Guillain-Barre syndrome and Campylobacter jejuni infection

Guillain–Barre syndrome (GBS) is an acute onset, usually monophasic immune-mediated disorder of the peripheral nervous system. The term GBS is often considered to be synonymous with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), but with the increasing recognition of variants over the past few decades, the number of diseases that fall under the rubric GBS have grown to include axonal variants and more restricted variants, such as Miller Fisher syndrome (MFS) [Table 1].[1] Table 1 Guillain–Barre syndrome—clinical variants Epidemiology The reported incidence rates for GBS are 1–2 per 100,000 population.[2–4] The lifetime likelihood of any individual acquiring GBS is 1:1000.[5] The subtypes of GBS have different incidence rates in different parts of the world. In Europe and North America AIDP is dominant contributing to 90% of the cases. In contrast in China and Japan AMAN being the most common subtype.[6,7] The picture is intermediate when we look at other population. In Indian series the incidence of AIDP and AMAN are virtually equal although AMAN is more common in younger patients.[8] There seems to be a slight preponderance of AIDP in studies by Gupta et al[9] and by Meena et al (unpublished data from NIMS, Hyderabad). Available Indian literature indicates a peak incidence between June–July and Sept–October.[10] In western countries, GBS is common in the 5th decade,[11] but in India it occurs more commonly at a younger age.[10,12] GBS is equally common in men and women and can occur at any age. There is a male preponderance among the hospitalized population.[10,12]

To study the clinical characteristics and effects of immunoglobulin treatment in children with the different types of Guillain-Barré syndrome (GBS). Data of 108 patients hospitalized for GBS were retrospectively analyzed; 75 cases in this group were given acute high dose of gamma globulin (IVIG) 400 mg/(kg·d) intravenously for 5 d. Clinical and electrophysiological data and information on treatment and recovery of the children were collected during the follow-up and were analyzed. According to the clinical and electrophysiologic findings, 32 patients manifested acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 34 had acute motor axonal neuropathy (AMAN), 3 had acute motor and sensory axonal neuropathy (AMSAN), 4 were inexcitable, 2 were unclassified. The clinical progress of the AMAN was faster than the AIDP group. Except for sensory nerve involvement, there was no significant difference in the clinical feature and severity. The mean time of the muscle strength began to recover was (5.59±3.63) days in the AIDP group and (7.21±4.68) days in the AMAN group after IVIG treatment. The time of the AIDP group was shorter than the AMAN group, but the difference was not statistically significant (t=-1.5702, P>0.05). The mean time of the muscle strength increased one grade was (8.88±4.39) days in the AIDP group and (12.67±8.35) days in the AMAN group. The difference was statistically significant (t=-2.3689, P<0.05). No patients in this group died. Follow-up data showed that the complete recovery time was not significantly different (t=0.2041, P>0.05). The clinical progress of the AMAN was faster than the AIDP group. Besides sensory nerve involvement, there was no significant difference in the clinical feature and severity. The AIDP group's clinical recovery was faster than AMAN's after the immunoglobulin treatment. The two groups were not significantly different in long-term prognosis.

Introduction. Guillain Barre Syndrome (GBS) is a post infectious polyneuropathy involving mainly motor but sometimes sensory and autonomic nerves. It is an acquired disease of the peripheral nerves that is characterized by rapidly progressing paralysis, areflexia and albumino-cytological dissociation in CSF. Methodology: Prospective, descriptive, observational, hospital based study was carried out to find out the clinico-epidemiological features of GBS including existing treatment modalities and its outcome. All cases fulfilled the criteria for AFP (Acute flaccid Paralysis) surveillance was included. Cases were reviewed for full medical history and examinations. To confirm the diagnosis, necessary investigations were carried out and combined with clinical symptoms. Results: Thirty patients were included in the study during study period. Among them 90% were diagnosed as GBS, 7.4% patients of GBS were associated with hypokalemic paralysis, 7.4% diagnosed as transverse myelitis and 3.7% diagnosed as idiopathic neuropathy. Different types of GBS were classified as AIDP (Acute inflammatory demyelinating polyneuropathy) 62.96%, AMAN (Acute motor axonal neuropathy) - 25.52%, AMASAN (Acute motor and sensory axonal neuropathy) - 3.3% and MFS (Miller fisher's syndrome) - 6.6% according to NCV result. Male female ratio is 1.7:1.0. There was 14.8% patients had relapse within 5 year. Associated diseases were URTI, pneumonia, sore throat and diarrhea. Facial Nerve palsy was commonest cranial nerve involvement.Sixty percentage of patients presented with sensory symptoms. There was transient bowel and bladder involvement in 20% of the cases. 69.2% patients became bed ridden at the nadir. There was albumin-cytological dissociation in 80% case. Majority of patients improved with supportive treatment alone, 19.5% patient required ventilator support among them 40% died. 7.4% of cases expired during treatment. Half of the patients fully recovered within 3 months. Conclusion: GBS is the commonest cause of AFP, AIDP being commonest subtype in our setting. We have to improve our existing treatment facilities and extend to different centers to detect and treat GBS. Most of the patients improve with supportive treatment alone. Ventilator support indicates grave prognosis. Key words: GBS (Gullein Barre Syndrome); AFP (Acute flaccid Paralysis); AIDP (Acute inflammatory demyelinating polyneuropathy; AMAN (Acute motor axonal neuropathy); AMASAN (Acute motor and sensory axonal neuropathy); MFS (Miller fisher's syndrome). DOI: 10.3126/jnps.v31i2.4065 J Nep Paedtr Soc 2010;31(2):93-97

Guillain-Barré syndrome (GBS), characterized by acute progressive limb weakness and areflexia, is the prototype of postinfectious autoimmune diseases. Campylobacter jejuni is the most frequently identified agent of infection in GBS patients, often preceding acute motor axonal neuropathy (AMAN), a variant of GBS. Anti-GM1, anti-GM1b, anti-GD1a, and anti-GalNAc-GD1a IgG antibodies are associated with AMAN. Carbohydrate mimicry [Galbeta1-3GalNAcbeta1-4(NeuAcalpha2-3)Galbeta1-] was seen between the lipo-oligosaccharide of C. jejuni isolated from an AMAN patient and human GM1 ganglioside. Sensitization with the lipo-oligosaccharide of C. jejuni induces AMAN in rabbits as does sensitization with GM1 ganglioside. Paralyzed rabbits have pathological changes in their peripheral nerves identical to changes seen in human GBS. C. jejuni infection may induce anti-ganglioside antibodies by molecular mimicry, eliciting AMAN. This is the first verification of the causative mechanism of molecular mimicry in an autoimmune disease. To express ganglioside mimics, C. jejuni requires specific gene combinations that function in sialic acid biosynthesis or transfer. The knockout mutants of these landmark genes of GBS show reduced reactivity with GBS patients' sera, and fail to induce an anti-ganglioside antibody response in mice. These genes are crucial for the induction of neuropathogenic cross-reactive antibodies. An approach for evaluating intravenous immune globulin, a treatment for GBS, based on our animal model of AMAN is also discussed in this review, and recent advances made in this field are described.

While monocyte to high-density lipoprotein cholesterol ratio (MHR) has been reported to be associated with nervous system lesions, the role of MHR has not been determined in patients with Guillain-Barré Syndrome (GBS). The purpose of our study was to explore the role of MHR in patients with GBS. A total of 52 GBS patients were involved in the study retrospectively, including patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN). We used Hughes Functional Grading Scale (HFGS) score to evaluate functional status in GBS patients. Among patients with different subtypes of GBS, MHR was significantly elevated in those with demyelination compared to patients without demyelination (p < 0.001); AIDP patients had an increased MHR compared with AMAN or AMSAN patients (p = 0.001; p = 0.013). There was a positive correlation between MHR and HFGS score (r = 0.463, p = 0.006) in AIDP patients, but not in AMAN or AMSAN. Multiple linear regression analysis revealed that MHR was independently associated with HFGS score (beta = 0.405, p = 0.013) in AIDP patients. Our study suggests that MHR as an inflammatory marker is elevated in patients with AIDP compared to AMAN or AMSAN patients, while MHR has a positive correlation with clinical severity in AIDP patients, suggesting that MHR may provide an additional information to reflect the pathophysiology of AIDP.

Infectious origins of, and molecular mimicry in, Guillain-Barré and Fisher syndromes

Introduction: Guillain-Barre syndrome (GBS) is an acute autoimmune disorder of peripheral nerves and their roots. The most common GBS variants are: acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor and sensory axonal neuropathy (AMSAN), Miller-Fisher syndrome (MFS) and other rarer variants. Aim: Evaluation of frequency of GBS variants and analysis of the outcome of the disease in a cohort of patients hospitalized at the Neurology Clinic, Clinical Center of Serbia. Material and Methods: This study included 43 patients with GBS, hospitalized in 2015. The data about clinical characteristics of the disease were collected by a retrospective analysis from electronic medical record. We used methods of descriptive statistics: mean, standard deviation and proportions. Results: Majority of our patients were male with male to female ratio 2.6 : 1. The most common variant in our study was AIDP (41.9%), then AMSAN (7.0%) and AMAN (4.7%). The most common first symptoms of the disease were weakness and numbness in the legs (18.6%). According to the Hughes scale, at admission, most of the patients had a mild form of the disease (65.1%), while at nadir 62.8% were non ambulatory, and 2.3% of patients required assisted ventilation. The outcome of the disease was favorable in 74.4% of patients, while 11 patients (25.6%) had a significant functional disability on discharge. In (4.7%) 2 patients of our cohort, lethal outcome was recorded. Conclusion: GBS is a rapidly progressive, monophasic disease, which has a generally good prognosis today, thanks to modern therapy. Our further research will be focused on the long-term outcome of GBS.

BackgroundIn Guillain–Barré syndrome (GBS), the diversity in electrophysiological subtypes is unexplained but may be determined by geographical factors and preceding infections. Acute motor axonal neuropathy (AMAN) is a frequent GBS...

Molecular mimicry between host tissue structures and microbial components has been proposed as the pathogenic mechanism for triggering of autoimmune diseases by preceding infection. Recent studies stated that molecular mimicry as the causative mechanism remains unproven for most of the human diseases. Still, in the case of the peripheral neuropathy Guillain-Barré syndrome (GBS) this hypothesis is supported by abundant experimental evidence. GBS is the most frequent cause of acute neuromuscular paralysis and in some cases occurs after infection with Campylobacter jejuni (C. jejuni). Epidemiological studies, showed that more than one third of GBS patients had antecedent C. jejuni infection and that only specific C. jejuni serotypes are associated with development of GBS. The molecular mimicry between the human gangliosides and the core oligosaccharides of bacterial lipopolysaccharides (LPSs) presumably results in production of antiganglioside cross-reactive antibodies which are likely to be a contributory factor in the induction and pathogenesis of GBS. Antiganglioside antibodies were found in the sera from patients with GBS and by sensitization of rabbits with gangliosides and C. jejuni LPSs animal disease models of GBS were established. GBS as prototype of post-infection immune-mediated disease probably will provide the first verification that an autoimmune disease can be triggered by molecular mimicry.

Background: Guillain-Barre syndrome (GBS) is the leading cause of acute flaccid paralysis in children. This study was aimed to compare the clinical spectrum and shortterm outcome of children with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN) subtypes of GBS in children. Methods: The study was a prospective cohort study done in a tertiary neurology hospital for 3 years. Children under 18 years of age fulfilling the Brighton diagnostic criteria for GBS were enrolled in the study. Based on the nerve conduction study, patients were subclassified as AIDP, AMAN, AMSAN, and others. Finally, a comparison was done in children with AIDP and AMAN subtypes. Results: A total of 102 children have fulfilled the Brighton diagnostic criteria of GBS during that study period. Among them, 83 children were included in the final analysis as NCS findings suggestive of AIDP and AMAN were found in 29(28.43%) and 54(52.94%) of cases respectively. No patient died in this cohort and follow-up was done at 3 months after discharge. A comparison of clinical data between the two groups revealed similar clinical features in most of the cases. The mean age difference between the two groups was statistically significant and AIDP was found to be more frequent in the 1-5 years age group. There was a significant association between gastroenteritis and AMAN subtypes. On symptom analysis, pain and tingling sensation were found predominantly in AMAN subtypes. Children having AMAN variants developed respiratory distress more than AIDP. Assisted ventilation were needed in 14.45% of cases and the majority of them were from the AMAN group. The mean duration of hospital stay and the mean disability scores at three months after discharge were significantly higher in the AMAN group. Conclusions: AMAN was the commonest GBS subtypes in children. AIDP was more frequent in the younger age group. Children with AMAN appeared to have higher short-term morbidity and slower recovery than those with AIDP. Bang. J Neurosurgery 2022; 11(2): 94-100

Wakerley and Yuki1 report the wide heterogeneity of the clinical spectrum of Guillain–Barre syndrome (GBS), highlighting atypical presentations and reviewing the differential diagnoses. The approach is practical and their intent...

S87 Issues in the electrodiagnosis of Guillain-Barré syndrome subtypes

Co-infection with two different Campylobacter jejuni strains in a patient with the Guillain–Barré syndrome

Background: Both Guillain–Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are neurodegenerative and inflammatory demyelination disorders. Sporadic reports showed that the increased levels of thyroid function and autoantibodies are associated with GBS, CIDP, or both, but no systematic study has been reported. We assessed the differences of thyroid function and autoantibodies between GBS and CIDP in a Chinese cohort.Methods: A total of 256 patients were enrolled in this study. 175 clinically confirmed GBS and CIDP patients were selected. Meanwhile, 81 patients hospitalized for diseases other than GBS or CIDP with mild symptoms were enrolled as a control group. Relevant clinical data, including thyroid function, and autoantibody examinations, were collected for statistical analysis.Results: In the comparison of thyroid function and autoantibody parameters, the levels of total thyroxine (TT4), thyroid peroxidase antibody (TPO-Ab), and thyroglobulin antibody (TG-Ab) in the GBS group were all higher than those in the CIDP and Control groups (P < 0.01). The thyroid antibody positive rates in the GBS and CIDP groups were 70.10 and 14.10%, respectively (P < 0.01). In the receiver operating characteristic (ROC) curve analysis, TT4, TPO-Ab, and TG-Ab were higher in the GBS group and lower in the CIDP group (P < 0.01). To achieve a high specificity of 97–99%, the diagnostic cutoff value of TPO-Ab was higher than 133 IU/mL (Sensitivity: 11.34%) or lower than 0.01 IU/mL (Sensitivity: 9.09%), while the diagnostic cutoff value of TG-Ab was higher than 261.1 IU/mL (Sensitivity: 2.06%) or lower than 0.46 IU/mL (Sensitivity: 11.69%). Multivariate logistic regression analysis showed that the differences in TPO-Ab were statistically significant between GBS patients with TPO-Ab was higher than 133 IU/mL and CIDP patients (P < 0.01); the differences in TG-Ab were statistically significant between GBS patients with TG-Ab was higher than 261.1 IU/mL and CIDP patients (P < 0.05).Conclusion: The elevation of thyroid autoantibodies was associated with GBS. TPO-Ab higher than 133 IU/mL or lower than 0.01 IU/mL and TG-Ab higher than 261.1 IU/mL or lower than 0.46 IU/mL had high specificity for differentiating between GBS and CIDP; therefore, TPO-Ab and TG-Ab can be used as biomarkers for the differential diagnosis of GBS and CIDP.

Inflammatory neuropathies are uncommon but important to diagnose because they are treatable. This review summarises the clinical approach to diagnosis and treatment of Guillain-Barre syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy...