Reversible cerebral segmental vasoconstriction

Vasoconstriction is not recognized as a cause of cerebrovascular disease except in the vasospasm seen following subarachnoid hemorrhage and possibly in migraine. However, we found four patients to have transient, fully reversible vasoconstriction and dilatation prominently involving arteries around the circle of Willis. All four patients were evaluated for severe headaches and fluctuating or recurring motor or sensory deficits. No cause for the clinical syndromes and angiographic abnormalities was found. Similar patients are reported in the literature under various nosologies. This newly recognized clinical-angiographic syndrome should be differentiated from other known causes of vessel constriction and dilatation; the precipitants of reversible vasoconstriction may then be better defined.     

Reversible cerebral vasoconstriction syndrome

Reversible cerebral vasoconstriction syndrome (RCVS) is an under-recognised condition. It is characterised by sudden and severe headaches (of “thunderclap” type) associated with multifocal reversible narrowing of the intracranial arteries on neuroradiology. The diagnosis is often established with the resolution of headaches and vasoconstriction. We report two patients with RCVS and review the available literature, to clarify the diagnostic criteria and discuss the treatment options.     

A pediatric case of reversible segmental cerebral vasoconstriction

BACKGROUND: Reversible segmental cerebral vasoconstriction (RSCV) is a recognizable clinical and radiographic syndrome consisting of thunderclap headache with or without focal neurological symptoms combined with reversible segmental vasoconstriction of proximal cerebral blood vessels. METHODS: We report a case of reversible segmental cerebral vasoconstriction in a child. RESULTS: A healthy 13-year-old boy experienced the sudden onset of a severe, diffuse headache upon surfacing from a deep dive in a swimming pool. Severity was maximal at the onset and improved over several hours. The same headache recurred three times over the next four days and a low baseline headache persisted throughout. Vomiting occurred once and mild photo/osmophobia were reported but throbbing, aura, or autonomic symptoms were absent. Focal neurological signs or symptoms were absent and he denied previous history of headaches, medications, drugs, or trauma. Two normal CT scans were performed within hours of separate headaches. Cerebrospinal fluid study on day 5 was bloody with no xanthochromia. MRI/MRA/MRV of the brain and vasculitic work-up were normal. Cerebral angiography on day 6 demonstrated smooth narrowing of multiple proximal cerebral vessels including supraclinoid internal carotid artery (ICA), M1, and A1 on the right and M1 on the left. By ten days, the patient's headaches had resolved and repeat angiography was normal. CONCLUSION: RSCV should be considered in a child with thunderclap headache.     

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.     

Reversible cerebral vasoconstriction syndrome identification of prognostic factors

Object

: Reversible cerebral vasoconstriction syndrome (RCVS) is described as a clinical and radiological entity characterized by thunderclap headaches, a reversible segmental or multifocal vasoconstriction of cerebral arteries with or without focal neurological deficits or seizures. The purpose of this study is to determine risk factors of poor outcome in patients presented a RCVS.

Methods

A retrospective multi-center review of invasive and non-invasive neurovascular imaging between January 2006 and January 2011 has identified 10 patients with criterion of reversible segmental vasoconstriction syndrome. Demographics data, vascular risks and evolution of each of these patients were analyzed.

Results

Seven of the ten patients were females with a mean age of 46 years. In four patients, we did not found any causative factors. Two cases presented RCVS in post-partum period between their first and their third week after delivery. The other three cases were drug-induced RCVS, mainly vaso-active drugs. Cannabis was found as the causative factor in two patient, Sumatriptan identified in one patient while cyclosporine was the causative agent in also one patient. The mean duration of clinical follow-up was 10.2 months (range: 0–28 months). Two patients had neurological sequelae: one patient kept a dysphasia and the other had a homonymous lateral hemianopia. We could not find any significant difference of the evolution between secondary RCVS and idiopathic RCVS. The only two factors, which could be correlated to the clinical outcome were the neurological status at admission and the presence of intraparenchymal abnormalities (ischemic stroke, hematoma) in brain imaging.

Conclusions

Fulminant vasoconstriction resulting in progressive symptoms or death has been reported in exceptional frequency. Physicians had to remember that such evolution could happen and predict them by identifying all factors of poor prognosis (neurological status at admission, the presence of intraparenchymal abnormalities).     

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。     

Reversible cerebral vasoconstriction syndrome: current and future perspectives

Reversible cerebral vasoconstriction syndromes (RCVS) are characterized by recurrent acute severe headaches, namely thunderclap headaches, and multifocal segmental vasoconstrictions. Interest has arisen in the definitions, clinical presentations, differential diagnoses, risk factors and complications of RCVS. This article will comprehensively review the milestone monographs and the latest research work addressing these issues. Studies that have focused on the relationship between RCVS and thunderclap headache will be detailed. We will also discuss research on the enigmatic pathophysiology and potential therapeutic approaches. Up-to-date information and challenges, undergoing studies and future research directions will be deeply probed.     

Vasoconstriction and long-term headache in reversible cerebral vasoconstriction syndrome

Journal of Neurology - Angiographic vasoconstriction in reversible cerebral vasoconstriction syndrome (RCVS) is often undetectable at symptom onset and the diagnosis relies on clinical...     

Reversible cerebral vasoconstriction syndrome: A thunderclap headache-associated condition

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by a sudden, severe headache at onset, vascular narrowing involving the circle of Willis and its immediate branches, and angiographic evidence of vasoconstriction reversibility within minutes to weeks of onset. RCVS is underrecognized and often misdiagnosed; it can defy clinical detection because it can mimic common conditions such as migraine and ischemic stroke. A lack of shared nosology has hampered awareness and understanding of the syndrome. Clinicians must consider primary angiitis of the central nervous system because of its high rates of morbidity and mortality if left untreated. RCVS has a number of primary and secondary associations (cerebral hemorrhage, vasoactive substances, the peripartum period, bathing, and physical exertion) but also occurs in isolation. RCVS can present in conjunction with hypertensive encephalopathy, preeclampsia, and reversible posterior leukoencephalopathy. This review provides an up-to-date account of RCVS.     

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.     

Concomitant reversible cerebral vasoconstriction syndrome and transient global amnesia

Journal of Neurology - Reversible cerebral vasoconstriction syndrome (RCVS) is a common cause of thunderclap headache (TCH), mainly recurrent, sometimes associated with seizures and/or neurological...     

Reversible cerebral vasoconstriction syndrome associated with brain parenchymal hemorrhage

We described a 7-year-old girl with reversible cerebral vasoconstriction syndrome associated with brain parenchymal hemorrhage. She initially presented with high fever and pancytopenia, leading to a diagnosis of most severe type aplastic anemia. We treated her with cyclosporine, methylprednisolone and anti-thymocyte globulin. Thereafter she recurrently complained of a very severe headache called as thunderclap, and finally exhibited loss of consciousness. Brain imaging revealed massive parenchymal hemorrhage between the left occipital and parietal lobes on computed tomography, and diffuse cerebral vasoconstriction on magnetic resonance angiography. The cerebral vasoconstriction resolved within two months, and thus we diagnosed her as having reversible cerebral vasoconstriction syndrome associated with brain parenchymal hemorrhage. This syndrome has been frequently reported in adult females, but rarely in children. However, even in children, a so called thunderclap headache may become a clue for the diagnosis of reversible cerebral vasoconstriction syndrome, especially in cases taking immunosuppressive agents. Immediate magnetic resonance angiography is essential to diagnose this syndrome, and a prompt application of calcium channel inhibitors should be considered to resolve constriction of the vessels and to prevent subsequent brain damage.     

应该重视对可逆性脑血管收缩综合征的诊断与治疗

可逆性脑血管收缩综合征(reversible cerebral vasoconstriction syndrome,RCVS)是一组罕见的临床综合征,自1988年Call和Fleming首次报告以来,至今已查到193例文献报道,其中欧洲91例,亚洲47例(日本5例、中国香港6例、中国台湾36例),北美洲27例及南美洲、大洋洲、非洲共28例.在有些文献报道中还将此综合征冠以不同的名称,如Call-Fleming综合征、中枢神经系统良性血管病、雷击样头痛伴血管收缩、药物诱导的脑动脉炎或脑血管病和产后血管病等.     

Case of posterior reversible encephalopathy syndrome with cerebral vasoconstriction]

A 56-year-old woman attended our hospital because of acute severe (thunderclap) headache. Neurological examination was normal and no abnormality was found on head CT or by cerebrospinal fluid examination. A few days later, she experienced a recurrence and suffered a seizure in her left upper and lower extremities. On neurological examination, she had conjugate deviation of the eyes toward the right side and left lower limb paralysis with Chaddock sign. MRI showed multiple hyperintense lesions in the bilateral occipital and parietal lobes, predominantly in the subcortical white matter at the right side on T2-weighted and FLAIR images. We diagnosed posterior reversible encephalopathy syndrome (PRES) because the ADC map showed a vasogenic edema pattern (increased ADC values in the hypodense lesions on diffusion-weighted image). Her blood pressure was normal and there were no underling diseases. As MRA showed vasoconstriction especially in bilateral posterior cerebral arteries, we initiated a therapy with a Ca-channel blocker. On follow-up MRI, the hyperintense lesions on T2-weighted and FLAIR images had almost disappeared, and vasoconstriction was also improved on MRA. This case suggested that cerebral vasoconstriction could underlie both thunderclap headache and PRES.