Guillain-Barre Syndrome

格林 - 巴利综合征
  • 文章类型: Journal Article
    Guillain-Barre syndrome rarely develops after allogeneic hematopoietic stem cell transplantation (allo-HSCT), and only a few reports exist in China. Guillain-Barre syndrome is an acute and life-threatening condition that requires early diagnosis and treatment. A patient with acute myeloid leukemia underwent allogeneic HSCT for >5 months and gradually developed limb muscle weakness and limited eye movement after coexisting with delayed acute intestinal graft-versus-host disease. After the examination of cerebrospinal fluid and electromyography, the diagnosis of Guillain-Barre syndrome was confirmed. After a high-dose intravenous immunoglobulin (IVIg) treatment, muscle strength gradually recovered, and the prognosis was good.
    异基因造血干细胞移植后合并吉兰-巴雷综合征较为罕见,国内报道很少。吉兰-巴雷综合征发病急,严重威胁患者生命,需尽早诊断及治疗。1例急性髓系白血病患者行异基因造血干细胞移植后5个月余,合并迟发急性肠道移植物抗宿主病后逐渐出现四肢肌无力、眼球运动受限,经脑脊液、肌电图等检查,明确诊断吉兰-巴雷综合征,经大剂量静脉免疫球蛋白治疗,肌力逐渐恢复,预后良好。.
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  • 文章类型: Case Reports
    背景:格林-巴利综合征(GBS)是由自身免疫引起的急性炎性周围神经病变。神经节苷脂和硫酸盐是周围神经的重要组成部分。抗硫酸脂抗体介导的补体与GBS的急性感觉运动性周围神经病变有关,其特点是疼痛和感觉异常。
    方法:这个孩子是一个7岁的女孩,有头痛和腹痛,其次是肢体麻木和疼痛。头颅成像显示心室扩张,周围神经功能传导检查显示多神经根病,脑脊液测试显示细胞计数正常,但蛋白质水平升高,所有这些都导致了GBS的诊断。静脉注射免疫球蛋白治疗后(400mg/kg×5天),症状没有改善,肌肉力量逐渐恶化,伴有阵发性面部潮红,心率波动,多汗症,脑脊液蛋白逐渐增加(高达3780.1mg/L)。根据这些发现结合血清抗硫酸脂IgM阳性,考虑了抗硫酸盐抗体相关的GBS,低剂量泼尼松龙(1mg/kg/d)治疗可改善症状。
    结论:抗硫酸盐抗体相关GBS与小纤维性周围神经病变相关。主要表现是疼痛,感觉异常和自主神经功能障碍。除了脊髓神经根吸收功能障碍引起的脑脊液蛋白增加,自主神经功能障碍可能与疼痛有关。当免疫球蛋白的治疗效果不理想时,可以考虑低剂量和短疗程的皮质类固醇,预后良好.
    BACKGROUND: Guillain‒Barre syndrome (GBS) is an acute inflammatory peripheral neuropathy caused by autoimmunity. Gangliosides and sulfatides are important components of peripheral nerves. Anti-sulfatide antibody-mediated complement is associated with acute sensorimotor peripheral neuropathy in GBS, which is characterized by pain and paresthesias.
    METHODS: The child was a 7-year-old girl with headache and abdominal pain, followed by limb numbness and pain. Cranial imaging showed ventricular dilatation, peripheral nerve function conduction examination showed polyradiculopathy, and cerebrospinal fluid tests showed normal cell counts but elevated protein levels, all of which led to the diagnosis of GBS. After treatment with intravenous immunoglobulin (400 mg/kg × 5 days), the symptoms did not improve, and muscle strength progressively worsened, accompanied by paroxysmal complexion flushing, heart rate fluctuation, hyperhidrosis, and a progressive increase in cerebrospinal fluid protein (up to 3780.1 mg/L). On the basis of these findings combined with serum anti-sulfatide IgM positivity, anti-sulfatide antibody-related GBS was considered, and treatment with low-dose prednisolone (1 mg/kg/d) led to symptom improvement.
    CONCLUSIONS: Anti-sulfatide antibody-associated GBS is associated with small fiber peripheral neuropathy. The main manifestations are pain, paresthesias and autonomic dysfunction. In addition to the dysfunction of spinal nerve root absorption caused by increased cerebrospinal fluid protein, autonomic dysfunction may be involved in pain. When the therapeutic effect of immunoglobulin is not satisfactory, a low dose and short course of corticosteroids can be considered, and the prognosis is good.
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  • 文章类型: Case Reports
    双侧面部麻痹伴感觉异常(FDP)是GBS的一种罕见变体,同时表现为双侧面神经麻痹和远端肢体感觉异常。越来越多的证据表明,在某些GBS患者中,抗GT1aIgG的存在作为脑神经麻痹发展的效应分子具有致病作用,而抗GT1a抗体在FDP中很少呈阳性。这里,我们报道了一例33岁男性FDP患者,表现为急性发作的双侧面神经麻痹和足部轻微感觉异常是唯一的神经系统表现.在患者中注意到没有可识别的发烧或皮肤爆发原因的先前感染。他还进行了脑脊液白蛋白细胞解离和异常神经传导研究。值得注意的是,特异性血清抗神经节苷脂检测显示抗GT1aIgG/IgMAb阳性.患者对静脉注射免疫球蛋白治疗反应良好。这个案例让人们意识到GBS的一种罕见变体,并首次表明抗GT1a抗体在FDP的发展中起着致病作用。该病例还表明,如果诊断为FDP,应实施及时的IVIG管理。
    Bilateral facial palsy with paresthesia (FDP) is a rare variant of GBS, characterized by simultaneous bilateral facial palsy and paresthesia of the distal limbs. Mounting evidence indicates that the presence of anti-GT1a IgG has a pathogenic role as an effector molecule in the development of cranial nerve palsies in certain patients with GBS, whereas anti-GT1a antibody is rarely presented positive in FDP. Here, we report the case of a 33-year-old male diagnosed with FDP presented with acute onset of bilateral facial palsy and slight paresthesias at the feet as the only neurological manifestation. An antecedent infection with no identifiable reason for the fever or skin eruptions was noted in the patient. He also exhibited cerebrospinal fluid albuminocytologic dissociation and abnormal nerve conduction studies. Notably, the testing of specific serum anti-gangliosides showed positive anti-GT1a IgG/IgM Ab. The patient responded well to intravenous immunoglobulin therapy. This case brings awareness to a rare variant of GBS, and provides the first indication that anti-GT1a antibodies play a causative role in the development of FDP. The case also suggests that prompt management with IVIG should be implemented if FDP is diagnosed.
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  • 文章类型: Case Reports
    背景:急性球麻痹(ABPp)综合征是格林-巴利综合征(GBS)的一种不寻常的变种。已经报道了ABPp患者的抗GT1a和抗GQ1b抗体,但没有与GD3抗体相关的报道。
    方法:对1例ABPp综合征患者的临床资料进行回顾性分析。并对文献报道的GBS患者合并ABP和面瘫进行了总结。
    结果:我们报道了一个13岁的女孩,表现为不对称的双面无力,球麻痹和短暂性肢体麻木,血清IgG抗GD3抗体阳性。通过回顾以前报道的GBS患者的ABP和面瘫,我们发现面部麻痹可以是单侧或双侧。双侧面神经麻痹可连续或同时出现,可以是对称的或不对称的。其他常见症状包括眼肌麻痹,感觉异常和共济失调。IgG抗GT1a和IgG抗GQ1b抗体最常见。大多数患者在随访的两周至一年内完全康复。
    结论:我们报道了一位患有不对称双面神经麻痹和球麻痹的患者,这似乎符合ABPp综合征的诊断。这是GBS的ABPp变体血清神经节苷脂GD3IgG抗体阳性的首次报道。
    BACKGROUND: Acute bulbar palsy-plus (ABPp) syndrome is an unusual variant of Guillain-Barré syndrome (GBS). Anti-GT1a and anti-GQ1b antibodies have been reported in patients with ABPp, but without reports related to GD3 antibodies.
    METHODS: Clinical data of a patient diagnosed as ABPp syndrome were reviewed clinically. And we summarized the GBS patients with ABP and facial paralysis reported in the literature.
    RESULTS: We reported a 13-year-old girl presented with asymmetric bifacial weakness, bulbar palsy and transient limb numbness, and had positive serum IgG anti-GD3 antibody. Through reviewing the GBS patients with ABP and facial paralysis reported previously, we found that facial palsy could be unilateral or bilateral. The bilateral facial palsy could present successively or simultaneously, and could be symmetrical or asymmetrical. Other common symptoms included ophthalmoplegia, sensory abnormality and ataxia. IgG anti-GT1a and IgG anti-GQ1b antibodies were the most frequent. Most of the patients had full recovery within two weeks to one year of follow-up.
    CONCLUSIONS: We reported a patient with asymmetric bifacial palsy and bulbar palsy, which seemed to fit the diagnosis of ABPp syndrome. This was the first report of ABPp variant of GBS with positive serum ganglioside GD3 IgG antibody.
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  • 文章类型: Journal Article
    目的:大多数研究通过比较COVID-19大流行之前和期间GBS的发病率来调查COVID-19与格林-巴利综合征(GBS)之间的关系。然而,调查结果不一致,可能是由于不同程度的封锁政策。中国的检疫要求和旅行限制在2022年12月7日左右解除。这项研究旨在探讨在中国没有COVID-19强制的社会限制的情况下,GBS的相对频率在重大疫情期间是否增加。
    方法:第一医院收治的GBS患者,山西医科大学,从2022年12月7日到2023年2月20日,从6月,2017年8月,包括2019年。比较不同时期住院患者GBS的相对频率。在GBS发病前6周内有和无SARS-CoV-2感染的患者组成COVID-GBS组和非COVID-GBS组,分别。
    结果:COVID-19大爆发期间(13/14,408)住院患者GBS的相对频率明显高于COVID-19流行前(29/160,669,P<0.001)。出现AIDP亚型的COVID-GBS患者(11/13)多于非COVID-GBS患者(10/27,P=0.003)。COVID-GBS的感染性症状发作与GBS之间的平均间隔(21.54±11.56天)长于非COVID-GBS(5.76±3.18天,P<0.001)。
    结论:COVID-19显著增加GBS的发病率。大多数COVID-GBS患者属于AIDP类别,对IVIg反应良好,预后良好.
    OBJECTIVE: Most studies investigated the relationship between COVID-19 and Guillain-Barré syndrome (GBS) by comparing the incidence of GBS before and during the pandemic of COVID-19. However, the findings were inconsistent, probably owing to varying degrees of the lockdown policy. The quarantine requirements and travel restrictions in China were lifted around December 7, 2022. This study aimed to explore whether the relative frequency of GBS increased during the major outbreak in the absence of COVID-19-mandated social restrictions in China.
    METHODS: GBS patients admitted to the First Hospital, Shanxi Medical University, from December 7, 2022 to February 20, 2023, and from June, 2017 to August, 2019 were included. The relative frequencies of GBS in hospitalized patients during different periods were compared. The patients with and without SARS-CoV-2 infection within six weeks prior to GBS onset formed the COVID-GBS group and non-COVID-GBS group, respectively.
    RESULTS: The relative frequency of GBS among hospitalized patients during the major outbreak of COVID-19 (13/14,408) was significantly higher than that before the COVID-19 epidemic (29/160,669, P < 0.001). More COVID-GBS patients (11/13) presented AIDP subtype than non-COVID-GBS cases (10/27, P = 0.003). The mean interval between onset of infective symptoms and GBS was longer in COVID-GBS (21.54 ± 11.56 days) than in non-COVID-GBS (5.76 ± 3.18 days, P < 0.001).
    CONCLUSIONS: COVID-19 significantly increased the incidence of GBS. Most COVID-GBS patients fell into the category of AIDP, responded well to IVIg, and had a favorable prognosis.
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  • 文章类型: Journal Article
    背景:格林-巴利综合征(GBS)是一种自身免疫性疾病,通常在先前的胃肠道(GI)感染后发展。然而,由于各种挑战,肠道菌群(GM)和GBS之间的确切关联仍然未知.本研究旨在通过双样本孟德尔随机化(TSMR)分析来调查GM和GBS之间的潜在因果关系。
    方法:利用MiBioGen联盟(n=13,266)提供的最大的全基因组关联研究(GWAS)荟萃分析作为基础,我们进行了TSMR来破译GM和GBS之间的因果关系。采用了各种分析方法,包括逆方差加权(IVW),MR-PRESSO,MR-Egger,和加权中位数。工具变量(IVs)的异质性使用Cochran的Q统计进行评估。
    结果:分析确定了三个微生物类群与GBS的风险关联显著增加,包括牙本质反乳球菌组(OR=1.40,95%CI:1.07-1.83),ruminococusgaovreauii组(OR=1.51,95%CI:1.02-2.25),和反刍动物科UCG009(OR=1.42,95%CI:1.02-1.97),而Eubacteriumbrachy组(OR=1.44,95%CI:1.10-1.87)和Romboutsia(OR=1.67,95%CI:1.12-2.47)显示出暗示性因果关系。另一方面,RuminocycaceaeUCG004(OR=0.61,95%CI:0.41-0.91)对GBS具有保护作用,而芽孢杆菌(OR=0.60,95%CI:0.38-0.96),γ蛋白细菌(OR=0.63,95%CI:0.41-0.98)和LachnoshispileaceaeUCG001(OR=0.69,95%CI:0.49-0.96)显示出对GBS的暗示性保护性关联。
    结论:MR分析表明特定GM分类群与GBS风险之间存在潜在的因果关系。然而,为了验证这些发现,必须进行涉及多元化人群的进一步广泛研究。
    BACKGROUND: Guillain-Barré Syndrome (GBS) is an autoimmune disease that typically develops after a previous gastrointestinal (GI) infection. However, the exact association between Gut Microbiota (GM) and GBS still remains unknown due to various challenges. This study aimed to investigate the potential causal association between GM and GBS by using a two-sample Mendelian Randomization (TSMR) analysis.
    METHODS: Utilizing the largest available genome-wide association study (GWAS) meta-analysis from the MiBioGen consortium (n = 13,266) as a foundation, we conducted a TSMR to decipher the causal relationship between GM and GBS. Various analytical methods were employed, including the inverse variance weighted (IVW), MR-PRESSO, MR-Egger, and weighted median. The heterogeneity of instrumental variables (IVs) was assessed using Cochran\'s Q statistics.
    RESULTS: The analysis identified three microbial taxa with a significantly increased risk association for GBS, including Ruminococcus gnavus group (OR = 1.40, 95 % CI: 1.07-1.83), Ruminococcus gauvreauii group (OR = 1.51, 95 % CI: 1.02-2.25), and Ruminococcaceae UCG009 (OR = 1.42, 95 % CI: 1.02-1.97), while Eubacterium brachy group (OR = 1.44, 95 % CI: 1.10-1.87) and Romboutsia (OR = 1.67, 95 % CI: 1.12-2.47) showed a suggestively causal association. On the other hand, Ruminococcaceae UCG004 (OR = 0.61, 95 % CI: 0.41-0.91) had a protective effect on GBS, while Bacilli (OR = 0.60, 95 % CI: 0.38-0.96), Gamma proteobacteria (OR = 0.63, 95 % CI: 0.41-0.98) and Lachnospiraceae UCG001 (OR = 0.69, 95 % CI: 0.49-0.96) showed a suggestively protective association for GBS.
    CONCLUSIONS: The MR analysis suggests a potential causal relationship between specific GM taxa and the risk of GBS. However, further extensive research involving diversified populations is imperative to validate these findings.
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  • 文章类型: Journal Article
    背景:格林-巴利综合征(GBS)是一种急性周围神经病变的缩影,其特征是针对包裹周围神经的髓鞘的自身免疫蒸馏。虽然人们普遍认为大多数GBS患者都有既往感染史,术后GBS发生的记录正在逐步增加。根据最近病例报告的详尽汇编,这种疾病的起源范围从1小时到1.2年不等。
    方法:此时此刻,我们提供了一个独特的案例:一个涉及一名51岁的绅士接受腰椎手术的案例,只会遇到肢体和呼吸肌肉组织的立即衰弱。
    方法:消除与麻醉剂相关的变量后,脑,和脊髓病变,出现了对超急性GBS发作的强烈怀疑。
    方法:免疫球蛋白治疗后,血浆置换,和辅助支持,病人的最终死亡变得明显。
    结果:迄今未发现任何进展。
    结论:鉴于GBS可能引发瘫痪,呼吸衰竭,和自主神经系统畸变,除了其他有害的后遗症,再加上在这种特殊情况下时间发作的异常罕见,无可否认,它在鉴别诊断和治疗行为领域为麻醉师提供了一个令人瞩目的难题。在麻醉下的术后恢复期,如果患者表现出异常的四肢肌肉组织活力和呼吸肌腱受损,GBS的前景绝不能被遗忘。诊断的准确性与适当的治疗措施相结合,很可能预示着患病患者的不同结局。
    BACKGROUND: Guillain-Barré syndrome (GBS) epitomizes an acute peripheral neuropathy hallmarked by an autoimmune retort directed at the myelin sheath enwrapping peripheral nerves. While it is widely acknowledged that a majority of GBS patients boast a history of antecedent infections, the documentation of postoperative GBS occurrences is progressively mounting. Drawing upon an exhaustive compendium of recent case reports, the disease\'s inception spans a gamut from within 1 hour to 1.2 years.
    METHODS: At this juncture, we proffer a singular case: an instance involving a 51-year-old gentleman who underwent lumbar spine surgery, only to encounter immediate debilitation of limb and respiratory musculature.
    METHODS: Post elimination of variables linked to anesthetic agents, encephalon, and spinal cord pathologies, a potent suspicion of superacute GBS onset emerged.
    METHODS: Subsequent to immunoglobulin therapy, plasmapheresis, and adjunctive support, the patient\'s ultimate demise became manifest.
    RESULTS: No progress was found to date.
    CONCLUSIONS: Given GBS\'s potential to instigate paralysis, respiratory collapse, and autonomic nervous system aberrations, alongside other pernicious sequelae, coupled with the exceptional rarity of the temporal onset in this particular instance, it undeniably proffers an imposing conundrum for anesthetists in the realm of differential diagnosis and therapeutic conduct. During the postoperative convalescence phase under anesthesia, should the patient evince deviant limb musculature vigor and compromised respiratory sinews, the prospect of GBS must not be consigned to oblivion. Precision in diagnosis conjoined with apt therapeutic measures could well be the harbinger of a divergent denouement for the afflicted patient.
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  • 文章类型: Case Reports
    背景:斑疹伤寒是一种由丘疹虫东方体引起的急性传染病。格林-巴利综合征(GBS)是一种自身免疫介导的周围神经病变,具有频繁的前驱感染史,但是与斑疹伤寒相关的GBS非常罕见。
    方法:我们报告了一名51岁的男性患者,他在斑疹伤寒治疗后出现构音障碍和周围性面瘫。CSF检查和电生理发现提示GBS的诊断。静脉注射免疫球蛋白治疗后,病人的神经状况迅速改善。
    结论:斑疹伤寒感染可能是GBS的潜在诱发因素,而与斑疹伤寒相关的GBS预后良好。
    BACKGROUND: Scrub typhus is an acute infectious disease caused by Orientia tsutsugamushi. Guillain-Barre syndrome (GBS) is an autoimmune-mediated peripheral neuropathy with a frequent history of prodromal infections, but GBS associated with scrub typhus is very rare.
    METHODS: We report a 51-year-old male patient who developed dysarthria and peripheral facial paralysis following the cure of scfrub typhus. CSF examination and electrophysiological findings suggested a diagnosis of GBS. After treatment with intravenous immunoglobulin, the patient\'s neurological condition improved rapidly.
    CONCLUSIONS: Scrub typhus infection is likely to be a potential predisposing factor in GBS, while scrub typhus-associated GBS has a favorable prognosis.
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  • 文章类型: Journal Article
    SARS-CoV-2导致2019年冠状病毒病(COVID-19),一种没有或没有一种或多种症状的传染病:发烧,咳嗽,头痛,喉咙痛,失去味道和气味,疼痛,疲劳和肌肉骨骼疼痛。为了预防COVID-19,有可用的疫苗,包括辉瑞公司开发的疫苗,Moderna,Sinovac,Janssen,和阿斯利康。最近的证据表明,一些接种COVID-19的个体偶尔会发展为格林-巴利综合征(GBS)的潜在副作用,一种严重的神经系统自身免疫性疾病,其中针对周围神经系统(PNS)的免疫反应可导致显著的发病率。GBS以前与几种病毒或细菌感染有关,接种某些COVID-19后发现GBS,虽然罕见,应提醒医生早期诊断和有针对性的治疗。在这里,我们回顾了5例在不同国家接种COVID-19疫苗后出现的GBS病例。
    SARS-CoV-2 causes Coronavirus Disease 2019 (COVID-19), an infectious condition that can present none or one or more of these symptoms: fever, cough, headache, sore throat, loss of taste and smell, aches, fatigue and musculoskeletal pain. For the prevention of COVID-19, there are vaccines available including those developed by Pfizer, Moderna, Sinovac, Janssen, and AstraZeneca. Recent evidence has shown that some COVID-19-vaccinated individuals can occasionally develop as a potential side effect Guillain-Barre syndrome (GBS), a severe neurological autoimmune condition in which the immune response against the peripheral nerve system (PNS) can result in significant morbidity. GBS had been linked previously to several viral or bacterial infections, and the finding of GBS after vaccination with certain COVID-19, while rare, should alert medical practitioners for an early diagnosis and targeted treatment. Here we review five cases of GBS that developed in different countries after COVID-19 vaccination.
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  • 文章类型: Case Reports
    背景:目前的研究报告了一例有神经泌尿道炎病史的病例。在COVID-19疫苗接种的2个月内,急性腹泻后出现严重的格林-巴利综合征(GBS),进行性肌无力,以及突然的呼吸和心脏症状.
    方法:通过措施解决了综合征,如气管插管和心肺复苏血管活性药物。接下来,我们进行了6个周期的人免疫球蛋白治疗(剂量为400mg/kg·d,连续5天)和3次血浆置换(PE,30ml/kg),其次是甲基强的松龙琥珀酸钠。康复训练持续进行。
    结果:患者意识恢复正常,他进行了正常的交流。肌肉力量逐渐恢复,但仍不能独立站立。目前,他正在家里康复。
    结论:对于先前患有神经根炎的患者,COVID-19疫苗接种可能会增加对GBS的易感性。因此,建议延长这些患者的疫苗接种间隔,并确保持续评估任何可能增加的风险.
    BACKGROUND: The current study reported a case with a history of neuroradiculitis. Within 2 months of the COVID-19 vaccine, critical Guillain-Barre Syndrome (GBS) appeared after acute diarrhea, progressive myasthenia, and sudden respiratory and cardiac symptoms.
    METHODS: The syndrome was addressed with measures, such as endotracheal intubation and cardiopulmonary resuscitation vasoactive drugs. Next, we conducted six cycles of human immunoglobulin treatment (dose of 400 mg/kg·d intravenously for 5 days consecutively) and three times plasma exchange (PE, 30 ml/kg), followed by methylprednisolone sodium succinate. Rehabilitation training was carried out continuously.
    RESULTS: The consciousness of the patient returned to normal, wherein he carried out normal communication. The muscle strength recovered gradually but still could not stand independently. Presently, he is recovering at home.
    CONCLUSIONS: For patients with previous radiculitis, COVID-19 vaccination may increase the susceptibility to GBS. Thus, it is recommended to extend the vaccination interval for these patients and ensure that any potential increased risk is continually assessed.
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