A Case Report of Recurrent Guillain-Barré Syndrome with Orthostatic Hypotension Syncope as the First Symptom

Guillain⁃Barré syndrome (GBS) is an immune-mediated peripheral neuropathy with acute or subacute onset of flaccid paralysis of the limbs with symmetrical hypesthesia and autonomic nerve involvement [1]. The clinical manifestations of autonomic nerve damage are complex and varied, which may involve extensive or limited autonomic function damage, including abnormalities of the skin, pupil, urinary tract, gastrointestinal tract, cardiovascular system, body temperature, lacrimal and salivary glands, and sexual function, etc. [2], and some patients may even have autonomic nerve damage as the only symptom, which is a variant of GBS and is prone to misdiagnosis or underdiagnosis. Recurrence of GBS is rare, and the manifestations of recurrence are often similar to those of the first symptoms [3], but the patient admitted to our hospital had syncope as the main clinical manifestation of recurrence, which was completely different from that of the first incidence, and syncope is not a common and typical clinical manifestation of GBS, so misdiagnosis is highly likely.

A Case-control Study on Children with Guillain-barre Syndrome in North China

To explore the risk factors for Guillain-barre syndrome. Methods Case-control study design was used in 51 cases of Guillain-barre syndrome,and 51 matched controls.All of the 51 cases in this study had been examined by electrophysiology. Serum IgG antibodies specific for C. jejuni were determined in all the subjects by ELISA. Each case and control were interviewed using an ad hoc questionnaire, including his/her demographic information,onset of the illness, their personal hygiene and so on. Results The study showed that Guillain-barre syndrome was associated with a few factors, such as polio vaccine immunization before onset of illness (OR=7.27), no hand washing after defecation and before meals (OR=6.15). Infection of C. jejuni was strongly associated with the illness (OR=9.5,P<0.001). Conclusion It is suggested that occurrence of Guillain-barre syndrome may correlate to infection of C. jejuni and poor personal hygiene in children.

Variant of Guillain-Barrésyndrome with anti-sulfatide antibody positivity and spinal cord involvement:A case report

BACKGROUND Guillain-Barrésyndrome(GBS)is an acute autoimmune-mediated polyneuropathy.Studies have increasingly reported the presence of anti-sulfatide antibody positivity with varying clinical symptoms in patients with GBS.However,spinal cord involvement is relatively rare in these cases.CASE SUMMARY A 68-year-old woman was admitted to the hospital with weakness of the limb for more than 3 d.Additional symptoms included neck pain,progressive numbness in the distal extremities,urinary and fecal retention,and reduced perception of temperature.She was diagnosed with an anti-sulfatide antibody-positive GBS variant and discharged after treatment with methylprednisolone and intravenous human immunoglobulin pulse therapy.Unlike common cases of anti-sulfatide antibody-positive GBS,this patient had atypical clinical symptoms of spinal cord involvement.No similar cases have previously been reported in China.CONCLUSION Although GBS is associated with a poor prognosis,a prompt diagnosis allows early administration of combined intravenous human immunoglobulin and methylprednisolone pulse therapy.

Peroral endoscopic myotomy for treatment of Guillain-Barre syndrome-associated achalasia: A rare case

Guillain-Barre syndrome (GBS)-associated achalasia is a very rare disease of uncertain cause. We report the case of a patient diagnosed with GBS-associated type I achalasia who was successfully treated with peroral endoscopic myotomy (POEM). A 30-year-old man who was diagnosed with GBS 3 mo before was referred to our department with dysphagia and meal-related regurgitation. The results of esophagography, endoscopy, and high-resolution manometry (HRM) revealed type I achalasia. POEM that utilized a submucosal tunneling technique was performed to treat the GBS-associated type I achalasia. After POEM, smooth passage of a contrast agent into the stomach was shown in follow-up esophagography, and follow-up HRM revealed a decrease in the mean integrated relaxation pressure 22.9 mmHg to 9.6 mmHg. The patient remained without dysphagia for 7 mo, even though the patient&#x02019;s neurological problems were not fully resolved. POEM may be a safe and effective treatment for GBS-associated type I achalasia.

Diagnosis and Treatment of Diabetic Ketoacidosis Mellitus with Guillain-Barré Syndrome: A Case Report

This article reports the diagnosis and treatment of a case of Diabetic ketoacidosis with Guillain-Barré syndrome. Diabetic ketoacidosis (DKA) is the most common acute diabetes mellitus, often diabetes and infection, insulin withdrawal or interruption of the history of triggers, with hyperglycemia, ketoacidosis, and acid poisoning as the main symptoms, rapid onset of ill-ness, and serious illness. Guillain-Barré syndrome (GBS) is an autoimmune-mediated peripheral neuropathy with frequent respiratory or gastrointestinal tract infections and low clinical incidence before 1 - 3 weeks. This case was characterized by a headache with vomiting acute onset, a relatively clear diagnosis of diabetic ketoacidosis, a symmetrical decrease in muscle strength in the extremities, and recovery of consciousness after aggressive correction of environmental disturbance in electrolytes, but very poor muscle strength in the extremities, protein-cell segregation in cerebrospinal fluid (CSF). Considering Guillain-Barré syndrome, the patient’s muscle strength gradually recovered after treatment with proglobulin shock. At present, the pathogenesis of the two is not clear, but because of its rapid progress, clinicians should raise awareness of diabetic ketoacidosis combined with Guillain-Barré syndrome, early diagnosis, and active treatment. Inform consent has been obtained from the patient for this report.

Miller fisher syndrome with positive anti-GQ1b/GT1a antibodies associated with COVID-19 infection:A case report

BACKGROUND Miller fisher syndrome(MFS)is a variant of Guillain-Barrésyndrome,an acute immune-mediated peripheral neuropathy that is often secondary to viral infections.Anti-ganglioside antibodies play crucial roles in the development of MFS.The positive rate of ganglioside antibodies is exceptionally high in MFS patients,particularly for anti-GQ1b antibodies.However,the presence of other ganglioside antibodies does not exclude MFS.CASE SUMMARY We present a 56-year-old female patient who suddenly developed right blepharoptosis and progressively worsening vision in both eyes.There were flu symptoms prior to onset,and a coronavirus disease 2019 test was positive.On physical examination,the patient exhibited bilateral extraocular muscle paralysis,weakened reflexes in both limbs,and impaired coordination.The cerebrospinal fluid examination results showed no obvious abnormalities.Bilateral peroneal nerve F-waves were not extracted.Serum anti-GD1b IgG and anti-GT1a IgG antibodies were positive.The patient received intravenous methylprednisolone(1000 mg/day),with the dosage gradually decreased.Additionally,intravenous high-dose immunoglobulin treatment was administered for 5 days(0.4 g/kg/day)from day 2 to day 6 of hospitalization.The patient’s symptoms improved after treatment with immunoglobulins and hormones.CONCLUSION Positive ganglioside antibodies may be used as supporting evidence for the diagnosis;however,the diagnosis of MFS is more reliant on clinical symptoms.

Effects of increased human tumor necrosis factor-like molecule 1A expression in peripheral blood of children with acute Guillain-Barre syndrome on interferon-gamma secretion

BACKGROUND： Human tumor necrosis factor-like molecule 1A （hTL1A） is a strong T helper cell type 1 （Thl） co-stimulator. Guillain-Barre syndrome （GBS） is an autoimmune disorder of the nervous system, which is mediated by Thl cells. OBJECTIVE： To determine hTL1A expression in peripheral blood T lymphocytes of acute GBS children and the effects of hTL1A on secretion of interferon-γ. DESIGN, TIME AND SETTING： A randomized, controlled, neuroimmunological in vitro study was performed at the Central Laboratory of First Hospital of Jilin University, China from November 2005 to November 2007. MATERIALS： Venous blood samples were obtained from 6 healthy donors, aged 6-12 years （all routine blood examination items were normal）, and 6 additional children with acute GBS, aged 6-12 years. The GBS children fell ill within 1 week and were not treated with hormones or immunoglobulin Purified recombinant human soluble tumor necrosis factor-like molecule 1A （rhsTL1A, 1 mg/mL, relative molecular mass 22 000, 6× His tag, soluble form） was supplied by the Central Laboratory of First Hospital of Jilin University, China. METHODS： Peripheral blood mononuclear cells were isolated from healthy donors using the standard Ficoll gradient centrifugation and were incubated in 96-well culture plates. The cells were assigned to the following groups： control （2 μg/mL phytohemagglutinin）, 2μg/mL phytohemagglutinin ＋ 25, 100 and 400 ng/mL rhsTL1A. T cell proliferation was quantified using the tritiated thymidine （3H-TdR） method. Serum interferon-γ levels in acute GBS children were detected by enzyme-linked immunosorbent assay （ELISA）. The ratio of hTL1A-positive T cells to CD3-positive T cells in peripheral blood of acute GBS children was determined using flow cytometry. Following in vitro pre-activation of peripheral blood mononuclear cells by 2 μg/mL phytohemagglutinin, the peripheral blood mononuclear cells were treated with 400 ng/mL exogenous rhsTLIA. Finally, peripheral blood mononuclear cell-secreted interferon-γlevels were measured by ELISA. MAIN OUTCOME MEASURES： The following parameters were measured： rhsTLIA stimulation index to stimulate proliferation of T cells; the serum interferon-γ levels in acute GBS children; the ratio of hTL1A-positive cells to CD3-positive cells; the levels of interferon-γ secreted by peripheral blood mononuclear cells in acute GBS children, as well as rhsTL1A-stimulated interferon-γ levels. RESULTS： T cell proliferation assay revealed that the stimulation index in each rhsTL1A group was greater than the control group. The stimulation index of the 400 ng/mL rhsTL1A group was the greatest. Serum interferon-γ levels in acute GBS children were significantly greater than the control group （P 〈 0.05）. The ratio of hTLIA＋ CD3＋ T cells to CD3＋ T cells in acute GBS children was significantly greater than the control group （P 〈 0.01 ）. Phytohemagglutinin stimulated peripheral blood mononuclear cells to a greater extent than 400 ng/mL rhsTL1A in the acute GBS group, and the secreted interferon-γ levels were significantly increased （P 〈 0.05）. CONCLUSION： In T cells pre-activated with 2 μg/mL phytohemagglutinin, proliferation was effectively increased with 400 ng/mL rhsTL1A treatment. Expression of hTLIA was increased in activated T cells from peripheral blood of acute GBS children, followed by increased interferon-γ secretion. These mechanisms are considered to be part of the pathological process that induces the secretion of inflammatory cytokines in GBS syndrome.

Guillain-Barre syndrome associated with peginterferon alfa-2a for chronic hepatitis C: A case report

The recommended therapy for chronic hepatitis C (CHC) infection is the combination of a Pegylated interferon and Ribavirin. Almost all such patients on combination therapy experience one or more adverse events during the course of treatment. Significant neurological side effects are rare. A few cases of Bell's Palsy, chronic inflammatory demyelinating polyneuropathy and even one case of acute demyelinating polyneuropathy with atypical features for Guillain-Barre syndrome (GBS) associated with Interferon therapy have been reported but no report of GBS with typical features has been published. We present a case report of typical GBS associated with Peginterferon alfa-2a and Ribavirin used for treatment of CHC infection.

Association of neuroelectrophysiology and analysis of cerebrospinal fluid immunoglobulin with pathogenetic conditions of patients with Guillain-Barre syndrome

BACKGROUND： Guillain-Barre syndrome （GBS） is an autoimmune disease which is characterized by demyelination of peripheral nerve and nerve root, and inflammatory reaction of lymphocyte and macrophage. Neuroelectrophysiological examination and cerebrospinal fluid （CSF） analysis are of significance for its diagnosis. OBJECTIVE： To study the association of neuroelectrophysiology and cerebrospinal fluid immunoglobulin （CSF-lg） with pathogenetic conditions of patients with GBS. DESIGN： Case control study SETTING： Department of Neurology, Shenzhen Municipal Shekou Group Hospital; Department of Neuroelectrophysiology, People＇s Hospital of Guangdong Province. PARTICIPANTS： A total of 32 GBS patients including 18 males and 14 females who aged from 17 to 72 years were selected as experimental group from the Department of Neurology, People＇s Hospital of Guang- dong Province from January 2004 to December 2005. All cases conformed with GBS diagnostic criteria established by Asbury in 1990 and they were divided into three types according to neurological criteria established by Chinese Neurology and Psychology Journal in 1993： mild, moderate and severe types. Another 30 patients with vascular headache were selected as control group from the same hospital including 14 males and 16 females who aged from 17 to 79 years. METHODS： ① Neuroelectrophysiological examination： Multiple-functional electromyography device provided by Nicolet Company, USA was used to measure nerve conduction velocity （NCV）, including motor nerve conduction velocity （MCV） and sensory nerve conduction velocity （SCV）; meanwhile, electromyologram （EMG）, somatosensory evoked potential （SEP） and electroencephalogram （EEG） were also measured. ② Detection of CSF-lg： Concentrations of IgG, IgA and IgM were measured with immunofixation electrophoresis. ③Follow-up： Among 32 GBS patients, 14 cases received follow-up after treatment and the longest fol- low-up time was 1 year after onset. Among them, 8 cases were reexaminined with neuroelectrophysiological and CSF examinations. MAIN OUTCOME MEASURES： Results of NCV, EMG, SEP and EEG; comparison of CSF-lg content; results of follow-up examinations. RESULTS： All 32 GBS cases and 30 patients with vascular headache were involved in the final analysis. ① Abnormal rate of neuroelectrophysiological test： 75% of NCV, 88% of F-wave, 53% of MCV, 25% of SEP, 47% of EMG and 31% of EEG. There were no significant differences among various types （P 〉 0.05）. ② Results of CSF-lg test： There were no significant differences among various types （P 〉 0.05）; however, abnormalities in experimental group was higher than those in control group （P 〈 0.01）. CONCLUSION ： Results of follow-up study suggest that improvement of clinical symptom is earlier than neuroelectrophysiological recovery; MCV and EMG recoveries are faster than that of NCV; the earlier the abnormality of EMG, the poorer the recovery. CSF4g recovers normally along improvement of clinical symptoms. It is of significance for neuroelectrophysiology and abnormality of CSF-Ig to determine degree of peripheral nerve demyelination and prognosis.

A rare presentation of Guillain-Barre syndrome with GQ1b positivity:A case report

Rationale:To report a case of cervicobrachial variant of acute inflammatory demyelinating polyneuropathy presenting with papilledema and GQ1b positivity.Patient concern:A 35-year-old female,68 days postpartum,presented with headache,vomiting,and gait difficulty in swallowing with bilateral upper limb weakness and difficulty in walking,13 days after ChAdOx1 nCoV-19 vaccination.Diagnosis:Guillain-Barre syndrome with GQ1b positivity.Intervention:Five cycles of plasmapheresis were given.Outcome:The patient’s clinical condition improved.Palatal weakness improved and she could walk without support.There were mild sensory symptoms involving upper limbs which gradually improved.Lessons:AIDP should be considered in case of weakness following ChAdOx1 nCoV-19 vaccination.Albumino-cytological dissociation and anti-GQ1b positivity are needed to confirmed the diagnosis.

Asymmetric limb weakness in Guillain-Barre syndrome:Three case reports

BACKGROUND Guillain-Barrésyndrome(GBS)is an autoimmune-mediated peripheral neuropathy characterized by symmetric weakness.Asymmetric weakness in GBS is uncommon and may be easily confused with other differential diagnoses.We herein present three cases of asymmetric GBS and review the literature on this atypical subtype of GBS in order to describe the characteristics of asymmetric GBS and to provide experience for clinicians.CASE SUMMARY Different from patients in the previous reports,our patients showed persistent asymmetric limb weakness from the onset to recovery phase.All three patients were serologically positive for antecedent infections.Two of the three cases had IgG antibodies against ganglioside GM1.Two patients received immunotherapy including intravenous immunoglobulin and plasma exchange,while one patient received only supportive treatment.Autoantibodies against gangliosides,asymmetry of congenital development of blood-nerve barrier and limb use may contribute to the development of asymmetric limb weakness in GBS.CONCLUSION Asymmetric GBS may be a rare clinical variant and should be considered when a patient develops acute and progressive asymmetric limb weakness.The differences in clinical features and prognosis between asymmetric GBS and classic GBS deserve further investigation in a large study.

Clinically Diagnosed Guillain-Barre Syndrome in Pregnancy: Case Report and Review of Literature

Background: Guillain-Barre syndrome (GBS) is an autoimmune disorder characterized by a heterogeneous group of pathological and clinical entities. It is associated with ascending areflexic paralysis, some autonomic dysfunction and respiratory failure in severe cases and ultimately death if not promptly diagnosed and treated. It may be preceded by an antecedent event in about two-third of cases. This could be an upper respiratory tract infection, viral illness, recent history of vaccination, pregnancy, cancer or even trauma. The condition is exceedingly rare in pregnancy and only few cases have been reported in literature. Case Report: This is a case of a 28-year-old Gravida 3, Para 1+1 and Estimated Gestational Age of 30 weeks and 4 days. There was a history of upper respiratory tract infection eight weeks prior to presentation which spontaneously resolved. On examination, she was a young woman, anxious, weak, afebrile, not pale, the neck could not hold the head upright and there was bilateral non tender pitting pedal oedema extending to her mid-shin. There were no cranial nerve deficits and no sign of meningeal irritation. There were normal muscle bulk with global hypotonia and flaccid quadriparesis, Power was 3/5. The proximal groups of muscles were more affected than the distal parts. Reflexes were diminished globally with plantar flexor response. She had immunoglobulin as treatment. Conclusion: In a low resource setting like ours it is important to have a high index of suspicion of GBS when an apparently healthy gravid woman presents with progressive weakness of the limbs.

Exceptional Association of a Cerebral Sinus Thrombosis and a Guillain-Barre Syndrome: A New Case Report and Review of the Literature

Background: The association of Guillain-Barre syndrome and cerebral sinus thrombosis is uncommon. Case Presentation: We report a 37-year-old patient hospitalized in medical ICU for respiratory distress following a Guillain-Barre syndrome. He had symptomatic treatment in addition to plasma exchange. In the presence of clonic movements, a brain venography magnetic resonance showed a thrombophlebitis of the left lateral sinus, and hence a low-molecular-weight heparin treatment was begun. Immunological, thrombophilia and serological tests were negative. After a favorable evolution, he was transferred to the neurology department. Conclusion: The combination of a Guillain-Barre syndrome and a cerebral sinus thrombosis would suggest a common process. A rigorous investigation, including the use of imaging, is necessary in front of any unusual clinical sign during a GBS.

COVID-19 Infection Presenting as Myalgia, Abnormal Liver Function Tests and the Guillain-Barre Syndrome

The severe acute respiratory syndrome coronavirus 2 infection typically presents with respiratory symptoms. Additionally, there are a number of less frequent neurological manifestations of infection with the coronavirus disease 2019 (COVID-19) with case reports suggesting an association with the Guillain-Barre syndrome. Most patients present with the typical upper respiratory symptoms in association with these neurological symptoms. We present a case of an unvaccinated gentleman with none of the typical respiratory symptoms of COVID-19 who presented with the Guillain Barre syndrome and myalgia. His symptoms settled following treatment with intravenous immunoglobulins. This case highlights the importance of testing for COVID-19 in patients without typical symptoms but who present with neurological illness and supports the use of intravenous immunoglobulin therapy.

Guillain-Barre综合征合并中枢神经系统脱髓鞘病变的临床研究

目的 探讨Guillain Barre综合征 (GBS)合并中枢神经系统 (CNS)脱髓鞘病变及两者之间的发病关系和可能的发病机制。方法 对 3例合并CNS脱髓鞘病变的GBS患者的临床及实验室资料进行分析。结果 例 1男 ,2 8岁 ,主要表现为全身软瘫和昏迷 ,血气分析结果正常 ,脑脊液有蛋白细胞分离、寡克隆带阳性 ,肌电图提示神经源性损害 ;MRI示双侧脑白质及颈髓广泛多发性脱髓鞘改变 ;应用血浆和免疫球蛋白治疗后好转。例 2女 ,5岁 ,因进行性四肢无力 ,呼吸困难入院 ,应用呼吸机和血浆治疗过程中出现脑干反射障碍 ,后放弃治疗死亡 ;死后行腓肠神经病检示周围神经脱髓鞘病变。例 3男 ,12岁 ,因进行性四肢无力伴大小便困难入院 ;颅脑MRI未见异常 ,脊髓MRI见T5～L4节段广泛脱髓鞘病变 ;脑脊液有蛋白细胞分离 ,寡克隆带阳性 ;肌电图示周围神经传导速度减慢。结论 合并意识障碍的GBS大多病情危重 ,其发病机制不详 。

Guillain-Barre综合征和Miller Fisher综合征的新诊断分类和标准

2014年8月Nature Review of Neurology杂志在Perspective栏目中发表GBS分类专家组（the GBS Classification Group）对Guillain-Barre综合征（GBS）和、Miller Fisher综合征（MFS）的新分类和诊断标准[1],将GBS、MFS和Bickstaffer脑干脑炎（BBE）作为一个疾病谱,并按照临床受累部位对此疾病谱中的表型进行了分类,并提出了诊断标准。新诊断标准沿用了临床核心特征和支持特征的表述,并对核心特征做出注解（表1）。

Guillain-Barre综合征的神经电生理学特点

神经电生理榆查对于Guillain-Barre综合征（GBS）的辅助诊断、治疗指导及预后判断有重要的意义。现对我院收治的28例GBS患者的神经电生理检查结果分析如下。

综合干预对GBS患儿血浆置换疗效和安全性的影响

目的观察综合干预对血浆置换(PE)治疗格林巴利综合征(GBS)患儿的疗效和安全性的影响。方法回顾性分析2 012年1月至2013年12月在西安市儿童医院住院治疗的54例GBS患儿临床资料,按其治疗时间2012年选取的病例为对照组,2013年为观察组,观察组和对照组各选27例资料完整的病例进行分析。两组患儿均给予内科综合治疗、PE治疗和基础护理,观察组在对照组基础上对患儿实施预见性综合干预措施,分析两组患儿住院时间、疗效及副反应等。结果观察组惠儿疗效显著高于对照组,且住院时间短于对照组,异常情况发生率低于对照组,以上指标两组之间比较,差异均有统计学意义(x^2=7.94,t=2.15,x^2=14.34;均P<0.05)。结论综合干预可提高GBS患儿PE治疗的疗效和安全性,缩短患儿住院时间。

Studies on Human Immunoglobulin G from GBS Patient (Ⅲ)-The Determination of Molecular Weight of Human Immunoglobulin G by Capillary SDS Gel Electrophoresis

Guillain-Barre syndrome (GBS) is considered to be an autoimmune disorder of peripheralnervous system. In this paper. capillary SDS gel electrophoresis was performed on neutral coatedfused-silica capillary to determine the molecular weight of purified IgG samples from GBS patient.