变异型Guillain-Barre综合征的临床特点

目的探讨变异型Guillain-Barre综合征(GBS)的临床特点。方法回顾性分析14例变异型GBS患者的临床资料。结果本组10例为女性,4例为男性;均为急性或亚急性起病,病前11例有相关诱因;临床表现为多脑神经型、急性运动轴索型、Miller-Fish综合征及全自主神经功能障碍型。12例行腰椎穿刺术,11例脑脊液蛋白增高;11例行肌电图检查,9例提示为周围神经源性损害;经个体化治疗所有患者的症状均有改善。结论变异型GBS类型及临床表现多样,以女性患者多见;脑脊液蛋白-细胞分离及肌电图检查为周围神经源性损害有助于诊断。     

以手术为诱因的重型吉兰巴雷综合征临床特点和预后分析

目的初步探讨以手术为前驱因素的重型吉兰巴雷综合症(Guillain-Barre syndrome,GBS)的临床特点和预后。方法从62例重型GBS患者中收集6例以手术为前驱因素GBS患者的临床资料,对患者的手术、起病时间、达峰时间、临床症状、电生理检查和治疗方案等进行总结,并随访至1年,结合休斯功能分级量表(Hughes functional grading scale,HFGS),评价患者的临床特点及其预后。结果根据临床及电生理表现,6例以手术为前驱因素的重型GBS患者均诊断为急性运动轴索性神经病。手术诱因包括体外循环手术、颈动脉内膜剥脱术、基底动脉瘤栓塞术、颅内血肿钻孔引流术、椎间盘置换术、椎管减压和椎弓根固定术,发病年龄在47~63岁之间,潜伏期多在10 d之内,达峰时间均在5 d之内,4例肢体无力起病,2例呼吸困难起病;发病3个月,6例患者均不能独立行走(HFGS=4),5例随访至1年,仅2例能独立行走(HFGS=2)。结论以手术为前驱因素的重型GBS少见,常以肢体无力或呼吸困难起病,达峰时间快,预后差。     

仅仅表现为双下肢无力的GBS 2例报告

正吉兰-巴雷综合征(Guillain-barre syndrome,GBS)是一种自身免疫介导的周围神经病变,主要累及多数脊神经根和周围神经,临床上急性或亚急性起病,以四肢弛张型瘫痪、腱反射消失、脑脊液出现蛋白-细胞分离为主要特点。典型病例不难诊断,但变异型GBS临床上少见,容易误诊、漏诊。近年来,不断有各种变异型GBS的报道~[1,2],其表现复杂多样,特别是以双下肢无力为首发症状并仅仅局限于下肢无力的GBS更为少见,国内尚未报道。本文报道我院收治的2例仅仅表现为双下肢无力的GBS患者的临床资料,并复习相关文献,以提高对该病的认识。1病例资料     

中国东北部吉兰-巴雷综合征临床及电生理表现

目的描述中国东北部吉兰-巴雷综合征(GBS)的临床、神经电生理表现。方法收集138例确诊GBS的患者,回顾性分析其临床及神经电生理结果。结果中国东北部GBS患者以中青年居多,前驱感染以消化道感染史最常见,临床首发症状以四肢同时出现无力最常见,急性炎症性脱髓鞘性多发性神经病(AIDP)为主要类型,且患者起病症状较重。静脉注射免疫球蛋白(IVIg)对GBS起病2 w后及轻型患者均有效。电生理上以复合肌肉动作电位(CMAP)波幅降低、F波及H反射异常为主要表现,且起病7 d内、7¹4 d及14 d后电生理表现无显著差异。结论中国东北部GBS有独特的临床及电生理表现,早期进行电生理检查有助于早期诊断GBS。     

以周围性面瘫为主要表现的吉兰－巴雷综合征变异型：附2例临床分析

该文报道了2例以周围性面瘫为主要表现的吉兰－巴雷综合征（GBS）变异型的患者。这2例患者均为以周围性面瘫起病,其中1例为双侧周围性面瘫,伴疼痛;另1例为左侧周围性面瘫合并头晕、思睡、食欲缺乏等。2例患者早期肌电图均未发现异常。1例存在脑脊液―蛋白细胞分离,另1例血清抗GQ1b抗体IgM（+）。诊断为GBS变异型。2例患者均给予静脉滴注人血免疫球蛋白,症状好转后出院。对以周围性面瘫起病的患者,需考虑是单纯的面神经炎还是以周围性面瘫起病的其他疾病,如GBS变异型等,以便正确诊治。 [国际神经病学神经外科学杂志, 2024, 51(2): 73-75]     

34例变异型吉兰-巴雷综合征的临床特点分析

目的 分析变异型吉兰-巴雷综合征(GBS)的临床特点。方法 回顾性分析2012年10月-2019年3月变异型GBS患者的临床表现、电生理及脑脊液特点。结果 共34例患者,包括Miller-Fisher综合征(MFS)及MFS变异亚型共12例,脑神经变异型(CNV)12例,急性感觉神经病(ASN)1例,急性泛自主神经病(APN)1例,咽-颈-臂(PCB)2例以及不能明确分型6例。34例变异型GBS患者中男20例、女14例(P=0.392); 发病年龄17～80岁,平均年龄(53.38±14.99)岁,中年(41～65岁)组所占比例最多(P=0.000); 20例有前驱事件,上呼吸道感染占65%; 首发症状以肢体麻木(38.2%)、吞咽困难(29.4%)、吐词不清(23.5%)多见; 97.1%的患者发病4周内达高峰; 需机械通气者5.9%; 疾病开始恢复的中位时间13 d,住院中位时长13.5 d; 64.7%的患者腱反射减弱或消失; 在完成腰椎穿刺检查的患者中脑脊液蛋白-细胞分离者58.6%; 发病到完善神经电生理检查的平均时间(10.70±7.32)d,85.2%神经电生理检查表现异常; 50%患者给予静脉注射免疫球蛋白(IVIg)治疗,14.7%患者给予激素治疗,8.8%患者给予免疫球蛋白联合激素治疗,除外1例患者主动要求出院,其余治疗均有效。结论 变异型GBS临床表现多样,常无典型急性四肢对称性迟缓性麻痹等症状,临床诊断需要综合判断,出现一些少见的临床表现并不能除外GBS的诊断,脑脊液和神经电生理检查可以帮助提高诊断,详尽的病史及神经系统查体尤为重要,免疫球蛋白和激素治疗有效。     

咽-颈-臂变异型吉兰-巴雷综合征1例报道及文献复习

目的探讨咽-颈-臂(pharyngeal-cervical-brachial,PCB)变异型吉兰-巴雷综合征(GBS)的临床特点和诊断。方法分析我院收入1例PCB变异型GBS患者的临床表现、辅助检查结果和临床治疗效果。结果 PCB变异型GBS临床表现口咽肌、颈部、肩部、双上肢无力,双下肢不受累或轻度受累。本例患者腰穿提示明显蛋白-细胞分离,给予静滴免疫球蛋白和甲强龙冲击治疗后症状明显好转。结论 PCB是GBS少见的变异型,极易出现误诊或漏诊,临床表现为咽-颈-臂肌无力,而下肢不受累或轻度受累的患者,应考虑PCB的可能,可结合脑脊液、电生理检查及早作出诊断和治疗。     

重症吉兰-巴雷综合征的预测因素分析

目的分析轻症与重症吉兰-巴雷综合征(Guillain-Barrésyndrome,GBS)患者临床及神经电生理检查特点,筛选出重症患者的预测因素。方法回顾性分析2006年1月至2015年11月在本院住院治疗的GBS患者101例。根据疾病高峰期Hughes评分划分为轻症组(0~2分)和重症组(3~6分)。分别统计两组患者发病年龄、性别、前驱感染史、首发症状、是否有延髓功能障碍、是否累及颅神经和自主神经、有无轴索损害等指标,分析两组之间差异,并筛选出重症患者的预测因素。结果以非感觉异常为首发症状(P0.001)、有延髓功能障碍(P0.001)、颅神经受累(P=0.025)、自主神经系统受累(P=0.018)、运动系统受累(P=0.004)以及轴索损害(P0.001)的患者容易发展为重型患者。多因素Logistic回归分析发现轴索损害(P=0.008,OR=4.632)、延髓受累(P=0.010,OR=10.420)、颅神经受累(P=0.047,OR=0.076)是发展为重型的独立危险因素。结论轴索损害、延髓受累和颅神经受累可能是GBS患者发展至重型的有意义的预测因素。     

咽-颈-臂变异型格林-巴利综合征1例报道及文献复习

目的探讨咽-颈-臂(pharyngeal-cervical-brachia,PCB)变异型格林-巴利综合征(Guillain-Barrésyndrome,GBS)的临床特点。方法报道1例PCB变异型GBS,并复习相关文献。结果 PCB变异型GBS主要表现为颈、肩、上肢近端、口咽肌肉无力,而下肢通常不受累或仅轻微受累。本例患者另外一项突出的临床表现是发病初即出现的双上肢近端剧烈疼痛。结论对于急性起病的双上肢疼痛、无力以及口咽肌无力患者,应警惕PCB变异型GBS的可能。     

抗GQ1b、GT1a、Sulfatide抗体阳性的类重症肌无力Miller-Fisher综合征1例报告

Miller-Fisher综合征（Miller-Fisher syndrome,MFS）是吉兰-巴雷综合征（Guillain-Barre syndrome,GBS）的一种临床变异型,以共济失调、眼肌麻痹及腱反射消失为主要临床特征,极少出现瞳孔改变和瞳孔对光反射异常,一般无症状波动,部分患者可检测到抗GQ1b IgG抗体阳性。本文报告了1例抗GQ1b、GT1a、Sulfatide抗体阳性的MFS,以波动性眼外肌麻痹起病,伴双侧瞳孔散大、对光反射迟钝及四肢麻木无力,症状少见不典型,临床极易误诊。     

吉兰-巴雷综合征脑脊液蛋白与面神经瘫痪的相关性

目的探讨吉兰-巴雷综合征(Guillain-Barre Snydrome,GBS)患者脑脊液蛋白与面神经瘫痪的关系及其临床意义。方法回顾性分析2005年1月至2015年9月我院神经内科确诊的111例GBS患者临床与生化资料,根据面神经瘫痪将患者分为面神经正常组与瘫痪组,比较两组患者间的临床、生化特征;根据脑脊液蛋白值将患者分为脑脊液蛋白正常组、轻度偏高组与异常偏高组,比较3组间面神经瘫痪数量的变化;分析脑脊液蛋白与面神经瘫痪的相关性。结果面神经正常组与瘫痪组之间患者性别、年龄、残疾量表评分(≥3)、上呼吸道感染、胃肠感染、肺部感染及近期疫苗接种史比较无统计学差异(P0.05),脑脊液蛋白、免疫球蛋白G(Ig G)、脑脊液白蛋白/血清白蛋白(QALB)比较有统计学差异(P0.05);3组不同脑脊液蛋白水平患者面神经瘫痪数量比较有统计学差异(F=3.48,P=0.03);脑脊液蛋白水平与面神经瘫痪数量呈正相关(r=0.288,P0.01)。结论 GBS患者脑脊液蛋白值越高面神经瘫痪发生的可能性越大,脑脊液蛋白有助于GBS患者面神经瘫痪的病情监测。     

Antecedent symptoms in Guillain-Barré syndrome: an important indicator for clinical and serological subgroups

OBJECTIVES: To examine whether Guillain-Barré syndrome (GBS) can be classified in clinical and immunological subgroups based on the type of prior illness. Background - The existence of antecedent symptoms supports the diagnosis of GBS in patients who experience acute muscle weakness progression. However, little is known about additional meanings of determining antecedent symptoms. MATERIALS AND METHODS: Prospective investigation of prior infectious illness in GBS and related disorders (n=176). RESULTS: The frequent antecedent symptoms in GBS and related disorders were fever (52%), cough (48%), sore throat (39%), nasal discharge (30%), and diarrhea (27%). Patients who had sore throats or coughs frequently had ophthalmoparesis (respectively P=0.0004, P=0.001) and IgG anti-GQ1b antibody (P=0.01, P=0.007). Fever was associated with bulbar palsy (P=0.047) and headache with facial palsy (P=0.04). Patients with diarrhea often had anti-ganglioside IgG (anti-GM1 [P=0.0006] and anti-GM1b [P=0.008]), IgM (anti-GM1 [P=0.03], anti-GM1b [P=0.02], and anti-GalNAc-GD1a [P=0.047]) antibodies and rarely showed ophthalmoparesis or bulbar palsy (respectively P=0.02, P=0.04). Diarrhea and abdominal pain were closely associated with Campylobacter jejuni serology (respectively P<0.0001, P=0.01), whereas other symptoms were not related to pathogens such as cytomegalovirus, Epstein-Barr virus, or Mycoplasma pneumoniae. CONCLUSIONS: Our comprehensive study showed that GBS preceded by sore throat, cough, fever, headache, or diarrhea respectively forms clinical or serological subgroups, or both. This association is not necessarily dependent on infection by the known trigger pathogens.     

37例格林－巴利综合征患者血清抗空肠弯曲菌脂多糖抗体检测

已有许多研究证实格林－巴利综合征（ＧＢＳ）与前驱空肠弯曲菌（Ｃｊ）感染有密切关系。但是，Ｃｊ诱发ＧＢＳ的病理机制仍不明确。作者试图探讨Ｃｊ菌体成份与ＧＢＳ发病的关系。以一株自ＧＢＳ患者分离得到的Ｃｊ之脂多糖（ＬＰＳ）为抗原，用酶联免疫吸附试验（ＥＬＩＳＡ）方法，检测了ＧＢＳ患者组（观察组）和健康成人组（对照组）的血清抗空肠弯曲菌脂多糖抗体。结果ＧＢＳ组血清呈阳性反应者为２０／３７（５４％）、对照组血清呈阳性反应的为１／３６（３％），差别具有统计学意义（Ｐ＜０．００１，ｘ２）。提示多数ＧＢＳ患者血清中存在着不同于健康人群的ＣｊＬＰＳ特异抗体．     

37例格林－巴利综合征患者血清抗空肠弯曲菌脂多糖抗体检测

已有许多研究证实格林－巴利综合征（ＧＢＳ）与前驱空肠弯曲菌（Ｃｊ）感染有密切关系。但是，Ｃｊ诱发ＧＢＳ的病理机制仍不明确。作者试图探讨Ｃｊ菌体成份与ＧＢＳ发病的关系。以一株自ＧＢＳ患者分离得到的Ｃｊ之脂多糖（ＬＰＳ）为抗原，用酶联免疫吸附试验（ＥＬＩＳＡ）方法，检测了ＧＢＳ患者组（观察组）和健康成人组（对照组）的血清抗空肠弯曲菌脂多糖抗体。结果ＧＢＳ组血清呈阳性反应者为２０／３７（５４％）、对照组血清呈阳性反应的为１／３６（３％），差别具有统计学意义（Ｐ＜０．００１，ｘ２）。提示多数ＧＢＳ患者血清中存在着不同于健康人群的ＣｊＬＰＳ特异抗体．     

Clinical characteristics of children with Guillain-Barré syndrome and factors associated with disease severity

Guillain-Barré syndrome (GBS) is the leading cause of pediatric acute flaccid paralysis.This study aimed to summarize the clinical features of children with GBS and to explore factors associated with the severity of weakness. One hundred and twenty-two children with GBS (73 males and 49 females) were retrospectively analysed. The median age (IQR) at diagnosis was 4.0 years (2.9–7.2 years), and 26.2% of patients were at the age of 2–3 years. Of the 122 cases, 71 (58.2%) had an antecedent infection, 58 (47.5%) had cranial nerve involvement, 36 (29.1%) had dysautonomia, 77 (63.1%) had sensory symptoms, 28 (23.0%) had difficulty in breathing of which 15 (12.3%) patients required mechanical ventilation, and 8 (6.6%) had normal tendon reflex or hyperreflexia. Cytoalbuminologic dissociation of the cerebrospinal fluid was observed in 97 cases (82.9%). Further, 120 patients underwent nerve conduction studies: 76 (63.3%) exhibited demyelinating features whereas 36 (30.0%) had axonal type of CBS. 70.2% of patients could walk independently at 12 weeks. Fourteen (11.5%) patients were classified into the mild group [GBS disability score (GBS-DS) < 3] and 108 (88.5%) were classified into the severe group (GBS-DS ≥ 3). The incidence of cranial involvement (P = 0.038) and decreased tendon reflexes (P = 0.048) were significantly different between the two groups. These findings suggested that cranial nerve involvement is associated with severe muscle weakness in children with GBS.     

Guillain-Barré syndrome in northern Tanzania: a comparison of epidemiological and clinical findings with western Norway

To study Guillain-Barré syndrome (GBS), the clinical files of GBS patients, 59 in northern Tanzania (1984–1992) and 56 in western Norway (1980–1992), were retrospectively reviewed and compared. The average annual incidence rate for GBS in the Kilimanjaro region was 0.83/100000 population as compared to 1.2/100000 reported in western Norway (25). GBS patients in the Tanzanian series were younger, had less antecedent infection and were a longer time interval from onset to admission and in hospital. On examination arm and sensory involvement were less common and urinary sphincteric involvement more common in the Tanzanian series. The overall mortality rate was higher in the Tanzanian series, 15.3% (9/59) versus 1.8% (1/56). HIV infection was found in 30.5% (11/36) of GBS patients in the Tanzanian series as compared to 3.4% (161/4687) in corresponding blood donors. There was no evidence of HIV infection in the Norwegian series. The HIV-seropositive GBS patients in comparison with HIV seronegatives had a shorter duration of onset, greater neurological involvement and a higher mortality rate, 45.5% (5/11) versus 16% (4/21). This study shows that apart from minor clinical differences and an increased mortality rate in the Tanzanian series GBS was similar in both series. GBS in the Tanzanian series was strongly associated with HIV infection, and seropositives by comparison with seronegatives appeared to have more severe disease.     

Epidemiological Features and Economic Burden of Guillain-Barré Syndrome in South Korea: A Nationwide Population-Based Study

Background and PurposeGuillain-Barré syndrome (GBS) is rare, but its symptoms are severe and they occasionally lead to long-term disability. Country-specific epidemiological evidence is useful for detecting potential problems at the population level. This study investigated the epidemiological and economic characteristics of GBS in South Korea.MethodsThe Korean National Health Insurance Service claims data from 2010 to 2016 were used to identify incident cases as newly hospitalized patients with a primary diagnosis of GBS (the 10th revision of the International Classification Disease code of G61.0). New cases were defined as patients not having claim records for GBS within one year prior to the hospital admission for GBS.ResultsThe incidence rate increased by 45.6% between 2010 and 2016, from 1.28 to 1.82 per 100,000 population. All age groups other than <20 years showed increasing trends. The incidence rate was highest in those aged 65 years to 74 years. Approximately 72% of the incident GBS cases had antecedent infection within 42 days before GBS was diagnosed. Children younger than 10 years constituted the highest proportion of antecedent infections (93.7%). The average length of stay per GBS hospitalization was 33.5 days. Patients had an average of 7.48 outpatient visits for GBS treatment per year. The economic burden from a societal perspective of treating GBS during the first year was USD 16,428.ConclusionsThe increasing incidence trend and substantial economic burden of GBS strongly advocate the development of effective strategies for preventing and managing GBS.     

Guillain-Barré syndrome: diagnostic criteria, epidemiology, clinical course and prognosis

Thirty-four patients were identified with Guillain-Barré syndrome (GBS) on review of 266 neuropathy cases admitted to a Copenhagen county hospital from June 1977 to January 1984. The age-adjusted incidence rate of GBS is 2.0 x 10(-5) years-1. The natural history of the disease, antecedent events, symptoms and signs, autonomic dysfunction, sequelae, CSF findings and mortality are described. Six cancer patients with GBS differed significantly from the non-cancer patients in a more protracted disease course and failure to improve. The National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) criteria for the diagnosis of GBS are discussed, and it is concluded that the criteria, although useful in comparative studies, are too restrictive when used in clinical practice.     

Epidemiology of the Guillain-Barré syndrome in the county of Hordaland, Western Norway

ABSTRACT – We have examined the incidence of the Guillain-Barré syndrome (GBS) in the county of Hordaland. We have previously reported a marked increase in the incidence of MS in the same population. 109 GBS-patients were diagnosed in the period 1957–1982 according to the criteria of NINCDS. The annual incidence rates were stable over time with an average incidence of 1.2 per 100,000 population per year. The male/female ratio was 1.7. No statistically significant difference in incidence was found between urban and rural areas. We found an increasing incidence with age, more marked among males than among females. A predilection for GBS to occur in the colder half of the year was also found. 57% of the patients reported an antecedent infection less than 4 weeks prior to the onset of neurological symptoms. The stable incidence rates for GBS over time in contrast to an increase in the incidence of MS in the same population, indicates that different pathogenetic mechanisms are important in the two demyelinating diseases.     

Clinical features and response to treatment in Guillain-Barré syndrome associated with antibodies to GM1b ganglioside

GM1b is a minor ganglioside in human peripheral nerves. Serum anti-GM1b antibodies frequently are present in patients with Guillain-Barré syndrome (GBS). In this collaborative study, we investigated the antecedent infections, clinical features, and response to treatment of GBS patients with anti-GM1b antibodies. Of 132 GBS patients who participated in the Dutch GBS trial that compared the effect of intravenous immunoglobulins and plasma exchange, 25 (19%) patients had anti-GM1b antibodies. IgM antibodies were present in 14, IgG antibodies in 15, and both isotypes in 4 patients. The 25 patients with anti-GM1b antibodies had a clinical pattern distinct from that of the other 107 GBS patients. They more often had an episode of gastrointestinal illness and frequently showed serological evidence of recent infection by Campylobacter jejuni. The anti-GM1b-positive subgroup was marked by more rapidly progressive, more severe, and predominantly distal weakness. Cranial nerve involvement and sensory deficits were less common in the patients with anti-GM1b antibodies. The presence of anti-GM1b antibodies was associated with slower recovery. The clinical manifestations predominantly were associated with anti-GM1b antibodies of the IgG isotype. Fourteen (56%) of the 25 patients with anti-GM1b antibodies also had anti-GM1 antibodies. The group of patients with both antibodies was clinically more homogeneous and had a more rapidly progressive, pure motor neuropathy. The subgroup of anti-GM1b-positive GBS patients responded well to treatment with immunoglobulins but not to plasmapheresis. The distinctive clinical features of the patients with anti-GM1b antibodies show that acute motor neuropathy represents a specific subgroup within GBS and that recognizing these patients may have consequences as to the choice of therapy.