Axonal Variant Research Articles

Mechanical ventilation, hospitalization time, deaths and disability according to the variants of Guillain-Barré syndrome: systematic review and meta-analysis.

Motivation for the study. It is unclear which variant of Guillain-Barré syndrome is mostly associated with adverse outcomes. Main findings. Variants had longer time in MV (7.42 days longer), and hospitalization (3.11 days longer). By subgroups, we found that children with the axonal variant had a greater use of MV; whereas, the demyelinating variant was more frequent among adults in MV. Implications. Our results could be used for the implementation of new public health policies, allowing health personnel to have a better knowledge of the prognosis of each variant and the necessary resources to face future outbreaks. To determine the requirement and time to mechanical ventilation and Intensive Care Unit (ICU), hospitalization and hospitalization time, death and disability of the axonal variants of Guillain-Barré Syndrome (GBS) in comparison with the acute demyelinating variant in patients of all the ages. The systematic review that included patients with GBS. The exposure variable was the axonal variants and the comparator was acute inflammatory demyelinating polyneuropathy (AIDP). The outcomes were the requirement and time on mechanical ventilation (MV), requirement and time in the ICU, hospitalization time, disability and death. The NewCasttle-Ottawa Scale (NOS) was used to assess risk of bias. A meta-analysis was conducted to calculate mean differences and relative risks (RR) with their 95% confidence intervals (CI) using inverse variances and random effects models. Of the 3116 articles found, 46 met the selection criteria. The time on MV was 7.42 days (95% CI: 0.36 to 1.48) and the hospitalization time was 3.11 (95% CI: 0.73 to 5.49) days for the axonal variants. The axonal variants had a RR of 0.47 (95% CI: 0.24 to 0.92) for the requirement of MV in adults, but it was 1.68 (95% CI: 1.25 to 2.25) in children. There was a high statistical heterogeneity. Axonal variants showed, on average, longer MV and hospitalization time, overall and by subgroups. A high MV requirement was found for axonal variants in children; it was lower for adults.

Efficacy of Intravenous Immunoglobulins for the Axonal Variants of Guillain–Barré Syndrome: A Systematic Review and Meta-analysis

Efficacy of Intravenous Immunoglobulins for the Axonal Variants of Guillain–Barré Syndrome: A Systematic Review and Meta-analysis

A retrospective analysis of the clinical profile and factors associated with mortality and poor hospital outcomes in adult Guillain–Barre syndrome patients

Guillain–Barré syndrome (GBS) is an acute autoimmune polyneuropathy with substantial geographic variations in demography, antecedent events, clinical manifestations, electrophysiological sub-types, diagnostic findings, treatment modalities, and prognostic indicators. However, there is limited contemporary data on GBS patient profiles and prognostic factors from low-resource settings like Ethiopia. The objective of this study is to investigate the clinical profile, factors associated with mortality, and hospital outcomes among GBS patients admitted to Tikur Anbessa Specialized Hospital (TASH) in Addis Ababa, Ethiopia. A retrospective cross-sectional study was conducted among 60 GBS patients admitted to TASH from January 2018 to December 2022. Data on demographics, clinical features, treatments, complications, and outcomes were extracted from medical records. Bivariate and multivariate logistic regression analyses identified factors associated with mortality and poor hospital outcomes. The cohort had a mean age of 28.5 years, with 76.7% aged 14–34 years. Males comprised 61.7% of cases. Ascending paralysis (76.7%) was the predominant presentation. Absent or reduced reflexes were seen in 91.7% of patients. The most common antecedent event was gastroenteritis (26.7%), followed by upper respiratory tract infection (URTI) (15%) and vaccination (11.7%). The mean interval from symptom onset to hospital presentation was 8.77 days, and the peak symptom severity was 4.47 days. The axonal variant (75.5%) was the most common subtype, followed by the demyelinating variant (24.5%). Intravenous immunoglobulin was administered to 41.7% of patients. Respiratory failure requiring invasive mechanical ventilator (MV) support occurred in 26.7% of cases. The mortality rate was 10%, with mechanical ventilation being the only factor significantly associated with mortality (95% CI 2.067–184.858; P < 0.010). At discharge, 55% had a good outcome, and 45% had a poor outcome, according to the Hughes Functional Disability Scale (HFDS). Mechanical ventilation (AOR 0.024, 95% CI 0.001–0.607) and a GBS disability score > 3 (AOR 0.106, 95% CI 0.024–0.467) were factors significantly associated with poor hospital outcomes. GBS in this cohort primarily affected individuals of young age, commonly preceded by gastroenteritis and characterized by a high frequency of the axonal variant. Mechanical ventilation was found to be significantly linked to mortality. Alongside mechanical ventilation requirements, severe disability upon presentation emerged as a crucial determinant of poor outcomes upon discharge, underscoring the importance of early identification of high-risk patients and prompt interventions.

Guillain-Barré syndrome: a comprehensive review.

Guillain-Barré syndrome (GBS) is a potentially devastating yet treatable disorder. A classically postinfectious, immune-mediated, monophasic polyradiculoneuropathy, it is the leading global cause of acquired neuromuscular paralysis. In most cases, the immunopathological process driving nerve injury is ill-defined. Diagnosis of GBS relies on clinical features, supported by laboratory findings and electrophysiology. Although previously divided into primary demyelinating or axonal variants, this dichotomy is increasingly challenged, and is not endorsed by the recent European Academy of Neurology (EAN)/Peripheral Nerve Society (PNS) guidelines. Intravenous immunoglobulin and plasma exchange remain the primary modalities of treatment, regardless of the electrophysiological subtype. Most patients recover, but approximately one-third require mechanical ventilation, and 5% die. Disease activity and treatment response are currently monitored through interval neurological examination and outcome measures, and the potential role of fluid biomarkers is under ongoing scrutiny. Novel potential therapies for GBS are being explored but none have yet modified clinical practice. This review provides a comprehensive update on the pathological and clinical aspects of GBS for clinicians and scientists.

Correlation of Cerebrospinal Fluid Total Protein and Serum Neutrophil-to-Lymphocyte Ratio with Clinical Outcomes of Guillain-Barre Syndrome Variants.

To evaluate the correlation of cerebrospinal fluid total protein and serum neutrophil-to-lymphocyte ratio with the clinical outcomes and the various clinical and electrophysiological variants of Guillain-Barre syndrome. Cross-sectional study. Place and Duration of the Study: Department of Neurology, Mayo Hospital and King Edward Medical University, Lahore, Pakistan, from November 2022 to April 2023. Fourty-six Guillain-Barre syndrome patients, aged 12-70 years, were included in the study diagnosed by using the Brighton's criteria. Functional disability and respiratory insufficiency were assessed by using the modified Hughes disability score and the Erasmus Guillain-Barre syndrome respiratory insufficiency score, respectively.Serum neutrophil-to-lymphocyte ratio and cerebrospinal fluid total protein were calculated for each patient at the time of admission. Axonal variants had a higher mean neutrophil-to-lymphocyte ratio (5.29 ± 4.38) than demyelinating variants (4.71 ± 3.4) and Miller-Fischer syndrome (3 ± 2.828). This ratio was positively correlated with the modified Hughes's disability score (r = 0.790, p = 0.001) and the Erasmus Guillain-Barre syndrome respiratory insufficiency score (r = 0.936, p = 0.002). Mean cerebrospinal fluid total protein was higher for demyelinating (218 ± 136 mg/dl) than axonal variants (86 ± 56 mg/dl) and Miller-Fischer syndrome (34 ± 21 mg/dl). However, higher modified Hughes disability score (4-6) (r = 0.020, p = 0.117) and a high Erasmus Guillain-Barre syndrome respiratory insufficiency score (5-7) (r = 0.115, p = 0.302) did not significantly affect mean cerebrospinal fluid total proteins. Serum neutrophil-to-lymphocyte ratio can be regarded as a reliable biomarker to assess disease severity and clinical outcome in Guillain-Barre syndrome. Cerebrospinal fluid total protein is a poor predictor of the prognosis and severity of Guillain-Barre syndrome. Guillain-Barre syndrome (GBS), Clinical outcome, Cerebrospinal fluid total protein (CSF-TP), Neutrophil-to-lymphocytic ratio (NLR), Prognostic biomarker.

Evaluating yield and utilization of ganglioside antibody testing in clinical practice

Background and objectivesGanglioside antibodies can help diagnose distinct acute and chronic inflammatory neuropathies including axonal variants of Guillain-Barre syndrome, Miller-Fisher syndrome (MFS), multifocal motor neuropathy, and chronic sensory ataxic neuropathies. Because ganglioside antibody testing may be routinely ordered in patients with suspected inflammatory neuropathy, we sought to evaluate its yield and utilization in clinical practice. MethodsWe performed a retrospective chart review of all patients at London Health Sciences Centre who underwent ganglioside antibody testing between April 2019 and August 2023. The disease phenotype was determined for each patient, and the proportion of all tests that yielded a true-positive result was calculated. Ganglioside antibody positivity was classified as a true-positive result if the disease phenotype was robustly associated with the detected ganglioside antibody and there was no other more likely diagnosis. ResultsWe identified 92 patients who underwent ganglioside antibody testing. One patient (1%) was classified as having a true-positive result; this patient had GQ1b-IgG positivity with MFS. Among 92 patients tested, 20 patients (22%) had a disease phenotype that was considered to be robustly associated with ganglioside antibody positivity. ConclusionsThe yield of ganglioside antibody testing in clinical practice is low. We found that this testing is frequently ordered in patients with disease phenotypes that are not robustly associated with ganglioside antibody positivity, indicating that suboptimal test utilization is a primary contributor to its low yield. Restricting ganglioside antibody testing to patients with characteristic disease phenotypes would be valuable to improving yield and utilization of this testing.

Injudicious use of cranio-spinal imaging in Guillain-Barré syndrome in a low resource country.

Background: Many patients being investigated for Guillain-Barré syndrome (GBS) undergo unnecessary neuroimaging. The objective of this study was to determine the proportion of patients with GBS undergoing neuroimaging investigation, and to investigate any association with different GBS variants using the Brighton criteria. Methods: This cross-sectional observational study was conducted in the leading tertiary care hospital in Pakistan; 148 patients being investigated for, and subsequently diagnosed with GBS between January 2017 and March 2020 were enrolled. Participants were asked if they had undergone neuroimaging of the craniospinal axis before or during hospital admission, and the purpose of any computed tomography (CT) scan was investigated. We enquired whether fundoscopy had been performed before lumbar puncture (LP) and determined the level of certainty based on the Brighton criteria. Results: The majority of participants were men (n = 107, 73%), with a mean age of 42.85 ± 18.40 years. The mean waiting time to their first interaction with a neurologist was 5.20 ± 4.01 days, and the demyelinating variant of GBS was more common than the axonal variant (1.6:1). Most patients were diagnosed with level I certainty using the Brighton criteria (n = 113, 76%). Brain and spine magnetic resonance imaging (MRI) were performed ahead of admission in 48 (32%) and 59 (39%) patients, respectively. Brain CT scan was performed in 121 (82%) patients before LP, while 27 (18%) only underwent fundoscopic examination before LP. Conclusion: Clinical examination is fundamental in the diagnosis of GBS. Neuroimaging may be inappropriate and unnecessary, and may detract attention from crucial peripheral neuropathy measures while misusing limited resources.

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.

Systemic Lupus Erythematosus Initially Presenting as Acute Motor andSensory Axonal Neuropathy Variant ofGuillain-Barre Syndrome ina Healthy Active Duty Female.

Guillain-Barre syndrome (GBS) is an acute monophasic immune-mediated polyradiculoneuropathy characterized by rapidly evolving ascending weakness, mild sensory loss, and hypo- or areflexia, typically progressing to peak symptoms over the course of 4 weeks. The precise mechanism is unclear but is proposed to be an immune-mediated reaction with the generation of antibodies against peripheral nerves triggered by a preceding viral infection. Acute motor and sensory axonal neuropathy (AMSAN) is a rare and severe variant of Guillain-Barre syndrome with limited published literature. Discussion of risk factors for this subtype has not been done in a systematic way. This case report involves a 34-year-old, active duty, West African female, who immigrated to the United States in 2019. She presented with worsening diplopia, bilateral distal upper and lower extremity paresthesias as well as progressively worsening bilateral upper extremity weakness. Her clinical picture was complicated by constitutional symptoms, diffuse lymphadenopathy, no preceding viral illness, and marked clinical deterioration. Ultimately, she was diagnosed with acute motor and sensory axonal neuropathy in the setting of a new diagnosis of systemic lupus erythematosus, a rarely described association emphasizing the importance of a thorough evaluation for underlying causes of acute neurologic pathologies.

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.

Antecedent Infections and Hospital Outcomes in Demyelinating and Axonal type of Guillain-Barre Syndrome among adult Patients

Background: Many differences are observed with respect to hospital outcome among demyelinating and axonal type of Guillain-Barre Syndrome. Objective: Aims of this study was to compare the hospital outcome at discharge among demyelinating and axonal type of Guillain-Barre syndrome. Methodology: This prospective observational study was conducted from Oct, 2017 to September, 2018 at the National Institute of Neurosciences and Hospital, Dhaka, Bangladesh. All demyelinating and axonal variant of GBS, presented within 2 weeks were included in this study. The clinical parameters were taken for baseline analysis. Outcomes were measured at discharge by MRC score, length of hospital and ICU stay, recovery and death. Results: Out of 108 GBS cases 55 (50.9%) demyelinating &amp; 53 (49.1%) were axonal subtype. Mean age; 40.20 ± 16.26 vs. 32.43 ± 14.93 years in demyelinating vs. axonal cases (p=0.011). Symptom onset and nadir of weakness are quicker in axonal than demyelinating cases (8.47±4.97 vs. 12.36±7.94) (p=0.017) and (6.7 ± 4.5 vs. 8.36 ± 4.59) (p=0.022)] days. Baseline MRC score were (18.04±9.85 vs 12.94±9.91) (p=0.009) demyelinating vs. axonal cases. At discharge, Mean MRC score, length of hospital stay, length of ICU stay were, (22.15±9.38 vs 17.39±9.46; p=0.013); (15.13±17.60 vs 23.58±23.55) days (p=0.038)} and (28.69±23.28 vs 37.0±20.01) days respectively. Conclusion: Hospital outcome of GBS patients is favorable to demyelinating subtype in respect of MRC score, length of hospital stay, length of ICU stay and also death and survivability. Bangladesh Journal of Medical Microbiology, January 2022;16(1):19-24

Clinical characteristics and management outcomes of Guillain-Barré syndrome: eight-year experience at a tertiary center in jordan - a retrospective cohort study.

This retrospective study looks at adult patients admitted to a major tertiary referral hospital in the north of Jordan between 2013 and 2021. A total of 30 patients met the inclusion/exclusion criteria. Males were predominantly affected (70%) with a male-to-female ratio of 2.33. Acute inflammatory demyelinating polyradiculoneuropathy variant was encountered in 60% of cases, whereas axonal variants, namely, acute motor axonal neuropathy and acute motor axonal and sensory neuropathy variants were seen in about 23% of cases. ICU admission was reported in 37% of patients and 6.7% required mechanical ventilation. Most patients had a favorable outcome with a GBS disability score of three or better at out-patient follow-up visits. Our cohort of patients showed a significant deviation in disease expression from that reported in other parts of the globe. This deviation was obvious in more prominent male predominance, frequencies of different GBS variants, and more favorable short-term morbidity/mortality outcomes. However, larger multicenter prospective studies are needed for confirmation of these results.

Correction of dosage of the drug: Post-infectious basal ganglia encephalitis and axonal variant of Guillain-Barré syndrome after COVID-19 infection: an atypical case report

Correction of dosage of the drug: Post-infectious basal ganglia encephalitis and axonal variant of Guillain-Barré syndrome after COVID-19 infection: an atypical case report

Blink Reflex Value in the Early Diagnosis of Guillain-Barre Syndrome

Introduction: Guillain Barre Syndrome (GBS) is an autoimmunemediated demyelinating or axonal polyradiculoneuropathy characterized by progressive ascending weakness plus areflexia. Three major subtypes are demyelinating, Axonal, and Miller-Fisher Syndrome. The hallmark of the definitive diagnosis of GBS is an electrodiagnostic study. The current study is aimed to assess the value of the blink reflex, as part of an electrodiagnostic study, in the diagnosis of GBS. Methods: The current census cross-sectional study has been conducted on 55 patients with the definitive diagnosis of GBS based on clinical and electrodiagnostic manifestations from January 2018 to February 2020 in Isfahan, Iran. The patients were categorized into three groups of demyelinated, axonal, and Miller-Fisher variants and blink reflex entities, including R1, R2i, and R2c were assessed for them. All data were analyzed by independent T-test paired T-test and Chi-square tests. Results: R1 was abnormal in R1 was abnormal in 17 (30.90%), R2i in two patients (3.63%), and R2c in two patients (3.63%). Among the studied patients, five (8.47%) had abnormal latency in R1, five (8.47%) in R2i, and five (8.47%) in R2c. The comparison of abnormality in blink reflex entities among diverse subtypes, including axonal, demyelinating, and Miller-Fisher variants, revealed no significant differences (P-value>0.05). Conclusion: At least one of the blink reflex indices was abnormal in up to 30.90% of the patients, it seems that the blinking reflex cannot be considered a reliable early diagnostic test for GBS, but this fact cannot completely rule out this method and additional studies should be done with more patients in other populations.

Mixed IgG and IgM anti-GM1 ganglioside antibody positive multifocal motor neuropathy with severe secondary axonal loss in a Filipino female

Approximately 40% of patients with multifocal motor neuropathy had anti-GM1 IgM antibodies, while only 1 out of 88 patients had anti-GM1 IgG antibodies. Unlike its predominantly demyelinating IgM counterpart, the anti-GM1 IgG antibody is often seen in the axonal variant of Guillain Barre syndrome. As it affects axons, it is also associated with worse prognosis. We report here a 58-year-old woman who was admitted for 3 months history of progressive asymmetric weakness, initially involving the right-hand extensors, eventually affecting the contralateral side and the lower extremities. The electrodiagnostic examination revealed multifocal pure motor demyelinating neuropathy with severe axonal loss. On nerve ultrasound, the axons were small in non-compressive areas. The extremely elevated anti-GM1 IgM titer (1:51,200, nv&lt;1:800) was consistent with the diagnosis of MMN. Her anti-GM1 IgG antibodies (1: 12,800, nv&lt;1:800) was also elevated. In conclusion, the presence of concomitant anti-GM IgG and anti-GM1 IgM may lead to an MMN with more severe axonal loss.

Complement inhibition prevents glial nodal membrane injury in a GM1 antibody-mediated mouse model.

The involvement of the complement pathway in Guillain-Barré syndrome pathogenesis has been demonstrated in both patient biosamples and animal models. One proposed mechanism is that anti-ganglioside antibodies mediate neural membrane injury through the activation of complement and the formation of membrane attack complex pores, thereby allowing the uncontrolled influx of ions, including calcium, intracellularly. Calcium influx activates the calcium-dependent protease calpain, leading to the cleavage of neural cytoskeletal and transmembrane proteins and contributing to subsequent functional failure. Complement inhibition has been demonstrated to provide effective protection from injury in anti-ganglioside antibody-mediated mouse models of axonal variants of Guillain-Barré syndrome; however, the role of complement in the pathogenesis of demyelinating variants has yet to be established. Thus, it is currently unknown whether complement inhibition would be an effective therapeutic for Guillain-Barré syndrome patients with injuries to the Schwann cell membrane. To address this, we recently developed a mouse model whereby the Schwann cell membrane was selectively targeted with an anti-GM1 antibody resulting in significant disruption to the axo-glial junction and cytoplasmic paranodal loops, presenting as conduction block. Herein, we utilize this Schwann cell nodal membrane injury model to determine the relevance of inhibiting complement activation. We addressed the early complement component C2 as the therapeutic target within the complement cascade by using the anti-C2 humanized monoclonal antibody, ARGX-117. This anti-C2 antibody blocks the formation of C3 convertase, specifically inhibiting the classical and lectin complement pathways and preventing the production of downstream harmful anaphylatoxins (C3a and C5a) and membrane attack complexes. Here, we demonstrate that C2 inhibition significantly attenuates injury to paranodal proteins at the node of Ranvier and improves respiratory function in ex vivo and in vivo Schwann cell nodal membrane injury models. In parallel studies, C2 inhibition also protects axonal integrity in our well-established model of acute motor axonal neuropathy mediated by both mouse and human anti-GM1 antibodies. These data demonstrate that complement inhibition prevents injury in a Schwann cell nodal membrane injury model, which is representative of neuropathies associated with anti-GM1 antibodies, including Guillain-Barré syndrome and multifocal motor neuropathy. This outcome suggests that both the motor axonal and demyelinating variants of Guillain-Barré syndrome should be included in future complement inhibition clinical trials.

Post-infectious basal ganglia encephalitis and axonal variant of Guillain-Barré syndrome after COVID-19 infection: an atypical case report

Neurological complications attributed to coronavirus disease-19 (COVID-19) infection have been reported including acute disseminated encephalomyelitis, Guillain-Barré syndrome, and so on. Herein, we report a 49-year-old woman presented with acute encephalopathy and paraplegia simultaneously after COVID-19 infection. Brain magnetic resonance imaging (MRI) showed symmetric hyperintense basal ganglia lesions on T2-weighted imaging. Cerebrospinal fluid pleocytosis, motor axonal neuropathy and enhancement of conus medullaris nerve roots on spine MRI were observed. We treated her with high-dose corticosteroid and intravenous immunoglobulin.

Assessment of neurological sequelae and new-onset symptoms in the long-term follow-up of paediatric Guillain-Barre syndrome: A longitudinal study from India.

Guillain-Barre syndrome (GBS) is the commonest cause of acute flaccid paralysis in children. There is a paucity of studies that assess the long-term outcome of paediatric GBS. To assess the frequency of neurological sequelae and the new-onset symptoms in the long-term follow-up of paediatric GBS and to identify the risk factors associated with them. This longitudinal study involved 78 children with GBS treated between January 2015 and 2021. The parents of those children were contacted to visit the hospital for a detailed neurological examination and to look for new-onset symptoms after the initial treatment for GBS. Of the 78 children, acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, and acute motor-sensory axonal neuropathy variants were observed in 30 (38.5%), 27 (34.6%) and 11 (14.1%) children, respectively. The median (interquartile range (IQR)) duration of follow-up was 3 (2, 4.5) years. The median (IQR) time to independent ambulation was 30 (13.5, 105) days. The neurological sequelae were found in 22 (28.2%) children. GBS disability score at admission (odds ratio (OR)=4.6; 95% confidence interval (CI): 1.1-19.8; P=0.04) and axonal variant of GBS (OR=4.1; 95% CI: 1.5-20.8; P=0.04) were found to be independent predictors of neurologic sequelae. A total of 28 children experienced new-onset symptoms after GBS, with frequent falls while running and fatigue being the predominant symptoms. Those children with demyelinating variant achieved independent ambulation earlier than the axonal group on survival analysis (log-rank P value=0.04). The presence of neurological sequelae and new-onset symptoms were found in 28.2 and 35% of the GBS children, respectively. High GBS disability score at admission and axonal variant of GBS were independent predictors of neurological sequelae. Knowledge about these would help in devising a plan for rehabilitation.

Prognostic Implications of Early Albuminocytological Dissociation in Guillain-Barré Syndrome.

Half of Guillain-Barré syndrome (GBS) present elevated cerebrospinal fluid (CSF) protein levels within 1 week since symptom onset and 80% within 2 weeks. Our objective was to determine the clinical and prognostic implication of albuminocytological dissociation in early GBS. An ambispective cohort study was conducted. Good outcome was considered if the patient was able to walk unaided (Guillain-Barré disability score [GDS] ≤ 2 points) at 3-month follow-up. Patients were classified into two groups: with and without albuminocytological dissociation; we compared clinical and paraclinic characteristics between the groups. We analyzed clinical and electrophysiological factors related to presenting early dissociation through a multivariate model. We included 240 patients who fulfilled Asbury criteria for GBS. On further selection, only 94 patients fulfilled inclusion. Mean age was 45.94 ± 17.1 years and 67% were male. Median time from symptom onset to admission was 5 days (IQR 3-6). Regarding albuminocytological dissociation and electrophysiological variants, we found a significant difference: acute inflammatory demyelinating polyneuropathy (AIDP) [60.6% vs 26.2%, p = 0.002], acute motor axonal neuropathy (AMAN) [21.2% vs 49.1%, p = 0.009] and acute motor sensory axonal neuropathy (AMSAN) [12.1% vs 1.6%, p = 0.05]. We did not observe significant differences in recovery of independent walking in short term between both groups. The presence of conduction block in any variant (OR 3.21, 95% CI 1.12-9.16, p = 0.02) and absence of sural registration (OR 5.69, 95% CI 1.48-21.83, p = 0.011) were independent factors related to early dissociation. Early dissociation (<7 days) is not associated with any particular clinical feature or unfavorable outcome. It is more common to see in AIDP rather than axonal variants.

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.