Axonal Guillain-Barré Syndrome Research Articles

Interleukin-10 promoter polymorphisms and haplotypes in patients with Guillain-Barré syndrome.

Interleukin-10 (IL-10) is a multifunctional cytokine that exerts both pro- and anti-inflammatory effects on the immune system as well as in the pathogenesis of Guillain-Barré syndrome (GBS). We investigated whether the three common polymorphisms -1082 G/A(rs1800896), -819 C/T(rs1800871), and -592 C/A(rs1800872) in the promoter region of IL-10 have any influence on the susceptibility, severity, and clinical outcome of GBS. IL-10 promoter polymorphisms were investigated in 152 patients with GBS and 152 healthy controls from Bangladesh using polymerase chain reaction and restriction fragment length polymorphism (PCR-RFLP), and allele-specific oligonucleotide-PCR (ASO-PCR). Haplotype patterns and frequencies were analyzed using Heatmaply R-package, chi-square, and Fisher's exact test. The serum level of IL-10 was measured through enzyme-linked immunosorbent assays. p-values < 0.05 were considered statistically significant. IL-10 promoter polymorphisms -1082 G/A, -819 C/T, and -592 C/A were not associated with GBS susceptibility. The homozygous -819 TT genotype showed a tendency with susceptibility (p = 0.029; pc = 0.08) and was prevalent in axonal variants of GBS compared to demyelinating subtypes and controls (p = 0.042, OR = 8.67, 95% CI = 1.03-72.97; pc = 0.123 and p = 0.005, OR = 4.2, 95% CI = 1.55-11.40; pc = 0.015, respectively). Haplotype analysis revealed 19 patterns of genotypes and high IL-10 expression haplotype combinations (GCC/GTA, GCC/ATA, and GCC/GCA) may have influence on disease severity (p = 0.026; pc = 0.078). Serum expression of IL-10 was elevated in GBS patients ([GBS, 12.16 ± 45.71] vs. [HC, 0.65 ± 5.17] pg/mL; p = 0.0027) and varied with disease severity ([severe-GBS, 15.25 ± 51.72] vs. [mild-GBS, 3.59 ± 19.79] pg/mL, p = 0.046). The -819 TT genotypes influence axonal GBS, and high frequency of IL-10 expression haplotype combination with elevated serum IL-10 may play an important role in disease severity.

Predictors of long-term health-related quality of life in Guillain-Barré syndrome: A hospital-based study

ObjectiveThis study aimed to determine the impact of patients’ baseline clinical, neurophysiological data, and management plan of Guillain-Barré syndrome (GBS) on long-term quality of life (QoL) and to identify its potential predictors. MethodsSeventy-nine GBS patients were recruited. On admission, participants were evaluated using the Medical Research Council (MRC) sumscore, GBS disability scale (GDS), and Erasmus GBS Respiratory Insufficiency Score (EGRIS). Neurophysiological data were collected, and a management plan was devised. MRC sumscore was repeated at nadir. MRC, GDS and Short Form Survey (SF-36) were assessed at first-year follow-up. ResultsThe mean age was 37.84 ± 17.26 years, with 43 male patients (54.4%). QoL at one year correlated significantly with baseline clinical variables (age, number of days between weakness and admission, MRC sumscore at onset and nadir, high GDS, and EGRIS scores). Antecedent events, especially diarrhoea, neck muscle weakness, autonomic dysfunction, cranial nerve involvement, and mechanical ventilation (MV), associated with worse QoL. Axonal GBS patients had lower QoL than AIDP patients, and PE patients exhibited lower QoL than IVIG patients. Multiple regression analysis showed that older age, diarrhoea, number of days between weakness and admission, neck muscle weakness, cranial nerve involvement, autonomic dysfunction, early MV, and MRC at onset and nadir and high GDS could predict poor QoL. ConclusionOlder age, more days between weakness and admission, neck muscle weakness, cranial nerve involvement, autonomic dysfunction, early MV, diarrhoea, low MRC at onset and nadir, high GDS at onset, axonal type, and PE treatment were potential predictors of poor QoL in GBS.

Early electrophysiological study variants and their relationship with clinical presentation and outcomes of patients with Guillain-Barré syndrome

This study compared the clinical outcomes of the two main neurophysiological types of Guillain-Barré Syndrome (GBS). Sixty-two GBS patients were examined clinically at onset using Medical Research Council (MRC), Hughes disability scales (HDS), and nerve conduction studies were evaluated in four limbs. The Modified Erasmus GBS outcome score (MEGOS) was assessed 2 weeks after onset. Outcomes were measured after 3 months using MRC and HDS scores. According to electrophysiological data two main groups identified acute inflammatory demyelinating polyneuropathy (AIDP = 31 cases) or acute axonal GBS including inexcitable forms (26 cases). The number of days between onset of weakness and admission was significantly shorter, and gastrointestinal symptoms were significantly higher among the axonal type than AIDP. MRC sum scores at onset and at nadir were significantly worse in the axonal type than in AIDP. Neck muscle weakness, impaired cough reflex, the need for mechanical ventilation, hypoalbuminemia, and hypernatremia were more common in the axonal type. At outcome, 74% of the AIDP were healthy/minor symptoms versus 38.46% of the axonal type. There was a high prevalence of the axonal variant (41.9%) compared with European and North American populations. The axonal type had a significantly worse outcome than AIDP type.

Clinical and demographic characteristics of guillain-barré syndrome (GBS) variants in a tertiary care hospital.

Objective: To evaluate clinical and demographic characteristics of guillian-barre syndrome. Material &amp; Methods: Study Design: Descriptive, Cross-sectional study. Setting: Department of Neurology, Lady Reading Hospital, Peshawar. Period: December 2020 to March 2022. Results: The study identified 117 patients with GBS, and the mean age was 43.59 ± 17.39 years. There were 78 male (67%) and 39 female (33%) patients. The most common GBS subtype was AMAN (46.2%), followed by AMSAN (34.2%), AIDP (16.2%), and atypical (2.6%). The study found a significant difference in gender distribution between the two subtypes, with male patients being more likely to have axonal GBS. Regarding antecedent events, the study found that 61% of patients had a preceding illness, with gastrointestinal infection being the most common (50%). The clinical features of GBS varied by subtype, with patients with AMAN being more likely to have walking difficulty, symmetric ascending weakness, and numbness/tingling, while patients with AIDP were more likely to have a fever and respiratory distress. Conclusion: The study's findings are broadly consistent with previous research, highlighting the heterogeneity of GBS in terms of its presentation and antecedent events. However, other differentiating points that need to be taken into consideration have also been mentioned. Furthermore, work needs to be done to raise awareness among medical professionals in Nothern Pakistan regarding the salient features of the problem.

Comparison of ulnar, median, and sural sensory nerve conduction studies between demyelinating and axonal forms of Guillain-Barré Syndrome

Background: Guillain-Barré syndrome (GBS) is an important neurological disease that can cause disability. Axonal GBS and acute inflammatory demyelinating polyradiculoneuropathy (AIDP) are two important subgroups.&#x0D; Methods: Patients with clinical and electrodiagnostic features compatible with GBS were included in this retrospective study. The patients were divided into two groups neurophysiologically as Axonal GBS and AIDP. Medical research council (MRC) scores of the patients' muscles, median / ulnar / posterior tibial / peroneal / sural nerve conduction study findings were included in the analyses. Sural sparing pattern was considered as the absence of median/ulnar compound nerve action potential (CNAP) and present sural nerve CNAP (SS-M/SS-U).&#x0D; Results: Twelve AIDP and 10 Axonal GBS patients were included in the study. MRC scores were not different between the two groups (p=0.895). CNAPs of the right median and ulnar nerves were smaller in AIDP patients than in Axonal GBS patients (p

A Case of Acute Motor Sensory Axonal Neuropathy, a Rare Form of Guillain-Barre Syndrome, Developing After Multiple COVID-19 Infections (P13-8.009)

<h3>Objective:</h3> Guillain-Barre syndrome (GBS), an autoimmune polyneuropathy characterized by ascending weakness and characteristic cerebrospinal fluid (CSF) findings, has been identified in patients previously diagnosed with COVID-19, treated for COVID-19 infection, or vaccinated against the same. GBS is divided into axonal and demyelinating subtypes, and confirmatory testing such as electromyography and nerve conduction studies can help differentiate between subtypes. Here we present a case with recurrent COVID-19 infections, most recently treated with bebtelovimab, who developed acute motor sensory axonal neuropathy (AMSAN), a rare variant of axonal GBS. <h3>Background:</h3> A 33-year-old female presented with progressive ascending weakness and sensory loss that started two weeks after COVID-19 infection. She was fully vaccinated, however, had four episodes of COVID-19 infection within the past year and a half. Two weeks after the most recent infection, which was treated with bebtelovimab, she developed rapidly progressive weakness and sensory impairment in bilateral lower extremities quickly involving upper extremities. She received two rounds of intravenous immunoglobulin (IVIG) three and five weeks after symptoms onset. Her symptoms continued to progress requiring hospitalization. Exam showed severe weakness in all limbs with distal muscle atrophy in upper extremities. Imaging was negative. CSF demostrated albuminocytological dissociation. Nerve conduction studies concluded severe motor and sensory axonal neuropathy with active denervation which led to the suspicion of Acute Motor and Sensory Axonal Neuropathy (AMSAN), a rare variant of GBS. She underwent plasmapheresis with only minimal improvement. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> N/A <h3>Conclusions:</h3> Many cases of GBS have been reported after COVID-19 infection but only a few of them are about AMSAN. This case presents unique features including multiple COVID-19 infections despite prior vaccination, and the development of this rare variant of GBS. It raises the concern of multiple COVID-19 infections having a cumulative impact on peripheral nervous system leading to more severe forms of GBS with poor response to treatment. <b>Disclosure:</b> Dr. Narvaez Caicedo has nothing to disclose. Mr. Rao has nothing to disclose. Dr. Patel has nothing to disclose. Dr. Wu has nothing to disclose.

Frequency, Age, Gender Distribution, and Seasonal Variation of Guillain-Barré Syndrome in a Province of Pakistan: A Retrospective Study

Characterized by the sudden onset of muscle weakness, Guillain-Barré Syndrome (GBS) is a rare autoimmune disorder which can progress to paralysis. GBS has different subtypes based on the clinical and electrophysiological characteristics, including acute inflammatory demyelinating polyradiculoneuropathy (AIDP), axonal GBS (AMAN and AMSAN), and Miller Fisher Syndrome (MFS). Objective: To study frequency of the disease in Khyber Pakhtunkhwa. Methods: In this retrospective study, medical records of 39 patients diagnosed with GBS at Lady Reading Hospital Peshawar, Pakistan, were analyzed to determine the prevalence of GBS subtypes in the country. Results: The results showed that the most prevalent subtype of GBS was AMAN, accounting for 59% of cases, followed by AMSAN at 25.6%, and AIDP at 15.3%. The axonal variety made up 84.6% of the total GBS cases in this study. On average, the patients with AMSAN were 39.2 years old, while patients with AMAN and AIDP were relatively younger, with mean ages of 30 and 28 years, respectively. There was a male predominance in all subtypes except for AIDP, which showed equal distribution. Conclusions: These findings provide valuable information on the distribution of GBS subtypes in Peshawar, Pakistan, which may have implications for the diagnosis and management of GBS in the country. Additionally, the study's results can contribute to the global understanding of GBS epidemiology and may help improve the diagnosis and treatment of GBS patients worldwide.

Dose-Effect Relationship of Motor Nerve Inexcitability on Outcome in Guillain-Barré Syndrome: A Prospective Cohort Study.

One or more inexcitable motor (IM) nerves are common during electrodiagnostic (EDx) study in Guillain-Barré syndrome (GBS). This study assessed the dose-effect relationship of IM nerves on outcome in patients with acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor and/or sensory axonal neuropathy (AMAN and AMSAN). Eighty-eight GBS patients admitted during May 2018-June 2023 underwent detailed clinical evaluation and EDx study. Admission and follow-up disability were assessed on a 0-10 Clinical Grading Scale (CGS). Outcome was recovery at 6 months, defined as good (CGS <3) and poor (CGS ≥3). Binary multivariate logistic regression with backward elimination was used to calculate independent predictors of outcome. Proportion of patients with complete recovery decreased significantly with increasing numbers of IM nerves (P < 0.01). Seventy-six patients were followed for 6 months. Among patients with IM nerves (n = 28), complete recovery was similar between AIDP and axonal GBS (70% vs. 50%, respectively; P = 0.40). However, in patients with recordable compound muscle action potentials (CMAPs) in all the motor nerves (n = 26), axonal GBS had significantly poor recovery compared to AIDP (75% vs. 9.1%; P = 0.01). Among patients receiving intravenous immunoglobulin (IVIg; n = 42), poor recovery was seen in 53.6% with IM nerves compared to 35.7% without (P = 0.28), while it was 37.5% versus 5.6% (P = 0.04), respectively, in those who did not receive IVIg (n = 34). However, only admission disability (odds ratio [OR] 0.88, 95% confidence interval [CI] 0.81-0.97; P = 0.007) was found to be an independent predictor of outcome. Although increasing numbers of IM nerves were associated with poor outcome on univariate analysis, they did not predict 6 months' outcome independently. Outcome did not differ between axonal GBS and AIDP among those with IM nerves. IVIg improved outcome in patients with IM nerves.

Study of total duration of distal compound muscle action potential in demyelinating and axonal Guillain-Barre’ syndrome

ABSTRACT Objectives Electrophysiology plays a crucial role in Guillain-Barré syndrome (GBS) diagnosis and subtype classification. The aim of our study was to assess the potential role of distal compound muscle action potential (dCMAP) for early differentiation between acute inflammatory demyelinating polyneuropathy (AIDP) and axonal GBS. Methods We retrospectively reviewed the medical records of 24 subjects with AIDP and 18 subjects with axonal GBS. We built up receiver operating characteristic curves for total dCMAP duration and negative phase of dCMAP duration, in order to derive cut-off values able to differentiate between AIDP and axonal GBS. Results The total duration of dCMAP was significantly prolonged in AIDP compared to axonal GBS. AUCs, odds ratio and positive predictive values were higher for total duration than for negative peak duration. Nerve conduction parameters in the lower limbs were more sensitive than those in the upper limbs in distinguishing AIDP from axonal GBS. Discussion Total duration of dCMAP dispersion may capture an adjunctive component of distal demyelination, not measured by the more traditional parameters and may thus represent a useful tool for early differentiation between AIDP and axonal GBS.

Guillain-Barré syndrome in children – High occurrence of Miller Fisher syndrome in East Asian region

BackgroundGuillain-Barré syndrome (GBS) is a rare acquired immune-mediated polyneuropathy. Updated population-based data concerning paediatric GBS is needed. MethodsPaediatric patients aged below 18 years diagnosed with GBS between 2009 and 2018 in all 11 paediatric departments in Hong Kong were identified from the Hong Kong Hospital Authority Clinical Data Analysis and Reporting System. The collected data from medical health records were reviewed by paediatric neurologist from each department. Estimated incidence of paediatric GBS was calculated. We also compared our findings with other paediatric GBS studies in Asia. Results63 subjects of paediatric GBS were identified, giving an estimated annual incidence of 0.62 per 100,000 population. Half of the subjects had acute inflammatory demyelinating polyneuropathy (AIDP) (n = 31; 49.2%), one quarter had Miller Fisher Syndrome (MFS) (n = 16; 25.4%), one-fifth had axonal types of GBS (n = 12; 19.0%), and four were unclassified. Paediatric subjects with axonal subtypes of GBS compared to the other 2 subtypes, had significantly higher intensive care unit (ICU) admission rates (p = 0.001) and longest length of stay (p = 0.009). With immunomodulating therapy, complete recovery was highest in those with MFS (100%), followed by AIDP (87.1%) and axonal GBS (75%). Our study also confirms a higher MFS rate for paediatric GBS in East Asia region and our study has the highest MFS rate (25.4%). ConclusionOur population-based 10-year paediatric GBS study provides updated evidence on estimated incidence, healthcare burden and motor outcome of each subtype of paediatric GBS and confirmed a higher occurrence of paediatric MFS in East Asia.

Eczematous skin reaction after intravenous immunoglobulin therapy in axonal Guillain-Barré syndrome

Background: Intravenous immunoglobulin (IVIG) treatment in Guillain-Barré syndrome (GBS) is relatively easy to administer and safe due to infrequent, non-severe side effects. Adverse effects can include skin reactions, the causes of which have not been fully elucidated. In this study, we investigated skin reactions following IVIG treatment for acute inflammatory neuropathy. We examined the relationship between the clinical results in the neuropathy and the clinical profile of skin reactions. Methods: We retrospectively analyzed patients with skin reactions that developed after IVIG treatment in acute immune-mediated or inflammatory neuropathy [GBS or Miller Fisher syndrome (MFS)] based on the dermatologic evaluation. Results: Ninety-six patients with acute inflammatory neuropathy were treated with IVIG during the study period. Thirteen cases (13.5%), with age at onset ranging from 22–69 years (mean, 50.3 years), developed skin reactions after IVIG treatment. The features accompanying skin reactions were hand eczema, including pompholyx (9/13, 82%), generalized eczematous eruption (2/13, 15.4%), and contact dermatitis, including folliculitis. The electrophysiological classification of 11 patients with classical GBS showed that nine patients (81.8 %) had axonal neuropathy, and one (9.1 %) had acute inflammatory demyelinating polyradiculoneuropathy. Conclusions: Eczematous skin reactions or pompholyx are the most common skin reactions following IVIG treatment in axonal GBS. These reactions were relatively minor and recovered well. Although adverse skin reactions to IVIG were usually benign and the precise mechanism of this cutaneous eruption remains unknown, its occurrence within days of IVIG treatment can be managed effectively if recognized by neurologists.

P.101 A-waves on electrodiagnostic studies in axonal and demyelinating cases of Guillain-Barré Syndrome (GBS)

Background: A-waves are lesser-known late responses of debated clinical significance, seen in routine motor nerve conduction studies (NCS). They are proposed to be a sensitive marker of demyelination and an early finding in acute demyelinating polyradiculoneuropathy (AIDP). We hypothesized that the presence and distribution of A-waves are discriminative markers in differentiating AIDP from axonal variants of Guillain-Barré Syndrome (GBS). Methods: We identified patients diagnosed with demyelinating and axonal forms of GBS at the Montreal Neurological Institute between 2016 and 2021. Clinical and electrophysiological data including raw NCS responses were retrospectively reviewed for 28 AIDP and 9 axonal GBS cases. Results: 20 of 28 AIDP cases had at least one A-wave in non-tibial nerves compared to 2 of 9 axonal cases. Among patients with NCS available within 2 weeks of symptom onset, 13 of 14 AIDP cases had non-tibial A-waves, compared to 0 of 6 axonal cases. Eight of 14 AIDP cases had one or more nerves with multiple A-waves within 2 weeks, compared to 0 of 6 axonal cases. Conclusions: In patients with GBS, the presence of A-waves in non-tibial nerves and of nerves with multiple A-waves are early indicators of the demyelinating variant. Early identification of GBS subtype is valuable for prognostication.

Guillain-Barré Syndrome Following the ChAdOx1-S/nCoV-19 Vaccine: A Case Report

Serious neurological complications following coronavirus disease 2019 (COVID-19) vaccination, such as cerebral venous sinus thrombosis or acute disseminated encephalomyelitis, have rarely been reported. Here, we report a case of Guillain-Barré syndrome (GBS) that occurred within 2 weeks of receiving the AstraZeneca ChAdOx1-S/nCoV-19 vaccine. A 61-year-old man presented with weakness and hypesthesia of the legs 9 days after the first dose of the ChAdOx1-S vaccine. The patient progressed to respiratory failure and severe quadriparesis. Nerve conduction studies showed markedly reduced amplitudes of compound muscle action potentials and sensory nerve action potentials in both upper and lower extremities, without definitive evidence of demyelination. The patient was diagnosed with axonal GBS and received intravenous immunoglobulin treatment, with a poor response. This is a rare case of GBS after COVID-19 vaccination in Korea. Physicians should be aware of this rare but serious complication of the COVID-19 vaccine to effectively manage such a situation in clinical practice.

Predictors of Mechanical Ventilation in Guillain-Barré Syndrome with Axonal Subtypes.

The early clinical predictors of respiratory failure in Latin Americans with Guillain-Barré syndrome (GBS) have scarcely been studied. This is of particular importance since Latin America has a high frequency of axonal GBS variants that may imply a worse prognosis. We studied 86 Mexican patients with GBS admitted to the Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, a referral center of Mexico City, to describe predictors of invasive mechanical ventilation (IMV). The median age was 40 years (interquartile range: 26-53.5), with 60.5% men (male-to-female ratio: 1.53). Most patients (65%) had an infectious antecedent (40.6% gastrointestinal). At admission, 38% of patients had a Medical Research Council (MRC) sum score <30. Axonal subtypes predominated (60.5%), with acute motor axonal neuropathy being the most prevalent (34.9%), followed by acute inflammatory demyelinating polyneuropathy (32.6%), acute motor sensory axonal neuropathy (AMSAN) (25.6%), and Fisher syndrome (7%). Notably, 15.1% had onset in upper limbs, 75.6% dysautonomia, and 73.3% pain. In all, 86% received either IVIg (9.3%) or plasma exchange (74.4%). IMV was required in 39.5% patients (72.7% in AMSAN). A multivariate model without including published prognostic scores yielded the time since onset to admission <15 days, axonal variants, MRC sum score <30, and bulbar weakness as independent predictors of IMV. The model including grading scales yielded lower limbs onset, Erasmus GBS respiratory insufficiency score (EGRIS) >4, and dysautonomia as predictors. These results suggest that EGRIS is a good prognosticator of IMV in GBS patients with a predominance of axonal electrophysiological subtypes, but other early clinical data should also be considered.

Guillain‐Barré syndrome (GBS) in a tertiary general hospital at Yangon, Myanmar

AbstractAimThe aim of the study was to study the profile of Guillain‐Barré syndrome (GBS) patients admitted at Yangon General Hospital (YGH), Myanmar.MethodsPatients with flaccid quadriparesis due to GBS admitted to the Department of Neurology, YGH in 2019 were prospectively studied.ResultsNinety‐four GBS patients were recruited. Mean age was 36.2 years. Male to female ratio was 2.4:1. Patients were admitted 12.1 ± 14.8 days after disease onset. 46.8% were from outside the Yangon region. Peak incidence was in rainy season (47.9%). 62.8% reported symptoms suggestive of preceding infection: 45.7% unspecified fever, 3.2% upper respiratory infection, 8.5% diarrhea, and 5.3% fever with rash. 27.7% had bulbar and 44.7% had facial weakness. 5.3% had GBS/Miller Fisher syndrome overlap. Electrophysiologically, 50% were acute inflammatory demyelinating polyneuropathy (AIDP), 30% acute motor axonal neuropathy (AMAN), and 20% acute motor‐sensory axonal neuropathy (AMSAN). Maximum disability was reached at 5.6 ± 4.78 days from onset. The plateau phase lasted 12.12 ± 14.27 days. 13.8% required ventilator support. 64.9% of patients received limited manual plasma exchange, 9.6% standard plasma exchange, 4.3% intravenous immunoglobulin and 21.3% no specific treatment. 79.8% had good clinical recovery at 4 weeks from inclusion. Mortality was 0%. Severity at admission was identified as the only poor recovery predictor.ConclusionGuillain‐Barré syndrome in a Yangon tertiary hospital peaked in rainy season and axonal GBS accounted for 50% cases. However, diarrhea was an uncommon antecedent. The good recovery was noted in 79.8% and largely predicted by severity of disease at admission.

Sural-sparing pattern: A study against electrodiagnostic subtypes of Guillain–Barre syndrome

ObjectiveTo study sural-sparing pattern in Guillain–Barre syndrome (GBS) and compare it among GBS’s electrodiagnostic subtypes, classified by two recent criteria. MethodsThis study retrospectively reviewed clinical data and electrodiagnostic studies (EDXs) of 88 GBS patients diagnosed in a tertiary care hospital (2010–2019). ResultsOverall, 79/88 (89.8%) and 36/45 (80%) patients had bilateral sensory nerve conduction studies (NCS) in the first EDX and follow-up EDX, respectively. Sural-sparing occurred in all subtypes (50% overall occurrence rate), most commonly in demyelination. There was no statistically significant difference in sural-sparing occurrence rates between demyelinating and axonal GBS; however, sural-sparing in axonal GBS tended to show a lower number of abnormal upper-limb sensory nerve action potentials (SNAPs) than demyelinating GBS. Shifting between sural-sparing and no sural-sparing occurred in approximately-one-fourth of patients receiving serial studies. Follow-up EDX additionally discovered 20% of all sural-sparing. Unilateral EDX could have omitted up to 30% of sural-sparing. ConclusionsSural-sparing is less obviously manifested in axonal than demyelinating GBS, with respect to the number of affected upper-limb SNAPs. Extended sensory NCS is worth in detecting sural-sparing as a supportive electrodiagnostic GBS feature. SignificanceThis report showed one different character of sural-sparing (number of affected upper-limb SNAPs) between demyelinating and axonal GBS.

Impact of Antecedent Infections on the Antibodies against Gangliosides and Ganglioside Complexes in Guillain-Barré Syndrome: A Correlative Study.

Background and Aims:Guillain-Barré Syndrome (GBS), an immune-mediated neuropathy, is characterized by antibodies against gangliosides/ganglioside complexes (GSCs) of peripheral nerves. Antecedent infections have been reported to induce antibodies that cross-react with the host gangliosides and thereby have a pivotal role in conferring an increased risk for developing GBS. Data pertaining to the impact of various antecedent infections, particularly those prevalent in tropical countries like India on the ganglioside/GSC antibodies is sparse. We aimed at exploring the association between six antecedent infections and the profile of ganglioside/GSC antibodies in GBS.Methods:Patients with GBS (n = 150) and healthy controls (n = 50) were examined for the serum profile of antibodies against GM1, GM2, GD1a, GD1b, GT1b, and GQ1b and their GSCs by ELISA. These antibodies were correlated with immunoreactivities against Campylobacter jejuni, Japanese encephalitis (JE), dengue, influenza, zika, and chikungunya infections.Results:The frequencies of antibodies against six single gangliosides (P < 0.001) and their GSCs (P = 0.039) were significantly higher in patients as compared to controls. Except for GT1b-antibody which was more frequent in axonal GBS, none of the other ganglioside/GSC antibodies correlated with the electrophysiological subtypes of GBS. Antecedent JE infection was significantly associated with increased frequency of antibodies against GD1a, GD1b, GT1b, and GQ1b. Antibodies against GSCs were not influenced by the antecedent infections.Interpretation:This study for the first time shows an association between antecedent JE infection and ganglioside antibodies in GBS. This finding reinforces the determining role of antecedent infections on ganglioside antibody responses and the subsequent immunological processes in GBS.

Clinical and serological prognostic factors in childhood Guillain-Barré syndrome: A prospective cohort study in Bangladesh.

Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in children. The objective of this study was to investigate the preceding infections, clinical, serological and electrophysiological characteristics and outcome of childhood GBS in Bangladesh. We included 174 patients with GBS aged <18 years from a prospective cohort in Bangladesh between 2010 and 2018. We performed multivariate logistic regression to determine the risk factors for poor outcome. Among 174 children with GBS, 74% (n = 129) were male. Around half of the patients (49%, n = 86) had severe muscle weakness, 65% (n = 113) were bedbound (GBS disability score 4) and 17% (n = 29) patients required mechanical ventilation at admission. Campylobacter jejuni serology and anti-GM1 IgG antibody were positive in 66% and 21% of the patients respectively. One hundred and forty-three (82%) patients did not receive standard treatment and half of them recovered fully or with minor deficits at 6-month. Twenty patients (11%) died throughout the study period. At 3-month of onset of weakness, complete recovery or recovery with minor deficit was significantly higher in demyelinating GBS patients compared to axonal GBS patients (86% vs 51%, P = .001). Cranial nerve palsy (OR = 4.00, 95%CI = 1.55-10.30, P = .004) and severe muscle weakness (OR = 0.16, 95%CI = 0.06-0.45, P = .001) were the important risk factors of poor outcome in children with GBS. Further large-scale studies are required for better understanding of factors associated with mortality and morbidity in childhood GBS.

Electrophysiology of Guillain-Barré syndrome in Bangladesh: A prospective study of 312 patients

ObjectiveTo describe the electrophysiological features in relation to clinical and serological findings of Guillain-Barré syndrome (GBS) in the national neuroscience hospital in Bangladesh. This is one of the few studies that investigated GBS patients using standardized electrophysiology in low-income countries. MethodsIn a prospective and observational study, we investigated 312 GBS patients by standardized clinical, serological and electrophysiological methods. Unilateral motor and sensory nerve conduction studies (NCS) were performed within two weeks of onset of weakness. Follow up NCS were performed in 189 patients and classified according to eight sets of established GBS criteria. Serology included assessment of anti-GM1 antibodies and anti-campylobacter jejuni lipo-oligosaccharide (LOS) antibodies. ResultsDepending on the criteria used, 44–59% patients had axonal GBS with anti-GM1 antibodies being present in 55–58% and 9–42% patients had demyelinating GBS with anti-GM1 antibodies being present in 7–35%. Conduction block (CB) with demyelinative slowing in the same nerve segment was found in 24% (74/312) patients, and CB without demyelinative slowing in the same nerve segment was found in 18% (56/312) patients, of whom anti-GM1 antibodies were found in 27% and 57% patients respectively. Follow-up NCS showed a change in GBS classification in 11–26% of patients, mainly from demyelinating to axonal GBS. ConclusionsThe predominant subtype of GBS in Bangladesh is axonal but demyelinating GBS also occurs with classification being strongly dependent on the applied criteria. SignificanceThe present study demonstrates the importance of reaching international agreement on GBS criteria that should be based on the best evidence.

Utility of Serial Nerve Conduction Studies in the Electrodiagnosis of Guillain-Barre Syndrome.

Guillain-Barre syndrome can be electrophysiologically classified into demyelinating and axonal subtypes and nerve conduction studies remain the mainstay in electrodiagnosis. Accurate electrodiagnosis has both therapeutic and prognostic significance and different criteria sets have been proposed for classification. To electrophysiologically classify GBS patients into AIDP and axonal subtypes according to various published criteria (Cornblath, 1990: Hadden, 1998, Rajabally, 2015), investigate if serial NCS changes the classification, and to identify additional parameters which may support the electrodiagnosis. In a retrospective study, we included all patients aged 15 to 80 years, admitted with a diagnosis of GBS between August 2015 and July 2017, who had at least two serial NCS. The various published criteria were applied to the two serial NCS and subtype classification along with diagnostic shifts on serial NCS were ascertained. At the first test, the established criteria gave a yield of 45.2% to 71% for AIDP, while 29% to 54.8% of patients were classified as axonal GBS. In the second study, there was a change in electrodiagnosis, ranging from 9.6% to 16.1%. The resolution of reversible conduction failure and misclassification of subtypes were the major reason for diagnostic shifts. Sural sparing pattern, facial nerve dysfunction, abnormal blink reflex, and phrenic nerve dysfunction were more common in AIDP. Serial nerve conduction studies allow an accurate electrodiagnosis of GBS subtypes, which has both therapeutic and prognostic implications. Also, the use of additional parameters such as blink reflex facial and phrenic nerve conduction may supplement routine NCS.