12例基底动脉极度过长弯曲临床特点分析

目的研究基底动脉极度过长弯曲是否与头晕有关。方法收集作者医院住院的基底动脉弯曲患者12例,分析其临床表现。入组标准为经MRA检查显示基底动脉弯曲成半圆,其程度相当于Giang等关于基底动脉主干过长弯曲分类标准的2~3级。结果12例患者中有11例反复发生头晕(91%),1例无头晕者存在桥脑梗死。结论基底动脉极度过长弯曲者极易发生头晕。     

椎基底动脉延长扩张症与前循环障碍关系的研究(附5例报告)

目的观察椎基底动脉延长扩张症(VBD)与前循环障碍的关系。方法回顾性分析VBD与颈内动脉闭塞/狭窄并存病例的临床及影像学资料。结果 5例患者中,临床表现为双眼同向偏盲或视力障碍3例,一侧肢体运动障碍2例,Gerstmann综合征1例。CT/MRI显示分水岭性脑梗死(后型)2例,大脑前及大脑中动脉供血区梗死各1例,无明显改变1例;CTA/MRA显示同时伴有一侧颈内动脉闭塞3例,双侧颈动脉狭窄及发育不良(烟雾病)各1例;基底动脉长度为34.0~39.5 mm,直径为4.6~4.8 mm;颅内椎动脉最大处直径为4.4~4.8 mm。结论 VBD可与颈动脉闭塞或狭窄共存,临床表现更为复杂。长期的前循环障碍可能是VBD形成的重要促发因素,应兼顾前后循环状况制定治疗方案。     

Vertebrobasilar Dolichoectasia Causing Symptomatic Hydrocephalus Due to Third Ventricular Compression

Vertebrobasilar dolichoectasia sometimes presents with symptoms related to mass effect like cranial nerve palsies, or with ischemia or hemorrhage. Symptomatic hydrocephalus as a complication of vertebrobasilar dolichoectasia is extremely uncommon. Furthermore, there are few cases of vertebrobasilar dolichoectasia, in which cerebrospinal fluid flow disorder mechanisms are clearly demonstrated in neuroimaging findings. Here, we describe a patient with vertebrobasilar dolichoectasia who presented with symptomatic hydrocephalus due to direct compression against the third ventricle, which was immediately relieved by ventriculoperitoneal shunt. This patient exhibited a progressive clinical course of acute hydrocephalus; however, a subclinical ventricular dilatation may have been present before the onset. Therefore, a careful follow-up is warranted to treat symptomatic hydrocephalus that may develop in patients with vertebrobasilar dolichoectasia.     

Brachium Pontis Stroke Revealing Neurofibromatosis Type‐2

NF2 is an autosomal dominant disorder with neuroectodermal dysplasia. Most patients present with characteristic clinical tumors during or beyond the adolescent age group. The diagnosis is mainly clinical. Vasculopathy is rarely associated with NF2. Vascular complication as the presenting symptom in NF‐2 is unknown. We report a case of a 2‐year‐old child with no prior family history of neurofibromatosis presenting with ataxia and brain‐stem stroke .     

The Effect of Microvascular Decompression for Hemifacial Spasm Caused by Vertebrobasilar Dolichoectasia

Objective

Hemifacial spasm (HFS) caused by vertebrobasilar dolichoectasia (VBD) is very rare, and in theses cases, it is difficult to decompress the nerve from its vascular compression. The objective of this study was to investigate the outcome of microvascular decompression (MVD) for HFS caused by VBD.

Methods

There were 10 patients of HFS caused by VBD at our hospital between September 1978 and September 2008. We evaluated magnetic resonance angiography (MRA) and time of flight magnetic resonance imaginge (TOF MRI) findings using the criteria for VBD. We compared the clinical outcomes of MVD for the 10 patients with VBD with the overall outcomes of the total 2058 MVDs performed for HFS.

Results

The results of MVD for HFS caused by VBD were successful in 90.9% of cases. The postoperative complication rate in VBD was 45.5%. Offending vessels in patients with VBD were identified visually during surgery. Adverse effects after MVD were found in 4 patients. We found that the diameter of VBD was significantly greater in patients with complications than in those with no complications (p=0.028).

Conclusion

Our data shows that MVD may be a good treatment modality for HFS caused by VBD but care must be taken to avoid adverse effects from the procedure. It is important to detach the dolichoectatic artery from its surrounding structures sufficiently to allow it to be easily movable. In addition, attempts should be made to lessen the retraction of the cerebellum during release of the dolichoectatic artery.     

Ⅰ型神经纤维瘤病合并烟雾综合征的临床表现及治疗预后

【摘要】
目的 初步探讨Ⅰ型神经纤维瘤病（neurofibromatosis type Ⅰ,NFⅠ）合并烟雾综合征（moyamoya syndrome,MMS）患者的临床表现、影像学特征及治疗预后。
方法 连续收集中国人民解放军第307医院神经外科2003年1月～2012年3月收治的NFⅠ-MMS患者,对其首发症状、首发年龄,血管病变的铃木分期等影像学特征、治疗方式,随访期内症状进展情况等进行分析。
结果 共收集4例患者,男女性别比为1∶3,症状首发年龄3例在18岁以下,1例为23岁。首发症状中,2例表现为短暂性脑缺血发作（transient ischemic attack,TIA）,1例表现为头痛,1例表现为脑梗死。4例患者的6侧病变铃木分期分别为,1期2侧,2期1侧,4期2侧,6期1侧。3例患者行血管重建手术,1例未行手术治疗,随访时间最短为10个月,最长为72个月,随访期内3例手术患者症状改善明显。
结论 NFⅠ-MMS大多好发于儿童时期,首发症状以脑缺血症状为主,血管病变程度可表现为铃木分期的各个阶段,血管重建手术可能对改善临床症状有帮助,有助于改善患者预后。     

Nephrotic Syndrome is Associated with Increased Risk of Ischemic Stroke

Background: To determine if the nephrotic syndrome (NS) is an independent risk factor of ischemic stroke. Methods: This is a retrospective nationwide cohort study through an analysis of the National Health Insurance Research Database in Taiwan. To evaluate the risk of stroke, the corresponding controls were selected at a 4:1 ratio in the number of subjects, and they were matched with the study group in age, gender, Charlson comorbidity index (CCI), and index date. Results: From a total of 16,245 surveyed subjects, ischemic stroke occurred in 1235 (7.6%) and hemorrhagic stroke in 129 (.74%) of them. The incidence of ischemic stroke was significantly higher in patients with NS (n = 3496) compared to control patients without NS (n = 13,984) (9.92 versus 7.10, per 1000 person-year, P < .001). In the multivariate analysis, the overall adjusted hazard ratio (aHR) of stroke in NS patients was 1.37 (95% CI, 1.21-1.54, P < .001). The risk factors of ischemic stroke were NS (aHR, 1.38 [95% confidence interval {CI}, 1.21-1.57]; P < .001), age greater than 45 years (aHR, 7.98 [95% CI, 6.47-9.48]; P < .001), male gender (aHR, 1.23 [95% CI, 1.10-1.38]; P < .001), CCI greater than or equal to 1 (aHR ≥ 1.25 in different CCI score groups, all at P ≤ .003), ischemic heart disease (aHR, 1.95 [95% CI, 1.67-2.29]; P < .001), heart failure (HR, 1.77 [95% CI, 1.30-2.42]; P < .001). Risk factors of hemorrhagic stroke were those aged greater than 45 years, or with systemic lupus erythematosus, but not NS. Conclusions: We provided the first evidence that patients with NS had an increased risk of ischemic stroke.     

Cerebral Microbleeds are Associated with Higher Mortality Among Ischemic Stroke Patients

Background

Several studies have shown that cerebral microbleeds (CMBs) increase the risk of long-term stroke-related mortality. The purpose of this study was to determine if the existence and burden of CMBs are a predictor of in-hospital death among patients with acute ischemic stroke (AIS).

Neurofibromatosis type 1 and infantile spasms

Background  There is no agreement on the prevalence, natural history and outcome of infantile spasms (IS) in neurofibromatosis type 1 (NF1). By contrast, its prevalence and outcome are well characterised in the setting of other neurocutaneous disorders (e.g. tuberous sclerosis). Materials and methods  The aim of the present study was to try to establish a genotype–phenotype correlation in IS in the setting of NF1. A retrospective (years 1990–2000) and prospective (years 2000–2006) study in three paediatric centres in Italy were taken as referral populations for: (1) children with NF1 and (2) neurological problems in childhood. Results  Ten NF1 patients have had IS. The calculated population-based: (1) prevalence of IS in NF1 (0.76%) was higher than the reported frequency of IS in the general population (0.02–0.05%) and (2) frequency of NF1 in the IS series in two out of three centres (0.62–0.90%) was lower than the estimated frequencies in the literature (1.5–3.0%). Patients had psychomotor delay preceding the spasms (50%), symmetrical spasms (50%), typical (80%) and modified (20%) hypsarrhythmia and foci of spikes and waves and a good response to corticosteroid treatment (50%). Outcome was good in 30%. Imaging revealed high-signal foci in atypical locations (sub-cortical and central brain regions). Deoxyribonucleic acid analysis revealed three novel NF1 gene mutations without genotype–phenotype correlation. Conclusion  Even though the combination of IS and NF1 does not seem to be coincidental, it is certainly an unusual event in NF1—rarer than in other neurocutaneous disorders. Spasms in NF1 are not associated with specific genetic defects.     

Hypertension Is Associated With Increased Mortality in Children Hospitalized With Arterial Ischemic Stroke

BackgroundRecently a single-center study suggested that hypertension after stroke in children was a risk factor for mortality. Our goal was to assess the association between hypertension and outcome after arterial ischemic stroke in children from a large national sample.MethodsUsing the Healthcare Cost and Utilization Project Kids' Inpatient Database, children (1-18 years) with a primary diagnosis of ischemic stroke (International Classification of Diseases, Ninth Revision [ICD-9] codes 433-437.1) who also had a diagnosis of elevated blood pressure (ICD-9 code 796.2) or hypertension (ICD-9 codes 401 and 405) from 2003, 2006, and 2009 were identified. Clinical characteristics, discharge outcomes, and length of stay were assessed. Multivariable logistic regression was used to assess the relationship between hypertension and in-hospital mortality or discharge outcomes.ResultsOf 2590 children admitted with arterial ischemic stroke, 156 (6%) also had a diagnosis of hypertension. Ten percent of children with hypertension also had renal failure. Among patients with arterial ischemic stroke, hypertension was associated with increased mortality (7.4% vs 2.8%; P = 0.01) and increased length of stay (mean 11 ± 17 vs 7 ± 12 days; P = 0.004) compared with those without hypertension. After adjusting for age, sex, intubation, presence of a fluid and electrolyte disorder, and renal failure, children with hypertension had an increased odds of in-hospital death (odds ratio 1.2, 95% confidence interval [1.1-3.3, P = 0.04]).ConclusionHypertension was associated with an increased risk of in-hospital death for children presenting with arterial ischemic stroke. Further prospective study of blood pressure in children with stroke is needed.     

高血糖与缺血性卒中

缺血性卒中是严重危害人类健康的致死性和致残性疾病。近年来，糖尿病患病率在世界范围内急剧增高。大多数研究资料均支持高血糖是卒中的独立危险因素，可促进卒中发病，加重卒中患者的病情，影响其神经功能的恢复和预后。有关高血糖的危害，已有较多基础研究，但临床研究则相对较少。文章就高血糖与缺血性卒中关系做了综述。     

High-on-Aspirin Platelet Reactivity Differs Between Recurrent Ischemic Stroke Associated With Extracranial and Intracranial Atherosclerosis

Background and PurposeIschemic stroke recurs despite the use of antiplatelet agents. Various mechanisms are involved in recurrence due to intracranial atherosclerosis (ICAS) and extracranial atherosclerosis (ECAS). High-on-aspirin platelet reactivity (HAPR) may differ between recurrent stroke due to ICAS and ECAS.MethodsPatients with recurrent ischemic stroke as a result of large-artery atherosclerosis despite taking aspirin were enrolled consecutively. Ischemic stroke was classified as stroke due to ICAS or ECAS according to the location of the culprit stenosis. An aspirin reaction units (ARU) value of >550 IU was defined as HAPR. HAPR and its associated factors were compared between the two groups and also considering the mechanism of stroke.ResultsAmong the 190 patients with recurrent stroke (111 with ICAS and 79 with ECAS), 36 (18.3%) showed HAPR. The ARU value was higher in the ECAS than the ICAS group (492±83 vs. 465±78, mean±standard deviation; p=0.028), as was the proportion of patients with HAPR (27.8% vs. 12.6%, p=0.008). Being male and having stroke due to ECAS (reference=stroke due to ICAS: odds ratio=5.760; 95% confidence interval=2.154–15.403; p<0.001) was independently associated with HAPR. The ARU value differed according to the stroke mechanism, and was highest in those with artery-to-artery embolism. Artery-to-artery embolism was independently associated with HAPR in both the ICAS and ECAS groups.ConclusionsRecurrent stroke due to ECAS was more strongly associated with HAPR and insufficient antiplatelet inhibition than was that due to ICAS. Artery-to-artery embolism was associated with HAPR in recurrent ischemic stroke as a result of ICAS or ECAS.