吉兰-巴雷综合征患者的肌电图测试与分析

目的:探讨神经传导速度、F波和针极肌电图对吉兰-巴雷综合征的诊断价值。方法:检测32例吉兰-巴雷综合征患者的正中神经、尺神经、腓总神经、胫神经、腓浅神经和腓肠神经的传导速度以及正中神经和胫神经F波的潜伏期测定,小指展肌、拇短展肌、肱二头肌、股直肌和胫前肌的针极肌电图检测。结果:吉兰-巴雷综合征的运动神经传导速度异常率为72.6%,感觉神经传导速度的异常率为57.8%,F波潜伏期的异常率为85.9%,结论:肌电图的测定可为吉兰-巴雷综合征的诊断提供客观依据,并可发现亚临床周围神经病变,神经电生理的检查对吉兰-巴雷综合征的诊断具有重要的参考价值。     

吉兰-巴雷综合征患者肌电图及神经传导速度检测

目的：探讨吉兰-巴雷综合征（GBS）的电生理改变特点。方法：对32例GBS患者进行肌电图（EMG）、神经电图及F波检测。测定运动神经传导速度（MCV）、感觉神经传导速度（SCV）及末端潜伏期（Lat）和波幅（Amp）;测定F波最短潜伏期、出现率。结果：肢体神经远端潜伏期延长占53．7％,MCV减慢占68．7％。F波异常占91．8％,SCV减慢占64．4％,EMG提示神经源性改变占71．9％。结论：GBS为广泛的周围神经损害,神经肌电图电生理检测在GBS诊断中起着重要的作用,对于吉兰一巴雷综合征的诊断及预后具有重要价值。     

吉兰-巴雷综合征40例患者的神经肌电图检测

吉兰-巴雷综合征（GBS）是神经系统由体液和细胞共同介导的单相性自身免疫性疾病，病变范围广泛而弥散，主要侵犯脊神经根、脊神经和颅神经，有时也累及脊膜、脊髓及脑部。神经电生理检查为其重要的诊断手段。本文报告我院自2003年7～12月就诊住院的40例GBS患者的神经电生理检测结果。     

吉兰-巴雷综合征患者的神经肌电图研究

目的 :探讨吉兰 巴雷综合征 (GBS)中急性炎性脱髓鞘性多发性神经根神经病 (AIDP)和慢性炎性脱髓鞘性神经根神经病 (CIDP)的电生理改变特点。方法 :对 38例GBS患者进行肌电图(EMG)、神经电图及F波检测。测定运动神经传导速度 (MCV)、感觉神经传导速度 (SCV)及末端潜伏期(Lat)和波幅 (Amp) ;测定F波最短潜伏期、出现率。对比CIDP组与AIDP组患者的电生理改变特点。结果 :CIDP与AIDP中EMG去神经电位出现率分别为 81%和 5 0 % ,运动单位电位 (MUP)多相波分别为 6 9%和 37% ,大力收缩时募集相呈单纯或单混相分别为 73%和 31% ;MCV减慢分别为 6 9%和 5 4% ,Lat延长分别 5 7%和 32 % ,Amp下降或引不出分别为 5 5 %和 40 % ;SCV减慢分别为 5 7%和 19% ,Lat延长为 42 %和 19% ,Amp下降或引不出为 43%和 10 % ,上述参数在两组间比较均具有显著统计学意义。结论 :CIDP与AIDP两组EMG均表现有广泛性周围性神经源性损害的电生理特征。CIDP组EMG、MCV、SCV等参数异常改变均较AIDP显著 ,且表现为运动与感觉同时受累。AIDP患者F波异常发生率明显高于MCV异常发生率 ,F波与MCV同时检测在吉兰 巴雷综合征诊断中有着重要的互补作用。     

神经肌电图在慢性吉兰-巴雷综合征诊断中的应用

慢性吉兰—巴雷综合征神经科临床较常见,起病隐袭,无明显前驱症状。临床主要表现为肌无力、肌萎缩,部分患者感觉障碍不明显,容易误诊为肌病、运动神经元病、颈椎病等,延误治疗。因此,及时行神经肌电图检测,明确诊断,给予正确治疗是非常重要的。现报告曾被误诊的3例典型病例如下。     

腕管综合征的肌电图临床分析

目的:探讨肌电图检查在腕管综合征诊断中的价值。方法:对25例临床诊断为腕管综合征患者进行神经传导和肌电图检查,分别记录感觉传导速度(SCV),远端潜伏期(DML),运动传导速度(MCV),环指潜伏期(Lat)差值比较法,肌电图(EMG)各项数据,并进行统计学分析。结果:25例患者中有2例正中神经感觉传导速度测定未引出诱发电位,21例正中神经SCV减慢,异常率92%,20例正中神经DML延长,异常率80%,2例正中神经MCV减慢,25例Lat差值均大于0.4 ms,异常率100%,5例拇短展肌见失神经电位和(或)运动单位电位时限延长呈神经源性损害,异常率20%,尺神经传导均在正常范围。结论:肌电图检查可确定正中神经的损伤程度,从而进一步更好的指导临床诊断及疗效观察,对腕管综合征患者的定位诊断和鉴别诊断具有重要价值。     

肌电图结合临床跟踪吉兰-巴雷综合征体会（附1例报告）

吉兰-巴雷综合征（GBS）是以周围神经和神经根脱髓鞘为主要病理改变的自身免疫性疾病,肌电图检查对GBS的诊断、分型及预后判断均具有重要价值。临床上,GBS患者能坚持多次随访肌电图不多见。笔者有幸遇到1例,坚持2年随访,肌电图检查6次,观察到肌电图检查结果与临床表现不一致,今报告如下：     

运动神经传导速度测定对腰骶神经综合征的诊断价值

对４８例腰骶神经根综合征及２４名正常者进行腰骶神经运动传导速度测定，同时对病人进行腓神经运动传导速度与肌电图测定。两组腰骶神经运动传导速度分别为４４．４５±１３．４ｍ／ｓ和５６．７５±３．８ｍ／ｓ，有显著性差异（Ｐ＜０．０１）。病人组肌电图均示神经源性损害，腓神经运动传导速度正常。本文揭示腰骶神经运动传导速度对腰骶根综合征有诊断价值。     

吉兰-巴雷综合征患者神经电生理检测结果分析

目的：探讨吉兰-巴雷综合征（GBS）患者的神经电生理特点。方法：对30例GBS患者进行运动神经传导速度（MCV）、感觉神经传导速度（SCV）、肌电图（MMG）及F波检测，共检测运动神经180条，感觉神经180条，肌肉60块，F波40条神经，并对结果进行分析。结果：在被检测的180条运动和感觉神经中，MCV、SCV减慢分别为70．0％和51．6％，潜伏期（Lat）延长分别为49．4％和44．4％，波幅（Amp）下降或引不出波形分别为58．3％、13．9％和14．4％、27．8％。EMG提示神经源性损害。F波潜伏期（Lat）延长为47．5％，出现率下降为57．5％，未引出波形为15．0％。结论：GBS为广泛的周围神经损害，存在以脱髓鞘为主伴有轴索变性的电生理改变。神经电生理检测对GBS的诊断是极为重要的诊断手段。     

吉兰-巴雷综合征患者神经电生理检测与临床观察

目的:研究吉兰-巴雷综合征(GBS)患者神经电生理检测结果与临床的关系.方法:对42例GBS患者进行运动神经传导速度(MCV),感觉神经传导速度(SCV)、F波、针极肌电图(EMG)检测两次(入院时为第一次和1个月后为第二次),第一次共检测266条运动神经(四肢及面部),168条感觉神经,84次F波,141块肌肉(四肢及面部)的EMG,1个月后复查并对结果进行分析.观察入院时和1个月后EMG检测结果与临床症状改变的关系.结果:首次检测上、下肢运动神经远端潜伏期延长者占所检运动神经的71%,MCV不同程度减慢者占59%,运动神经波幅降低者占37%,SCV不同程度减慢者占所检感觉神经的54%,F波异常者占90%,EMG为神经原性损害者占所检肌肉的35%.第二次复查神经电生理完全恢复正常者占74%.结论:GBS为广泛的周围神经损害,其病理改变以脱髓鞘损伤为主,部分合并有轴索损伤,在病程早期神经电生理检测就可以有阳性发现,但后期其恢复滞后于临床症状的恢复,特别是合并有轴索损伤者.神经电生理检测对GBS的诊断具有重要价值.     

肌电图、神经电图在格林—巴利综合征中的应用

目的:探讨肌电图(EMG)、神经电图在格林-巴利综合征(GBS)中的特点。方法:对31例GBS患者进行运动神经124条、感觉神经93条及128块肌肉进行EMG;运动神经传导速度(MCV);远端潜伏期;F波的出现率、潜伏期、波形;感觉神经传导速度(SCV)及一过性离散(TD)测定。结果:每例均有3条以上神经受累;远端潜伏期延长58％;MCV减慢70％;SCV减慢50％;F波异常率73％;TD异常55％。EMG出现自发电位占32％。结论:GBS为广泛的周围神经远端,近端损害,感觉运动均受累,存在以脱髓鞘为主伴有轴索变性的特点。EMG、神经电图的无创性,简便及可重复性使其成为GBS极为重要的诊断手段。     

Hypocretin/orexin and narcolepsy: new basic and clinical insights

Narcolepsy is a chronic sleep disorder, characterized by excessive daytime sleepiness (EDS), cataplexy, sleep paralysis and hypnagogic hallucinations. Both sporadic (95%) and familial (5%) forms of narcolepsy exist in humans. The major pathophysiology of human narcolepsy has been recently discovered based on the discovery of narcolepsy genes in animals; the genes involved in the pathology of the hypocretin/orexin ligand and its receptor. Mutations in hypocretin-related genes are rare in humans, but hypocretin ligand deficiency is found in a large majority of narcolepsy with cataplexy. Hypocretin ligand deficiency in human narcolepsy is probably due to the post-natal cell death of hypocretin neurones. Although a close association between human leucocyte antigen (HLA) and human narcolepsy with cataplexy suggests an involvement of autoimmune mechanisms, this has not yet been proved. Hypocretin deficiency is also found in symptomatic cases of narcolepsy and EDS with various neurological conditions, including immune-mediated neurological disorders, such as Guillain–Barre syndrome, MA2-positive paraneoplastic syndrome and neuromyelitis optica (NMO)-related disorder. The findings in symptomatic narcoleptic cases may have significant clinical relevance to the understanding of the mechanisms of hypocretin cell death and choice of treatment option. The discoveries in human cases lead to the establishment of the new diagnostic test of narcolepsy (i.e. low cerebrospinal fluid hypocretin-1 levels for ‘narcolepsy with cataplexy’ and ‘narcolepsy due to medical condition’). As a large majority of human narcolepsy patients are ligand deficient, hypocretin replacement therapy may be a promising new therapeutic option, and animal experiments using gene therapy and cell transplantations are in progress.     

骨间后神经卡压综合征的临床及神经电生理诊断

目的：探讨骨间后神经卡压综合征的临床和神经电生理特点。方法：回顾性分析2006年2月至2010年2月临床疑诊骨间后神经卡压综合征患者的资料,结合神经电生理检查确诊共23例,分析其临床和神经电生理诊断特点。结果：所有患者虽然临床发病过程、病情轻重有所不同,但23例肌电图都有异常：重点检查食指固有伸肌、伸指总肌及尺侧伸腕肌基本均有自发电位出现。18例（78％）运动传导反应异常;运动传导远端潜伏期、波幅、传导速度异常分别是16例（70％）、17例（74％）和12例（52％）;桡神经浅支感觉传导正常。结论：神经电生理检查是诊断骨间后神经卡压综合征的有效方法,对临床体征不明确、定位困难者,早期肌电图检查更为重要。     

手足口病并发脑干脑炎19例临床分析

目的总结广东地区手足口病并发脑干脑炎患儿的临床特征,为手足口病并发脑干脑炎的诊断和治疗提供依据。方法收集2008年5～7月诊断为手足口病并发脑干脑炎患儿的临床资料,分析临床表现、实验室检查、神经电生理检查、影像学检查、治疗及转归等特征。选择同期诊断为手足口病并发病毒性脑炎的患儿作为对照组,进行脑干脑炎危险因素的单因素和多因素Logistic回归分析。结果19例手足口病并发脑干脑炎患儿进入分析。①临床表现：19例患儿均有发热和皮疹;神经系统症状多见于肢体震颤（15例）,眼球异常运动（游动或上翻）（12例）,烦躁和惊恐（8例）;脑神经损害多见于单侧脑神经损害（14例）,舌咽和迷走神经麻痹（9例）。15例出现呼吸衰竭,3例出现肺出血。②实验室检查：外周血WBC升高8例;血糖在正常范围;脑脊液压力升高4例。③神经电生理检查：8例EEG异常,14例脑干听觉诱发电位（BAEP）异常。④影像学检查：3例胸部X线检查提示双肺渗出性病变;15例行头颅CT检查均未见异常;3例头颅MR I检查提示脑干异常信号病灶。⑤转归：2例死于中枢性呼吸和循环衰竭,余17例治愈出院,随访6个月未见神经系统症状。⑥单因素Logistic回归分析显示,肢体震颤、呼吸节律改变、呼吸衰竭、眼球异常运动（游动或上翻）、饮水呛咳、口角歪斜、舌体震颤、末梢循环不良、交叉瘫及BAEP异常与脑干脑炎的发生有关;多因素Logistic回归分析显示,肢体震颤和眼球异常运动（游动或上翻）和BAEP异常是手足口病并发脑干脑炎的危险因素（OR分别为436.9,52.2和93.5）。结论外周血WBC、血糖以及脑脊液检查对手足口病并发脑干脑炎的诊断不具有特异性。手足口病患儿如出现肢体震颤、眼球异常运动（游动或上翻）等症状,应警惕脑干脑炎的发生。BAEP检查对脑干损伤有较好的诊断价值。及时和规范治疗后大多数患儿的预后良好。     

Connective tissue spectrum abnormalities associated with spontaneous cerebrospinal fluid leaks: a prospective study

We aimed to assess the frequency of connective tissue abnormalities among patients with cerebrospinal fluid (CSF) leaks in a prospective study using a large cohort of patients. We enrolled a consecutive group of 50 patients, referred for consultation because of CSF leak. All patients have been carefully examined for the presence of connective tissue abnormalities, and based on findings, patients underwent genetic testing. Ancillary diagnostic studies included echocardiography, eye exam, and histopathological examinations of skin and dura biopsies in selected patients. We identified nine patients with heritable connective tissue disorders, including Marfan syndrome, Ehlers–Danlos syndrome and other unclassified forms. In seven patients, spontaneous CSF leak was the first noted manifestation of the genetic disorder. We conclude that spontaneous CSF leaks are associated with a spectrum of connective tissue abnormalities and may be the first noted clinical presentation of the genetic disorder. We propose that there is a clinical basis for considering spontaneous CSF leak as a clinical manifestation of heritable connective tissue disorders, and we suggest that patients with CSF leaks should be screened for connective tissue and vascular abnormalities.     

Clinical characteristics and laboratory assay of adult Japanese encephalitis patients in an outbreak in Yuncheng, Shanxi Province, 2006]

OBJECTIVE: To study the clinical and laboratory characteristics of adult Japanese encephalitis (JE) patients in a JE outbreak in Yuncheng, Shanxi Province in 2006. METHOD: All the clinical data from the Second People's Hospital in Yuncheng city were analyzed, part of patients' sera and cerebrospinal fluid were tested by serology and RT-PCR. RESULTS: The majority of patients were middle-aged and elderly, 77.8% (35/45) of the total cases were more than 40 years old. Severe and fulminating type cases accounted for 60.0% (27/45). Most patients had underlying diseases. IgM antibody to JE virus (JEV) in serum was positive in each of the 45 patients analyzed and 4-fold or greater rise in sera neutralization antibody titer were found in convalescent serum. JEV nucleic acid was positive in part of cerebrospinal fluid specimens. CONCLUSION: Viral encephalitis emerged in Yuncheng city, Shanxi Province was Japanese encephalitis B, and most of the cases belonged to elderly group.     

Prospective Validation of a Diagnosis Model as an Aid to Therapeutic Decision-Making in Acute Meningitis

 The aim of this study was to validate a diagnosis model that provides pABM, the probability of bacterial versus viral meningitis, based on four parameters collected at the time of first lumbar tap: cerebrospinal fluid protein level, cerebrospinal fluid polymorphonuclear cell count, blood glucose level, and leucocyte count. The model was evaluated prospectively as an aid to therapeutic decision-making in 109 consecutive patients with acute meningitis and negative cerebrospinal fluid Gram stain. In each case pABM was computed before a therapeutic decision and three diagnoses were established successively: (i) clinical evaluation, i.e. before pABM computation (bacterial meningitis, viral meningitis, or meningitis of undetermined origin); (ii) computation of pABM (viral meningitis if pABM<0.1, bacterial meningitis otherwise); and (iii) determination of definitive diagnosis (bacterial meningitis: positive cerebrospinal fluid culture; viral meningitis: negative cerebrospinal fluid culture, no other aetiology and no treatment; meningitis of undetermined origin: cases fitting neither of the first two diagnoses). The computed diagnosis was viral meningitis in 78 of the 80 cases diagnosed definitively as viral meningitis, and bacterial meningitis in four of the five cases diagnosed definitively as bacterial meningitis. Negative and positive predictive values and accuracy of the model were 98.7%, 66.7%, and 96.5%, respectively. The clinical diagnosis was undetermined in 22 cases, 15 of which were diagnosed definitively as viral cases; in all of these 15 cases, the computed diagnosis was viral meningitis, leading the physician to refrain from starting antibiotics in all of them. The results confirm that the model evaluated is reliable and aids in the identification of patients in whom antibiotics can be safely avoided.     

Imaging examinations in children with hydrocephalus

Hydrocephalus is characterized by an imbalance of cerebrospinal fluid (CSF) formation and absorption. It is manifested as a dilatation of the ventricular system. About 55% of all hydrocephalus cases have congenital origin. There are two types of hydrocephalus: communicating and non-communicating with subarachnoid space and the diagnosis depends on the computed tomography (CT) and magnetic resonance (MR) images. The treatment is different for each type of the hydrocephalus. Causes and symptoms of hydrocephalus are changing with the patient's age. Before the age of two we can observe progressive enlargement of the head and widened anterior fontanel. Ophthalmological examination reveals optic nerves atrophy. Children older than two years with hydrocephalus and obliterated anterior fontanel have normal head circumference. They may often present clinical symptoms such as the atrophy of optic nerves and papilloedema of optic disc. The most common reason of hydrocepahalus in children before two years of age is intraventricular haemorrhage in the perinatal period whereas in children older than two years is inflammatory process. Imaging examinations are needed not only to diagnose hydrocephalus but also to assess enlargement of the ventricular system during the therapy.     

Assessment of the usefulness of X-ray myelography and magnetic resonance myelography,performed with an open low-field device,in diagnosing perinatal preganglionic injuries of the brachial plexus

Introduction

The goal is to assess the usefulness of diagnostic imaging in diagnosing perinatal preganglionic injuries of the brachial plexus.

Material and methods

The clinical material included 40 children of both genders, aged 2 to 35 months. The authors analysed the results of diagnostic imaging examinations (myelography in 20 cases and magnetic resonance [MR] myelography in 20 cases), intraoperative view and clinical course.

Results

In 13 out of 40 (32.5%) examined children, no evidence of avulsion of the roots of the spinal nerves was found either by diagnostic imaging or during the surgery. In 3 cases (7.5%) with avulsed roots of the spinal nerves, the diagnostic imaging and intraoperative appearance were in agreement. Total agreement of the diagnostic imaging and intraoperative view was found in 40% of cases. In 9 patients (22.5%) suspected avulsion of roots of the spinal nerves was not confirmed during the surgery. However, the further clinical course of the disease in these cases indicated high probability of avulsion of roots without their pull-out from the intervertebral foramens. In the remaining cases, the findings were as follows: false positive results – 7 (17.5%), false negative results – 1 (2.5%), results underestimating injury – 3 (7.5%), results overestimating injury – 2 (5%).

Conclusions

It was determined that the usefulness of pre-operative diagnostic imaging is limited. Due to the risk of occurrence of false positive and false negative results, final decisions concerning selection of the surgical technique must be based on the analysis of the intraoperative view and preoperative clinical symptoms.