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

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.     

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).     

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.     

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.     

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 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.     

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 and posterior reversible encephalopathy syndrome associated with intracranial hypotension

Background

Many demographic and physiological variables have been associated with TBI outcomes. However, with small sample sizes, making spurious inferences is possible. This paper explores the effect of sample sizes on statistical relationships between patient variables (both physiological and demographic) and outcome.

Methods

Data from head-injured patients with monitored arterial blood pressure, intracranial pressure (ICP) and outcome assessed at 6 months were included in this retrospective analysis. A univariate logistic regression analysis was performed to obtain the odds ratio for unfavorable outcome. Three different dichotomizations between favorable and unfavorable outcomes were considered. A bootstrap method was implemented to estimate the minimum sample sizes needed to obtain reliable association between physiological and demographic variables with outcome.

Results

In a univariate analysis with dichotomized outcome, samples sizes should be generally larger than 100 for reproducible results. Pressure reactivity index, ICP, and ICP slow waves offered the strongest relationship with outcome. Relatively small sample sizes may overestimate effect sizes or even produce conflicting results.

Conclusion

Low power tests, generally achieved with small sample sizes, may produce misleading conclusions, especially when they are based only on p values and the dichotomized criteria of rejecting/not-rejecting the null hypothesis. We recommend reporting confidence intervals and effect sizes in a more complete and contextualized data analysis.

     

可逆性脑血管收缩综合征:一例报告并文献分析

目的 报告1例临床罕见的可逆性脑血管收缩综合征病例,以期提高对该病的认识.方法 对1例可逆性脑血管收缩综合征患者的临床症状、影像学特征及临床转归进行回顾性分析,并复习相关文献.结果 女性患者,25岁.以声音刺激后发作性、雷击样、剧烈头痛为首发症状.继之出现右侧肢体无力,症状持续.MRI检查显示右侧外囊、左侧枕叶及左侧额顶叶皮质多发长T1、长T2信号;TCD检查显示双侧颈内动脉末端、大脑中动脉血流速度明显增快伴涡流、杂音,提示血管狭窄;MRA检查双侧大脑前动脉、大脑中动脉、大脑后动脉呈节段性狭窄,似"串珠样"改变.经氯吡格雷、羟乙基淀粉和维生素B1治疗后,临床症状完全恢复.出院后复查TCD显示血流速度和频谱基本正常,MRI显示病灶明显缩小,MRA可见血管形态恢复正常.临床诊断为可逆性脑血管收缩综合征.结论 临床表现为雷击样头痛的患者应考虑可逆性脑血管收缩综合征的可能,其继发性脑梗死易发生于大脑后循环供血区和分水岭区.TCD和MRA检查可作为诊断、药物疗效评价及随访观察的重要方法.     

Reversible cerebral vasoconstriction following carotid endarterectomy

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by the triad of severe "thunderclap" headaches, neurological symptoms and transient segmental cerebral arterial vasoconstriction. We report a patient with RCVS following carotid endarterectomy and review the literature on RCVS following carotid revascularisation.     

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.