtraction angiography allowed the identification of an underlying etiology for 87% of patients. CAA, RCVS and PRES represented more than 50% of the etiological mechanisms. Among older patients, sSAH was mainly related to CAA while in younger patients, RCVS represented the most frequent etiology.     

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.     

Initial vasodilatation in a child with reversible cerebral vasoconstriction syndrome

We describe the case of a 10-year-old boy who developed reversible cerebral vasoconstriction syndrome (RCVS) after cerebellitis. He received intravenous immunoglobulin and methylprednisolone to treat the cerebellitis. However, he then presented with a sudden severe headache, vomiting, and generalized tonic-clonic seizure. Brain magnetic resonance angiography (MRA) initially revealed diffuse cerebral vasodilatations, and diffuse multifocal segmental vasoconstrictions developed several days later. His clinical symptoms gradually resolved after several days, in the absence of any specific therapy. MRA performed 46 days after symptom onset showed that the multifocal segmental vasoconstrictions had resolved, suggesting a diagnosis of RCVS. The imaging features of RCVS include multifocal segmental vasoconstriction. However, our case suggests that diffuse cerebral vasodilatation may in fact be evident during the early stage of disease.     

Cortical Subarachnoid Hemorrhage in Behcet's Disease

ObjectiveCortical subarachnoid hemorrhage (cSAH) can be resulted from by a wide variety of causes. A cSAH probably associated with Behcet's disease is herein reported for the first time.Case reportA 58-year-old male with stable Behcet's disease evaluated for sudden onset headache. Emergency computed tomography disclosed cSAH in the left frontal sulci. A ruptured middle cerebral artery cortical branch aneurysm was found. Surprisingly, this aneurysm obliterated completely after diagnostic catheter angiography.ConclusionThis case highlights three important points; First, non-mycotic (non-infectious) distal cerebral artery aneurysms may rarely cause cSAH. Second, distal dissecting aneurysms may rarely become obliterated after diagnostic cerebral angiography. Third, such aneurysms may be one of the long-term consequences of vascular inflammation in Behçet's disease.     

Reversible cerebral vasoconstriction syndrome

ABSTRACT

Reversible cerebral vasoconstriction syndrome (RCVS) is a relatively rare, non-progressive angiopathy frequently heralded by severe thunderclap headache. It is characterised by vasoconstriction of cerebral arteries which usually resolves within three months of onset. Transient focal neurological signs may occur, and persistent deficits associated with haemorrhagic comorbidities have been reported in a small percentage of individuals. In this paper we report the case of RH, a 36-year-old woman who presented at a university teaching hospital in Sydney with a clinical history and radiological evidence consistent with RCVS. There were no haemorrhagic events during the course of her illness, and vasoconstriction resolved within a few days, following treatment with verapamil. Neuropsychological evaluation 16 months later revealed significant deficits in autobiographical memory, verbal and non-verbal new learning and active delayed recall, cognitive flexibility, abstraction and (to a lesser extent) immediate attention span and information processing speed. RH’s case was unusual because the “trigger” for RCVS (Ear, Nose and Throat surgery) has not been previously reported, and because despite there being no haemorrhagic complications during the course of RCVS and no subsequent radiological abnormalities, she had significant cognitive impairment. To date, persistent neuropsychological deficits have not been recognised as a feature of RCVS.     

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.     

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.