Possible overlap between reversible cerebral vasoconstriction syndrome and symptomatic vasospasm after aneurysmal subarachnoid hemorrhage

A 34-year-old woman with a previous history of severe headache (“thunderclap”) was admitted with a diagnosis of aneurysmal subarachnoid hemorrhage (SAH). The patient developed symptomatic vasospasm on day 5 that resolved rapidly after having increased arterial blood pressure. She experienced also short-lasting excruciating headache. On day 12, while velocities had normalised, as revealed by transcranial Doppler (TCD), for more than 48 h, she developed aphasia and right hemiplegia associated with diffuse segmental vasospasm on the left middle cerebral artery. Intra-arterial infusion of vasodilatory agents was required. Recurrence of symptomatic vasospasm was noted on day 25, with a great number of territories involved as shown in the cerebral angiogram. A second intra-arterial treatment was needed. The patient complained of multiple episodes of extremely severe headache (“thunderclap”), with also transient dysarthria and hemiparesia on day 30. She was discharged on day 38 after full recovery. The clinical and TCD/radiological findings were consistent with a reversible cerebral vasoconstriction syndrome overlapping SAH related symptomatic vasospasm.     

Reversible cerebral vasoconstriction syndrome: case report

A 28-year-old woman had thunderclap headache (TCH), after 7 days she had left hemiparesis. She had a history of oral contraceptive and citalopram medications. Brain magnetic resonance (MR) angiography demonstrated multiple stenotic segments. Digital subtraction angiography (DSA) showed multiple segments of narrowing in vessel calibre. Two probable diagnoses performed; primary angiitis of the central nervous system and reversible cerebral vasoconstriction syndrome (RCVS). Because of clinical characteristics and normal cerebrospinal fluid findings she was set on medication for probable RCVS. Follow-up MR angiography after 4 weeks and DSA after 7 weeks demonstrated improvement in vessel calibre. Thus, diagnosis RCVS was established. Diagnosis and management of TCH contain many potential difficulties. Clinicians should consider the imaging of cerebral arteries, even if computed tomography scan and lumbar puncture are normal in TCH. Potential precipitating factors and triggers should also be known and avoided. This case was presented as a poster at the European Headache and Migraine Trust International Congress (EHMTIC), 4–7th September 2008, London, UK entitled “The Call–Fleming Syndrome: case report”.     

Reversible cerebral vasoconstriction syndrome associated with autonomic dysreflexia

A 32-year-old man with a residual spastic quadriparesis from a traumatic C5–C6 fracture experienced a severe thunderclap headache. The medical history revealed an episode of autonomic dysreflexia (AD) due to neurogenic bladder/urinary tract infection (UTI). Blood pressure monitoring at admission revealed hypertension; blood pressure reaching 160/100 mmHg (average blood pressure in these patients and also in this patient being 90/60 mmHg). CT scan of the head, cerebrospinal fluid examination, CT angiography and MR angiography of the brain vessels were normal. Another UTI and a subsequent spell of AD were diagnosed. The patient continued to experience recurrent thunderclap headaches. Selective catheter cerebral angiography revealed multiple calibre changes in the intracranial blood vessels. A diagnosis of reversible cerebral vasoconstriction syndrome (RCVS) due to AD was considered. A magnetic resonance imaging (MRI) of the brain after 2 weeks revealed ischaemic changes in the left hemisphere. Follow-up brain MRI after 3 weeks showed reduction in size of the ischaemic changes, and catheter angiography after 6 weeks demonstrated improvement/normalization. A diagnosis of RCVS could be established. Repeated MRI/CT of the brain after 6 months demonstrated a large infarction in the left hemisphere. RCVS has been reported to occur in various clinical settings. It can occur in the setting of AD in patients with traumatic cervical cord injury. Prompt recognition of RCVS may be of vital importance to avoid further morbidity in patients with spinal cord injury.     

Reversible cerebral vasoconstriction syndrome (RCVS): an interesting case report

BackgroundReversible Cerebral Vasoconstriction Syndrome is a condition of transient cerebral vascular spasms, which usually presents with recurrent thunderclap headaches and recovers within 3 months. Several probable triggers and underlying factors, such as sex hormones, vasoactive drugs, head trauma or surgery, and tumors, have been implicated.Case presentationIn this paper, we present a 53-year-old woman with thunderclap headaches and normal lab tests who was radio-clinically diagnosed with reversible cerebral vasoconstriction syndrome and treated accordingly. Then, she experienced the recurrence of RCVS after about 2 years and headaches after 1 year in association with high blood pressure, high blood sugar, hypothyroidism, hyperlipidemia, and a urine metanephrine level of 5 times higher than the normal limit, suggesting a diagnosis of pheochromocytoma. After confirmation of the diagnosis with further imaging studies, surgical removal of the tumor resolved all the signs and symptoms.ConclusionOften underdiagnosed, pheochromocytoma could be an important condition associated with RCVS. It is important for clinicians to bear this diagnosis in mind while dealing with similar cases of recurrent thunderclap headaches.     

An often unrecognized cause of thunderclap headache: reversible cerebral vasoconstriction syndrome

Thunderclap headache (TCH) can have several causes of which subarachnoid hemorrhage (SAH) is most common and well known. A rare cause of TCH is the reversible cerebral vasoconstriction syndrome (RCVS) which is characterized by a reversible segmental vasoconstriction of the intracranial vessels. We describe two patients with TCH due to RCVS and the probable precipitating factor, namely, cannabis and an anti-migraine drug. In RCVS, cerebrospinal fluid examination is (near) normal, in contrast to SAH and (primary) cerebral vasculitis. Brain MRI may be normal or shows infarction. MRA can demonstrate vasoconstriction of the great arteries, but a normal MRA does not rule out the diagnosis. Caliber changes on cerebral angiography cannot adequately differentiate between RCVS and vasculitis. Calcium-channel antagonists may be a good therapy and repeated transcranial Doppler ultrasonography can be a reliable non-invasive investigation to monitor the effect of treatment and demonstrate reversibility of the vasoconstriction.     

A common cause of sudden and thunderclap headaches: reversible cerebral vasoconstriction syndrome

Background

Thunderclap headache (TCH) is a sudden headache (SH) with accepted criteria of severe intensity and onset to peak within one minute. It is a well-known presentation for subarachnoid hemorrhage (SAH) but most patients with TCH or SH run a benign course without identifiable causes. Reversible cerebral vasoconstriction syndrome (RCVS), a recently recognized syndrome characterized by recurrent TCH attacks, has been proposed to account for most of these patients.

Methods

We recruited consecutive patients presenting with SH at our headache clinic. Computed tomography and/or magnetic resonance imaging with angiography were performed to exclude structural causes and to identify vasoconstriction. Catheter angiography and lumbar puncture were performed with patients consent. Reversibility of vasoconstriction was confirmed by follow-up study.

Results

From July 2010 to June 2013, 31 patients with SH were recruited. Twenty-four (72.7%) of these SH patients exhibited headache fulfilling the TCH criteria. The diagnosis of RCVS was confirmed in 14 (45.2%) of patients with SH and 11 (45.8%) of patients with TCH. Other diagnoses were as follows: primary headaches (SH: 41.9%, TCH: 45.8%) and other secondary causes (SH: 12.9%, TCH: 8.3%). Compared with non-RCVS patients, patients with RCVS were older (50.8 ± 9.3 years vs. 40.8 ± 10.0 years, P = 0.006) and less likely to experience short headache duration of < 1 hour (23.1% vs. 78.6%, P = 0.007). Patients with RCVS were more likely to cite bathing (42.9% vs. 0%, P = 0.004) and less likely to cite exertion (0% vs. 29.4%, P = 0.048) as headache triggers.

Conclusions

Reversible cerebral vasoconstriction syndrome is a common cause of SH and TCH. Considering the potential mortality and morbidity of RCVS, systemic examination of cerebral vessels should be performed in these patients.     

Reduced circulating endothelial progenitor cells in reversible cerebral vasoconstriction syndrome

Background

The pathophysiology of reversible cerebral vasoconstriction syndrome (RCVS) remains elusive. Endothelial dysfunction might play a role, but direct evidence is lacking. This study aimed to explore whether patients with RCVS have a reduced level of circulating circulating endothelial progenitor cells (EPCs) to repair the dysfunctional endothelial vasomotor control.

Methods

We prospectively recruited 24 patients with RCVS within one month of disease onset and 24 healthy age- and sex-matched controls. Flow cytometry was used to quantify the numbers of circulating EPCs, defined as KDR⁺CD133⁺, CD34⁺CD133⁺, and CD34⁺KDR⁺ double-positive mononuclear cells. The Lindegaard index, an index of vasoconstriction, was calculated by measuring the mean flow velocity of middle cerebral arteries and distal extracranial internal carotid arteries via color-coded sonography on the same day as blood drawing. A Lindegaard index of 2 was chosen as the cutoff value for significant vasoconstriction of middle cerebral arteries based on our previous study.

Results

Patients with RCVS had a reduced number of CD34⁺KDR⁺ cells (0.009 ± 0.006% vs. 0.014 ± 0.010%, p = 0.031) but not KDR⁺CD133⁺ cells or CD34⁺CD133⁺ EPCs, in comparison with controls. The number of CD34⁺KDR⁺ cells was inversely correlated with the Lindegaard index (rs = -0.418, p = 0.047). Of note, compared to controls, patients with a Lindegaard index > 2 (n = 13) had a reduced number of CD34⁺KDR⁺ cells (0.007 ± 0.005% vs. 0.014 ± 0.010%, p = 0.010), but those with a Lindegaard index ≤ 2 did not.

Conclusions

Patients with RCVS had reduced circulating CD34⁺KDR⁺ EPCs, which were correlated with the severity of vasoconstriction. Endothelial dysfunction might contribute to the pathogenesis of RCVS.     

Upshaw-Schulman syndrome diagnosed during pregnancy complicated by reversible cerebral vasoconstriction syndrome

Upshaw-Schulman syndrome (USS) is an inherited type of thrombotic thrombocytopenic purpura (TTP) that is extremely rare, but often diagnosed during pregnancy. Reversible cerebral vasoconstriction syndrome (RCVS) is the transient stenosis of several cerebral arteries that is frequently diagnosed post-partum.We describe a 28-year-old woman with USS complicated by RCVS after delivery that was treated by plasma exchange with a good outcome. She was referred to our hospital with thunderclap headache, anemia and thrombocytopenia that occurred immediately postpartum. She was diagnosed with TTP and multiple cerebral infarctions. Plasma exchange promptly improved her symptoms on hospital day 3. Moreover, multiple stenoses of cerebral arteries indicating RCVS were resolved. Since her sister also had an episode of thrombocytopenia during pregnancy, inherited TTP was suspected and genetic analyses confirmed USS. Pregnancy is a risk for not only TTP, but also RCVS. Endothelial damage might be an underlining cause and vasospasm after delivery is a trigger of RCVS. Plasma exchange was effective against both TTP and RCVS.     

蛛网膜下腔出血后脑梗死的CT表现

目的探讨蛛网膜下腔出血后脑梗死的CT表现。方法分析18例蛛网膜下腔出血后脑梗死的临床特点与CT表现。结果蛛网膜下腔出血后脑梗死发生在蛛网膜下腔出血后7-13d(平均10.5d),其梗死范围大、部位多,CT以双侧对称性分布的多发病灶为特点。结论CT对蛛网膜下腔出血后脑梗死有很大的诊断价值。     

Reversible cerebral vasoconstriction syndrome: a complicated clinical course treated with intra-arterial application of nimodipine

Thunderclap headache (TCH) is a neurological emergency that warrants immediate and comprehensive diagnostic determination. When no pathology can be identified the condition is classified as primary TCH, which is considered benign and self-limiting. TCH has also been reported as the initial symptom of reversible cerebral vasoconstriction syndrome (RCVS), which subsumes a variety of conditions, inconsistently coined Call–Flemming syndrome, benign angiopathy of the central nervous system, drug-induced arteritis, or migrainous vasospasm. Serious complications such as borderline ischaemic stroke have been reported. Although no standardized treatment regime exists, one commonly described but unproven therapy is parenteral or oral application of the calcium channel blocker nimodipine. Here, we report on a case of RCVS, where a progressive course prompted intra-arterial application of nimodipine, which resolved vasoconstriction immediately. We discuss the use of intra-arterial nimodipine application as a potential emergency treatment for a complicated or treatment-refractory course of RCVS.     

颅内多发性动脉瘤患者术前破裂责任动脉瘤的判断

目的 探讨多发性颅内动脉瘤患者诊疗过程中误判破裂责任动脉瘤的原因和解决方法.方法 对2003-2009年上海市浦东新区浦南医院收治的25例颅内多发性动脉瘤致自发性蛛网膜下腔出血(SAH)患者的临床资料进行回顾分析,术前根据Nehls等报道的评判原则诊断破裂责任动脉瘤,被确定破裂的责任动脉瘤均在48 h内完成夹闭手术治疗.不能通过一次手术入路夹闭治疗的多发动脉瘤则远期进一步手术治疗.结果 25例患者均通过手术证实破裂动脉瘤的确切部位,其中术中确认的责任破裂动脉瘤与术前诊断一致者20例(80%);而术前判断错误者4例(16%),且术后均发生再出血,其中2例因再出血死亡;还有1例诊断不明确.结论 约80%左右的责任动脉瘤可通过术前CT、脑血管造影检查明确诊断,有疑议时应增加检查手段,如CTA或MRI等;术中需对责任动脉瘤进行确认;即使已经发现了责任动脉瘤,也要对其他部位的动脉瘤进行术中确认.

Abstract：

Objective To discuss the reasons of false judgments of localization of the rupture aneurysms and find the way to fix this problem in patients with multiple intracranial aneurysms. Methods The clinical data of 25 consecutive patients, who presented with their first spontaneous subarachnoid hemorrhage and had multiple intracranial aneurysms from 2003 to 2009 in our hospital, were analyzed retrospectively. The rupture aneurysms were determined according to Nehls' method that reported before, and the supposed responsible rupture aneurysms w0ere clipped within 48 hours after hemorrhage in all patients. More aneurysms that could not be accessed in the same surgical session were surgically terated later. Results The location of the rupture aneurysm was verified at the time of surgery in all 25 patients. The concordance rate of the prediction and the reality of the rupture aneurysm was 80% (20/25). Four patients ( 16% ) ,in whom the ruptured aneurysm was not correctly identified,rebled after surgery,and 2 patients died as a result of the rebleeding One patients had no clear diagnosis at the end. Conclusion In the reported cases, about 80% rupture aneurysms could be correctly diagnosed before treatment according to the CT and DSA examinations. If clear diagnosis couldn't be made,additional examinations should be considered, such as CTA or MRI. Rupture aneurysms must be confirmed during the operation and the other aneurysms should be checked to exclude additional responsible aneurysms in all cases.     

脑脊液置换及椎管内注药治疗蛛网膜下腔出血后迟发性脑血管痉挛

目的探讨救治蛛网膜下腔出血(SAH)后迟发性脑血管痉挛(DCVS)的有效方法。方法治疗组用大剂量生理盐水置换脑脊液并椎管内尿激酶注射治疗20例，并与对照1组单纯大剂量生理盐水等量脑脊液置换10例和对照2组单纯小剂量生理盐水置换脑脊液10例进行比较。结果治疗组2、3d症状明显减轻至消失，均未出现DCVS。对照1组中4例5d、6例7d症状减轻至消失，均未出现DCVS。对照2组中2例7d症状减轻至消失，未出现DCVS；8例发生DCVS，其中6例中度病残，2例死亡。结论应用大剂量生理盐水置换脑脊液可快速消除蛛网膜下腔出血患者的急性症状，对DCVS也有较好预防和治疗作用，而早期加用小剂量尿激酶椎管内注射，可以明显提高疗效，同时又不会增加再出血的危险。     

蛛网膜下腔出血患者症状性脑血管痉挛的预测与研究

目的研究蛛网膜下腔出血患者中症状性脑血管痉挛的预测.方法76例蛛网膜下腔出血患者均行脑血管造影、TCD检查、Fisher量表评分,同时对性别、年龄、吸烟、高血压等因素进行分析,研究上述因素是否可对临床症状性脑血管痉挛进行预测.结果脑血管造影及TCD检查时有脑血管痉挛、Fisher分型Ⅲ型、高龄患者临床脑血管痉挛的发生率显著高于对照组,而性别、吸烟、高血压不是脑血管痉挛的危险因素.结论脑血管造影、TCD检查、Fisher量表可以进对临床症状性脑血管痉挛进行预测.     

释放脑脊液对治疗家兔蛛网膜下腔出血后脑血管痉挛的作用

目的探讨家兔蛛网膜下腔出血后释放脑脊液在治疗迟发型脑血管痉挛（DCVS）中的作用及其机制。方法采用枕大池二次注血法制作家兔蛛网膜下腔出血（SAH）模型,动物随机分为SAH组、治疗组和盐水对照组,各组分别于建模后1 d、3 d、5 d、7 d、10 d处死固定,取基底动脉。应用Le ica-Q550CW图像分析系统及软件测量基底动脉周长。数据结果应用统计软件SPSS 13.0进行处理。结果第3天,SAH组和治疗组的血管周长均小于盐水组,第5天治疗组的血管周长大于SAH组,并与盐水组无统计学差异。第7天治疗组血管周长大于SAH组及盐水组。结论 SAH后通过释放脑脊液对DCVS有治疗作用。     

高血糖对动脉瘤出血后症状性脑血管痉挛的影响

目的探讨动脉瘤性蛛网膜下腔出血后血糖水平与症状性脑血管痉挛的发生及预后的关系。方法回顾性分析175例动脉瘤性蛛网膜下腔出血患者的临床特征和血糖水平。用Cox回归来分析入院时血糖的平均值、住院期间血糖的平均值、胰岛素用量、糖尿病病史、Hunt-Hess分级、Fisher分级。结果在175例患者中,53例(30.4%)发生症状性血管痉挛。入院时血糖为(9.7±2.2)mmol/L和住院期间血糖值(9.2±1.4)mmol/L相比,显著升高。多因素分析显示,住院期间血糖值[RR=1.02,95%可信区间(CI)1.02~1.04],Hunt-Hess≥3级[RR=2.22,95%可信区间(CI)1.20~3.98],FisherⅢ级[RR=1.26,95%可信区间(CI)1.14~3.0]是影响症状性血管痉挛发生的危险因素。结论高血糖是症状性脑血管痉挛发生的相关因素,控制高血糖可减少症状性脑血管痉挛的发生,改善临床预后。     

Posterior reversible leukoencephalopathy syndrome presenting in a post-partum, 25-year-old-female with concomitant subarachnoid hemorrhage

Hinchey et al., first described that posterior reversible leukoencephalopathy syndrome has having a unique neuroradiographical finding of vasogenic edema and clinical symptoms including headache, altered mental status, seizure and visual disturbances in 1996. We present a rare case of posterior reversible leukoencephalopathy syndrome in a 2-week, post-partum G2P2A0 (normal spontaneous vaginal delivery at forty-weeks, without complications) 25-year-old-female with subarachnoid hemorrhage.     

Hydrocephalus after subarachnoid hemorrhage:A metaanalytic comparison of aneurysm treatments

AIM: To compare two treatments for ruptured cerebral aneurysm with reference to the relative risk of developing hydrocephalus.METHODS: We reviewed the English language literature on the risk of developing hydrocephalus after aneurysm treatment.Data were divided by type of study(randomized controlled trial,cohort trial,nonrandomized comparison,prospectively- and retrospectively-collected observational study).They were also divided by type of aneurysm treatment(microvascular- clipping,or endovascular- coiling).Additional predictive variables collected for each publication were average age,gender distribution,measures of hemorrhage volume and subarachnoid hemorrhage severity,aneurysm locations,time to treatment,duration of follow-up and date of publication.We employed meta-analysis to calculate pooled risk ratios of developing hydrocephalus in cases receiving aneurysm clipping vs those receiving coiling.Meta-regression was used to correct pooled results for covariates.RESULTS: Because indications for the two treatments are different,there is little clinical equipoise for treating most cases.The single randomized,controlled trial dealt with a small subset of ruptured aneurysms.Neither this nor pooled values from other studies which compared the two treatments had the power to demonstrate significant differences between the two treatments.Nor was there an apparent difference when observational data were meta-analytically pooled.However,when meta-regression was used to correct for predictive variables known to differ between the two treatment groups,a highly-significant difference appeared.Coiling is used more commonly in older,sicker patients with aneurysms in certain locations.These cases are more likely to develop hydrocephalus.When corrected for these covariates,the risk of hydrocephalus was found to be significantly lower in coiled vs clipped cases(P = 0.014).CONCLUSION: Pooled observational data were necessary to demonstrate that coiling ruptured cerebral aneurysms is associated with a lower risk of developing hydrocephalus than is clipping.