米勒·费希尔综合征的临床研究

目的探讨米勒·费希尔综合征的临床特点并对其进行综述。方法回顾分析8例米勒.费希尔综合征患者的临床特点。结果主要神经系统症状:头晕,恶心呕吐,视物模糊,视物成双,四肢无力,手足发麻感,行走不稳。主要神经系统体征:瞳孔直接及间接对光反射消失,双侧眼外肌麻痹,水平眼震,周围性面瘫,共济失调,深、浅感觉减退,腱反射减弱或消失。首发症状主要表现为头晕、视物成双和四肢无力。脑脊液蛋白升高8例,脑脊液细胞数无升高病例。肌电图检查示神经源性损害4例,脑电图异常3例。CT和MRI检查发现异常1例。激素和人血免疫球蛋白同时使用可有效缓解病情,7例患者出院时症状明显好转,1例不明显。结论米勒.费希尔综合征主要累及周围神经系统,临床表现相似,大多数预后较好。     

伴胸腺增生和胸腺瘤的重症肌无力患者电生理特点

目的 研究伴有胸腺异常增生或胸腺瘤的重症肌无力(MG)与胸腺正常MG患者的电生理指标.方法 对61例MG患者进行低频重复电刺激(RNS)、单纤维肌电图(SFEMG)和胸部CT扫描,对比胸腺增生、胸腺瘤和胸腺正常组的伸指总肌(EDC)和眼轮匝肌(OO)的颤抖值.结果 影像学发现14例胸腺异常,病理证实胸腺瘤7例.眼轮匝肌的MCD值、>55 μs电位对百分比、阻滞电位对百分比3项指标在胸腺正常组和异常组之间分别为(68.23±21.56)μs vs(94.25±224.92)μs,(58.89±27.30)%vs(86.51±214.66)%,(32.08±25.11)%vs(52.24±29.93)%(均P<0.01).结论 RNS阳性率在胸腺正常和异常组之间是相近的,OO的SFEMG检查是较敏感的诊断手段.     

咽-颈-臂变异型吉兰-巴雷综合征的研究进展

<正>咽-颈-臂(pharyngeal-cervical-brachial, PCB)变异型吉兰-巴雷综合征(Guillain-Barré syndrome, GBS)以急性口咽部、颈部及双上肢肌肉麻痹为主要临床表现,伴双上肢腱反射减弱,下肢不受累或轻微受累的周围神经病[1-4]。PCB变异型GBS与Miler-Fisher综合征为同一谱系,是局限性轴索型GBS的一种特殊类型。目前国内外尚无关于PCB变异型GBS患病率的大型流行病学调查。有报     

米勒·费希尔综合征3例报告

１引言米勒·费希尔（MillerFisher）综合征（Fisher综合征）是急性吉兰－巴雷综合征（GBS）的一种特殊类型,我们于１９９５年至１９９８年曾诊治３例Fisher综合征,现报告如下。２病例报告例１男性,２０岁。视物成双、言语不清１２日于１９９５年８月２日入院。半个月前病人患感冒、咳嗽,全身不适,体温３８℃左右,经治疗３日后症状缓解（具体不详）;l周后出现视物成双,言语含糊不清,鼻音重,双眼睑下垂,１２日后出现双眼球活动受限,四肢无力,走路蹒跚,不能走直线。体格检查：一般情况可,神志清晰。双眼睑下垂,双眼球正中…     

Fisher综合征5例分析

Fisher综合征也称Miller-Fisher Syndrome,是急性炎性脱髓鞘性多神经病(AIDP)即格林-巴利综合征的变异型.临床少见,常易误诊,我院1992-01～2003-09收治5例分析如下.     

格林-巴利综合征腰丛神经磁共振成像研究

目的探讨磁共振神经成像(MRN)对格林-巴利综合征(GBS)的诊断和鉴别诊断价值。方法对临床诊断为GBS(21例)、慢性炎症性脱髓鞘性多发性神经病(CIDP,4例)患者及正常人(30例)行腰丛神经MRN检查。正常组观测腰2~5神经根、神经节及神经干、股神经的形态、大小及信号;GBS及CIDP组观察神经及其周围的改变,测量右侧腰4神经根、神经节、神经干及股神经的短轴径线及神经肌肉信号比。结果正常组MRN可清晰显示腰2~5神经的走行,神经节呈圆或椭圆形膨大,大小约为3~6mm,神经根及干呈条状,边缘光滑,大小约为2~5mm,神经节信号高于神经根和干,40%的神经根鞘有少量脑脊液,股神经、闭孔神经、腰骶干的显示率分别为100%、83.3%、43.3%。80.9%的GBS腰丛神经周围有片状模糊高信号,神经根和节大小和信号无明显改变,25%神经干增粗,50%的神经干信号增高,边缘模糊。CIDP神经周围无片状高信号,神经根及节无明显增大,神经干及股神经均明显增粗,信号增高。结论MRN可清晰显示腰丛神经及主要大分支;GBS在神经周围有渗出病变,神经增粗不明显,CIDP神经周围无渗出病变,神经干明显增粗。MRN可作为GBS及CIDP诊断和鉴别诊断的重要手段。     

急性格林-巴利综合征变异型1例

1 病例报告 男,22岁.因双手、双足麻木4 d,行走不能2 d为主诉入院.发病2周前有上呼吸道感染史.既往健康.入院后神经系统检查：神清,懒言,颈强（＋＋＋）,克氏征（＋）,双侧瞳孔等大同圆,对光反射正常,左侧眼裂较右侧大,左侧额纹变浅,左眼睫毛征（＋）,右侧闭目肌力减弱,左侧鼻唇沟变浅,伸舌右偏,双上肢近端肌力Ⅱ级,远端肌力Ⅴ级,双下肢近端肌力Ⅱ级,远端Ⅴ级,左侧病理征阳性,右侧病理征可疑阳性,四肢腱反射减弱,四肢肌张力减低,全身深浅感觉正常,尿潴留.辅助检查：头部CT未见异常.AST 50 U/L,ALT 134 U/L,γ-GT 116 U/L.     

变异型吉兰-巴雷综合征8例临床分析

目的：进一步加深对变异型吉兰-巴雷综合征（GBS）临床和电生理的认识。方法：回顾性总结8例变异型GBS的临床表现，分析神经电生理结果。结果：4例患者以感觉异常为首发症状，2例以双侧周围性面神经瘫痪为主要表现，另2例出现头晕、复视和行走不稳；脑脊液（CSF）检查呈蛋白-细胞分离现象（其中1例面瘫者脑脊液正常）；8例患者神经电生理检查提示周围神经脱髓鞘改变为主。结论：变异型GBS临床上因无肢体瘫痪，表现多样化。诊断有赖于CSF和神经电生理检查。     

格林巴利综合征的相关研究进展

格林巴利综合征(Guillain-Barre Syndrome, GBS)是一种急性自身免疫性多发性神经疾病,表现为迅速发展的上行乏力、轻度感觉减退和反射变弱或消失.急性炎症性脱髓鞘性多发性神经病(Acute Inflammatory Demyelinative Polyradiculoneuropa-thy,AIDP)是最早确定也是欧洲最常见的 GBS 表型,伴随不同程度的继发性轴索损伤.急性运动性轴索 神 经 病 (Acute motor axonal neuropathy, AMAN)和急性运动感觉轴索型神经病(Acute Mo-tor and Sensory Axonal Neuropathy,AMSAN)为GBS亚型:前者呈现原发性轴索障碍,仅累及运动神经,是亚洲、中美和南美洲最常见的 GBS 表型;后者更罕见,累及感觉和运动神经.此外,Miller Fisher综合征(Miller Fisher Syndrome,MFS)也是GBS的轴索型变体,Bickerstaff 脑炎(Bickerstaff's Brain-stem Encephalitis,BBE)则为MFS 的变体[1].GBS在西方国家年发病率为 1.1~1.8/100,000 人,中国为 0.66/100,000 人[2].其发病率随年龄线性增长且男性更易患病,但在婴幼儿、儿童的自然进程呈现更多良态:约 1%~6%GBS 患者罹患复发性 GBS (Recurrent Guillain Barre Syndrome,RGBS),极少见儿童患RGBS的情况[3].目前 GBS 仍属危重病:25%的患者将因呼吸障碍或气道保护接受机械通气(Mechanical Ventilation,MV)治疗,3%~11%的患者死于GBS的并发症.尽管大部分患者基本康复,仍有20%~38%残疾[4].本文通过综述 GBS 相关的主要进展,涉及诊疗、预后和可能的机理等内容,旨在为临床综合治疗GBS提供一定参考.     

94例极重型格林-巴利综合征抢救体会

目的 探讨影响极重型格林-巴利综合征(GBS)抢救成功的因素。方法 予气管插管或气管切开、辅助呼吸及鼻饲,采用激素及综合冶疗,并预防和处理并发症。结果 治愈28例,显效11例,死亡55例。结论 极重型GBS抢救成功的关键是尽早气管插管或气管切开并予辅助呼吸,以保持呼吸道通畅和改善心脑缺氧,同时应对患者进行心电和血气监护。另外,应积极预防和处理气管切开后的并发症。而大剂量地塞米松治疗不可取。     

Miller Fisher syndrome: an uncommon acute neuropathy

The syndrome of ophthalmoplegia, ataxia, and areflexia was first described in 1956 by Miller Fisher. This syndrome has long been believed to be a variant of Guillain-Barré syndrome (GBS), mainly because of its areflexia, cerebrospinal fluid findings, and its postinfectious presentation. The case of an 11-year-old male with Miller Fisher syndrome (MFS) is described. MFS differs from GBS in several key clinical features and presents an extensive and challenging differential diagnosis. It is useful to recognize MFS as both a variant of GBS and a distinct entity with its own therapeutic considerations.     

格林-巴利综合征患者血清中髓鞘脂自身抗体水平的测定

应用免疫学方法检测了格林－巴利综合征（ＧＢＳ）病人和正常对照组各３０例的血清中髓鞘脂自身抗体含量，并进行了动态观察。结果显示，ＧＢＳ病人血清中髓鞘脂自身抗体水平明显高于正常对照组（Ｐ〈０．０１），ＧＢＳ患者髓鞘脂自身抗体出现的阳性率为２０％。动态观察发现，ＧＢＳ患者血清中髓鞘脂自身抗体水平的下降与临床症状的改善相一致。     

系统护理干预对格林-巴利综合征病人康复的影响

[目的]探讨系统护理干预对格林-巴利综合征病人康复的影响。[方法]将38例格林-巴利综合征病人分为治疗组和对照组各19例。两组在执行相同治疗的同时,对照组采用常规护理,治疗组在此基础上实施系统护理干预。[结果]系统护理干预后差异有统计学意义（P均〈0.05）。[结论]系统护理干预对格林-巴利综合征病人康复治疗有显著提高。     

血浆置换治疗格林巴利综合征不良反应的观察及护理

目的观察血浆置换(PE)治疗格林-巴利综合征(GBS)的不良反应并探讨相关护理措施。方法回顾性分析36例进行PE治疗的GBS患者的临床资料,观察PE治疗GBS的不良反应。结果 36例患者共进行139次PE治疗,其中10例患者出现荨麻疹,4例出现血压下降,2例出现口周麻木,1例出现血压升高,1例呼吸困难加重。结论 PE存在一定风险,护士的术前访视、心理护理、术前准备以及术中监测、观察、处理和术后护理是保证治疗成功的重要基础。     

Headache associated with miller fisher syndrome

Miller Fisher syndrome, a variant of Guillain-Barre syndrome, is composed of the clinical triad of ophthalmoplegia, ataxia, and areflexia. A variety of other symptoms and signs have been reported in this syndrome, but headache is not a commonly reported symptom. We report a 35-year-old man with anti-GQ(1b) antibody-confirmed Miller Fisher syndrome presenting with severe and persistent headache, and we propose that the headache is caused by antibody-mediated effects on the trigeminovascular pain pathway.     

Miller Fisher syndrome and plasmapheresis.

Treatment for Miller Fisher syndrome (MFS) is controversial, and even the natural history and prognosis are not fully understood. We retrospectively reviewed our cases of MFS for the last 3 years. The analysis of 4 MFS cases revealed that we had performed plasmapheresis or additional immunotherapy to each of 4 patients, and their symptoms resolved for up to 50 days after the onset (ataxia improved 20-35 days and ophthalmoplegia for 25-50 days) except for 1 patient, and that Guillain-Barré syndrome had been diagnosed in 1 patient who had developed profound muscle weakness. We also discovered that MFS patients had a deviated T-helper Type-1 (Th1)/T-helper Type-2 (Th2) polarization and that plasmapheresis can shift Th2-dominant status to Th1-dominant status in patients with MFS. Although plasmapheresis may remove humoral factors, including anti-GQ1b, and may induce a shift of the Th1/Th2 cytokine-producing cell balance in peripheral blood, the therapeutic rationale has not yet been established. Therefore, controlled clinical trials are required to show whether plasmapheresis leads to earlier recovery with fewer neurologic deficits in patients with MFS.     

Use of the International Classification of Functioning,Disability and Health to identify preliminary comprehensive and brief core sets for Guillain Barre syndrome

Purpose.?To identify the preliminary comprehensive and brief core sets for Guillain Barre syndrome (GBS), in a Delphi process using the International Classification of Functioning, Disability and Health (ICF).

Method.?Focus groups and a consensus process were used to identify ICF core sets for GBS. This included: preliminary ICF studies; empirical patient data collection for 77 GBS participants; review of the evidence base and treatment in GBS literature followed by a Delphi exercise with 23 physicians and allied health professionals in Melbourne, Australia.

Results.?The expert consensus selected 99 second level ICF categories (in three rounds) which identify health domains relevant to GBS for multidisciplinary assessment. These domains were consistent with current practice and existing GBS literature. The comprehensive core set includes: 27 (23%%) categories from the component ‘body function’, 7 (12%%) categories from ‘body structures’, 43 (36%%) from ‘activities and participation’ and 22 (29%%) from the component ‘environmental’ factors. The brief set comprised 20 categories, 20%% of categories in the comprehensive core set.

Conclusion.?The core set categories for GBS-related health need to be addressed in multidisciplinary care programs. Future clinical ‘rating’ of this set may facilitate scale development using the ICF in GBS. Further research is needed to confirm the generalisability of this set in clinical settings.     

血浆置换治疗伴呼吸肌无力危象的格林-巴利综合征和重症肌无力疗效观察

目的 观察血浆置换治疗伴呼吸肌无力危象的格林-巴利综合征（GBS）和重症肌无力（MG）患者的效果.方法 选择中山大学附属第一医院肾内科32例伴呼吸肌无力危象的GBS和MG患者中,15例采用单纯药物治疗,17例联用血浆置换,比较两组患者呼吸机辅助呼吸和住院时间.结果 血浆置换治疗组和单纯药物组呼吸机辅助呼吸时间分别为（8.7±8.4）d、（19.8±15.0）d,住院时间分别为（33.3±21.9）d、（52.7±28.4）d,两组比较差异有统计学意义（P＜0.05）.结论 血浆置换可有效缩短伴呼吸肌无力危象的GBS和MG患者呼吸机辅助呼吸和住院时间,值得临床推广应用.