Search

Guillain-Barré syndrome (GBS) and acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) are the most common autoimmune polyneuropathies. Their aetiology is unclear. The pathomechanism includes damage mainly to the myelin sheath and, in the long-term process, secondary axonal loss. Both inflammatory polyneuropathies involve different combinations of motor, sensory and autonomic fibres in the peripheral nerves. The differential diagnosis should be based on clinical and neurophysiological features, and laboratory tests. Numerous studies aim to demonstrate the most common errors in the diagnosis of Guillain-Barré syndrome and acute-onset CIDP. Misdiagnosis can result in the wrong treatment. We still do not have reliable markers to help diagnose the disease or to monitor the effectiveness of the therapy.

• Study assessed four adverse events following 2022–2023 seasonal influenza vaccines. • Study population was 12.7 million Medicare enrollees 65 years and older. • There was no increase in post-vaccination incidence rates for three adverse events. • Anaphylaxis rates were elevated and possibly modified by concomitant vaccination. While safety of influenza vaccines is well-established, some studies have suggested potential associations between influenza vaccines and certain adverse events (AEs). This study examined the safety of the 2022–2023 influenza vaccines among U.S. adults ≥ 65 years. A self-controlled case series compared incidence rates of anaphylaxis, encephalitis/encephalomyelitis, Guillain-Barré Syndrome (GBS), and transverse myelitis following 2022–2023 seasonal influenza vaccinations (i.e., any, high-dose or adjuvanted) in risk and control intervals among Medicare beneficiaries ≥ 65 years. We used conditional Poisson regression to estimate incidence rate ratios (IRRs) and 95 % confidence intervals (CIs) adjusted for event-dependent observation time and seasonality. Analyses also accounted for uncertainty from outcome misclassification where feasible. For AEs with any statistically significant associations, we stratified results by concomitant vaccination status. Among 12.7 million vaccine recipients, we observed 76 anaphylaxis, 276 encephalitis/encephalomyelitis, 134 GBS and 75 transverse myelitis cases. Only rates of anaphylaxis were elevated in risk compared to control intervals. With all adjustments, an elevated, but non-statistically significant, anaphylaxis rate was observed following any (IRR: 2.40, 95% CI: 0.96–6.03), high-dose (IRR: 2.31, 95% CI: 0.67–7.91), and adjuvanted (IRR: 3.28, 95% CI: 0.71–15.08) influenza vaccination; anaphylaxis IRRs were 2.54 (95% CI: 0.49–13.05) and 1.64 (95% CI: 0.38–7.05) for persons with and without concomitant vaccination, respectively. Rates of encephalitis/encephalomyelitis, GBS, or transverse myelitis were not elevated following 2022–2023 seasonal influenza vaccinations among U.S. adults ≥ 65 years. There was an increased rate of anaphylaxis following influenza vaccination that may have been influenced by concomitant vaccination. [ABSTRACT FROM AUTHOR]

In May 2023, the Food and Drug Administration (FDA) licensed Arexvy and Abrysvo vaccines for prevention of respiratory syncytial virus (RSV) lower respiratory tract disease in adults aged =60 years. In prelicensure trials, Guillain-Barré syndrome (GBS) was identified as a potential safety concern. During August 4, 2023-March 30, 2024, at least 10.6 million adults aged =60 years received a recommended RSV vaccine. During May 3, 2023-April 14, 2024, CDC reviewed data reported after RSV vaccination to V-safe, an active U.S. surveillance system that invites enrolled participants to complete web-based surveys, and reports to the Vaccine Adverse Event Reporting System (VAERS), a passive, voluntary surveillance system that accepts adverse event reports from the public, providers, and manufacturers. Findings from V-safe and VAERS were generally consistent with those from trials. Reporting rates of GBS after RSV vaccination in VAERS (5.0 and 1.5 reports per million doses of Abrysvo and Arexvy vaccine administered, respectively) were higher than estimated expected background rates in a vaccinated population. CDC and FDA are conducting population-based surveillance to assess risks for GBS and other adverse events. Findings from these studies will help guide development of Advisory Committee on Immunization Practices recommendations. [ABSTRACT FROM AUTHOR]

Objective: Most studies investigated the relationship between COVID-19 and Guillain-Barré syndrome (GBS) by comparing the incidence of GBS before and during the pandemic of COVID-19. However, the findings were inconsistent, probably owing to varying degrees of the lockdown policy. The quarantine requirements and travel restrictions in China were lifted around December 7, 2022. This study aimed to explore whether the relative frequency of GBS increased during the major outbreak in the absence of COVID-19-mandated social restrictions in China. Methods: GBS patients admitted to the First Hospital, Shanxi Medical University, from December 7, 2022 to February 20, 2023, and from June, 2017 to August, 2019 were included. The relative frequencies of GBS in hospitalized patients during different periods were compared. The patients with and without SARS-CoV-2 infection within six weeks prior to GBS onset formed the COVID-GBS group and non-COVID-GBS group, respectively. Results: The relative frequency of GBS among hospitalized patients during the major outbreak of COVID-19 (13/14,408) was significantly higher than that before the COVID-19 epidemic (29/160,669, P < 0.001). More COVID-GBS patients (11/13) presented AIDP subtype than non-COVID-GBS cases (10/27, P = 0.003). The mean interval between onset of infective symptoms and GBS was longer in COVID-GBS (21.54 ± 11.56 days) than in non-COVID-GBS (5.76 ± 3.18 days, P < 0.001). Conclusions: COVID-19 significantly increased the incidence of GBS. Most COVID-GBS patients fell into the category of AIDP, responded well to IVIg, and had a favorable prognosis. [ABSTRACT FROM AUTHOR]

Background: Guillain-Barré syndrome (GBS) frequently leads to respiratory failure and autonomic dysfunction, resulting in approximately one-third of patients requiring mechanical ventilation. Objective: This study aimed to identify clinical predictors for mechanical ventilation in patients with GBS. Methods: This research was conducted from 2010 to 2021 using registries from a tertiary hospital in an upper middle-income Latin American country. Participants were categorized into two groups based on their ventilation status. Demographic data were collected, and independent predictors of the need for mechanical ventilation were determined through multivariate logistic regression analysis. Results: Dysautonomic events occurred in 36% of the patients, with 17% requiring mechanical ventilation; the average duration of intubation was 1.16 ± 3.18 days. The multivariate analysis indicated that bulbar dysfunction significantly increased the likelihood of requiring mechanical ventilation by 19-fold (OR 18.67, 95% CI 5.85-59.42), followed by ophthalmoplegia, which increased the likelihood by sixfold (OR 5.68, 95% CI 1.28-25.19). Conclusion: Bulbar dysfunction, dysautonomia, and lower Medical Research Council (MRC) scores were significant predictors of the need for mechanical ventilation in hospitalized GBS patients. These findings support the need for close monitoring and early admission to the intensive care unit (ICU) admission for at-risk patients. [ABSTRACT FROM AUTHOR]

Background: Guillain–Barré syndrome (GBS) is one of the most common causes of acute neuromuscular paralysis in developed and developing countries. It is a complex autoimmune disorder characterized by progressive skeletal muscle weakness, potentially involving respiratory muscles. The purpose of this case report was to explore the importance of combined use of incentive spirometry and progressive muscle training as essential therapies in a 20-year-old male diagnosed with GBS. Case presentation: This is a case report of a 20-year-old Nigerian male university student who was brought to the trauma center on a wheelchair with weakness of both upper and lower limbs which was said to be progressive, and a diagnosis of GBS was made by the attending physician. On examination, he was discovered to have quadriparesis with mild respiratory muscle involvement. He deteriorated with cardiopulmonary compromise and was transferred into intensive care unit. He was managed with supplementary oxygen therapy until there was significant improvement and he was weaned off oxygen therapy and then needed further evaluation and management. The patient was placed on immunoglobulin in conjunction with incentive spirometry, progressive strengthening exercise and exercise training which proved to be effective as he regained all the lost function within a few weeks of symptoms. Results: This intervention delves into the synergistic potential of fortifying respiratory muscles and improving respiratory function alongside overall muscle strength which resulted in the patient regaining all the lost function within a few weeks. Conclusions: The combined use of incentive spirometry and progressive muscle training in addition to immunoglobulin was effective in modulating GBS related impairments. We recommend the use of a multifaceted strategy in the management of patients with GBS and other similar health conditions. [ABSTRACT FROM AUTHOR]

Background: Increasing evidence has suggested that alterations in the gut microbiome are correlated with autoimmune neurologic disorders, yet the causal relationship between them has yet to be established. Methods: From the published genome-wide association study (GWAS) summary statistics, we obtained data on the gut microbiota and three autoimmune neurologic disorders (Multiple Sclerosis, Guillain-Barré Syndrome, and Myasthenia Gravis). We then implemented a two-sample Mendelian Randomization (MR) to determine the causal relationship between the gut microbiota and the diseases. To validate the results, we conducted a series of sensitivity analyses. Finally, to verify the direction of causality, a reverse-causality analysis was done. Results: We discovered that a higher relative abundance of the genus Ruminococcus2 (OR: 1.213, 95% CI: 1.006--1.462, p = 0.043, PFDR = 0.048) and the genus Roseburia (OR: 1.255, 95% CI: 1.012--1.556, p = 0.038, PFDR = 0.048) were associated with a higher risk of MS. Furthermore, the higher the abundance of the class Mollicutes (OR: 3.016, 95% CI: 1.228--7.411, p = 0.016, PFDR = 0.021), the genus Eubacterium (hallii group) (OR: 2.787, 95% CI: 1.140--6.816, p = 0.025, PFDR = 0.025), and the phylum Tenericutes (OR: 3.016, 95% CI: 1.228--7.411, p = 0.016, PFDR = 0.021) was linked to a greater probability of GBS. Additionally, the higher the abundance of the genus Ruminococcaceae UCG005 (OR: 2.450, 95% CI: 1.072--5.598, p = 0.034, PFDR = 0.036), the genus Holdemania (OR: 2.437, 95% CI: 1.215--4.888, p = 0.012, PFDR = 0.024), genus Lachnoclostridium (OR: 3.681, 95% CI: 1.288--10.521, p = 0.015, PFDR = 0.025) and the genus Eubacterium (ruminantium group) (OR: 2.157, 95% CI: 1.211--3.843, p = 0.003, PFDR = 0.016) correlated with a greater chance of MG occurrence. No SNPs were identified as outliers through sensitivity analysis. Then, the results of the reverse MR analysis did not indicate any reverse causality. Conclusion: Our findings demonstrate a causal relationship between the gut microbiota and three autoimmune neurologic disorders, providing novel insights into the mechanisms of these autoimmune neurologic disorders that are mediated by gut microbiota. [ABSTRACT FROM AUTHOR]

Background: Anti-GQ1B syndrome includes a group of diseases characterized by antibody-mediated polyneuropathy. Guillain Barre syndrome (GBS) and the Miller-Fisher syndrome (MFS) have been related to COVID-19 vaccine application. Clinic case: 48-year-old man, with history of Pfizer-BioNTech vaccination against COVID-19, 5 days prior to the symptoms, who assisted to the Emergency room with blurred vision and diplopia; adding dysarthria, facial diplegia and left upper limb weakness after 48 hours. In his first evaluation it was found ophthalmoplegia, facial diplegia, decreased gag reflex, weakness of thoracic limbs, bilateral trapezius muscle and areflexia. Serum studies and nuclear magnetic resonance of the brain were performed without alterations. It was complemented with IgG anti-ganglioside GQ1b antibodies with a positive result. Once the diagnosis was confirmed, treatment was started with immunoglobulin calculated at 2 g per kg for 5 days. The patient was discharged once the immunoglobulin was administered with evaluation at 2 months without ataxia, ophthalmoplegia, areflexia and weakness. Conclusions: Following the documented reports of GBS and its variants secondary to vaccination, neurological side effects have been catalogued as being of great importance. Therefore, the reported case can be used as a point of reference to consider this clinical spectrum as a differential diagnosis in patients with post-vaccination neurological symptomatology. [ABSTRACT FROM AUTHOR]

Objective: Guillain–Barré Syndrome (GBS) is an autoimmune degenerative disease commonly presenting with acute progressive sensorimotor paralysis, sometimes associated with dysautonomia, facial diplegia and severe respiratory distress. Gastroenteritis due to Campylobacter jejuni and respiratory infection secondary to Epstein–Barr virus usually precede GBS; however, vaccination or recent dengue infection as temporal causes are rarely reported. Case Presentation: Here, we present a 20‐year‐old man, who complained of a 5‐day history of a progressively worsening tingling sensation isolated in the hands and feet, and unilateral Bell's palsy on the left side of his face, which occurred 2 weeks after his severe acute respiratory syndrome coronavirus 2 vaccination and 6 weeks from his recent hospitalization from Dengue fever. Except for the left complete unilateral facial paralysis, weakness in the intrinsic hands and feet muscles, and sensory ataxia, the rest of his physical examination was unremarkable. On work‐up, the findings of albuminocytological dissociation and distal sensorimotor demyelinating polyneuropathy in the nerve conduction study supported the diagnosis of GBS. Conclusion: The enhanced immune response from a recent dengue infection and severe acute respiratory syndrome coronavirus 2 vaccination might increase the risk of GBS. The predominantly distal GBS phenotype has rarely been reported in the literature, adding to the peculiarity of this case. [ABSTRACT FROM AUTHOR]

Background and purpose: Clinical symptoms and laboratory indices for acute inflammatory demyelinating polyneuropathy (AIDP), a variant of Guillain−Barré syndrome, and acute‐onset chronic inflammatory demyelinating polyneuropathy (A‐CIDP) were analyzed to identify factors that could contribute to early differential diagnosis. Methods: A retrospective chart review was performed on 44 AIDP and 44 A‐CIDP patients looking for any demographic characteristics, clinical manifestations or laboratory parameters that might differentiate AIDP from acutely presenting CIDP. Results: In Guillain−Barré syndrome patients (N = 63), 69.84% (N = 44) were classified as having AIDP, 19.05% (N = 12) were found to have acute motor axonal neuropathy, 6.35% (N = 4) were found to have acute motor and sensory axonal neuropathy, and 4.76% (N = 3) were found to have Miller Fisher syndrome. Serum uric acid (UA) was higher in A‐CIDP patients (329.55 ± 72.23 μmol/L) than in AIDP patients (221.08 ± 71.32 μmol/L) (p = 0.000). Receiver operating characteristic analyses indicated that the optimal UA cutoff was 283.50 μmol/L. Above this level, patients were more likely to present A‐CIDP than AIDP (specificity 81.80%, sensitivity 81.80%). During the follow‐up process, serum samples were effectively collected from 19 AIDP patients during the rehabilitation phase and 28 A‐CIDP patients during the remission stage, and it was found that UA levels were significantly increased in A‐CIDP (remission) (298.9 ± 90.39 μmol/L) compared with AIDP (rehabilitation) (220.1 ± 108.2 μmol/L, p = 0.009). Conclusion: These results suggest that serum UA level can help to differentiate AIDP from A‐CIDP with high specificity and sensitivity, which is helpful for early diagnosis and guidance of treatment. [ABSTRACT FROM AUTHOR]

Background: Guillain-Barré syndrome (GBS), as the most common cause of acute flaccid paralysis worldwide, is considered a part of a clinical spectrum in which discrete, complete, or incomplete forms of GBS and overlapping syndromes lie on the basis of their clinical features. The term overlapping Miller Fisher syndrome (MFS)/GBS is used when patients with MFS also suffer from progressive motor weakness of the limbs. Anti-ganglioside GQ1b has been specifically associated with MFS and ophthalmoplegia. Case description: Here, we report a Chinese girl who was diagnosed with overlapping MFS/GBS showing acute flaccid paralysis of all four limbs, sensory symptoms, cranial nerve dysfunction, autonomic involvement, ophthalmoplegia, and ataxia. She had high serum and cerebrospinal fluid titres of monospecific anti-GM4 IgG antibody instead of anti-GQ1b antibody in the acute phase. Conclusion: Anti-GM4 antibodies usually coexist with other antiganglioside antibodies, leading to missed diagnoses. The findings of the present study show that antibodies to ganglioside GM4 may in overlapping MFS/GBS as the lone immunological factors. [ABSTRACT FROM AUTHOR]

Background: Brain- and blood-derived protein analysis in the cerebro-spinal fluid (CSF) in various studies performed abroad found that some proteins and their isoforms were altered significantly in Guillain-Barre' syndrome (GBS) patients in comparison to controls. However, data are lacking in India with respect to the blood- or brain-derived proteins in patients of GBS. Objective: This study aimed to identify the role of apolipoprotein A IV (Apo A IV) and haptoglobin as potential protein markers in CSF of patients with GBS in our population. Materials and Methods: The study comprised 28 participants where 12 confirmed cases of GBS and 16 control subjects admitted for non-infectious neurological disorders were recruited after obtaining approval from the Institutional Ethics Committee. CSF glucose, protein, and adenosine deaminase were analyzed using an autoanalyzer. The concentrations of Apo A IV and haptoglobin were estimated with enzyme-linked immuno-sorbent assay (ELISA) kits. Results: The CSF protein concentrations of cases were higher as compared to controls. The concentrations of haptoglobin and Apo A IV were higher in the confirmed cases of GBS as compared to the control subjects, and this difference was found to be significant. The receiver operating characteristic curve analysis for haptoglobin revealed that the area under the curve (AUC) was 0.867 (95% CI: 0.732-1.001), with a sensitivity of 83.8% and a specificity of 63.3%. The AUC for Apo A IV was 0.883 (95% CI: 0.758-1.009), with a sensitivity of 91.7% and a specificity of 73.3%. Conclusions Haptoglobin along with Apo A IV can emerge as a potential biochemical marker in CSF for the diagnosis of GBS. [ABSTRACT FROM AUTHOR]

Background: Varicella infection, commonly known as chickenpox, can lead to various neurological complications. The study aimed to investigate the clinical characteristics, imaging findings, and outcomes of neurological complications following varicella infection. Methods: An observational study was conducted involving sixty patients presenting with neurological abnormalities subsequent to acute varicella infection were included. Data collection involved comprehensive neurological examinations and investigations, including imaging studies and cerebrospinal fluid analysis. Treatment outcomes were assessed through a multi-disciplinary approach and follow-up evaluations over a 3-month period. Statistical analysis was performed using SPSS version 21.0, emphasizing descriptive and inferential statistics. Results: The participants had a mean age of 38 years and 60% males. Encephalitis (41.7%), meningitis (25.0%), and Guillain-Barré Syndrome (16.7%) were the most prevalent complications. Complications peaked 30 days after varicella infection. Intravenous Immunoglobulin (IVIG) improved Guillain-Barré Syndrome more than plasmapheresis. Complication severity increased neurological symptom duration. In 60 patients, demographic characteristics were significantly associated with post-varicella neurological sequelae. Older patients had increased encephalitis and meningitis risks (p < 0.05), but gender did not significantly affect risk (p > 0.05). Post-infection complications peaked within 30 days and then decreased (p < 0.01). IVIG was more effective than plasmapheresis in treating Guillain Barré Syndrome (p < 0.05). Corticosteroids improved cerebellitis symptoms faster (p < 0.01). Prolonged symptoms were linked to severe consequences (p < 0.001), increasing our understanding of post-varicella neurological sequelae. Conclusion: The study underscores the diverse spectrum of neurological complications following varicella infection. Early recognition and appropriate management are essential for favorable outcomes. The findings contribute to better understanding and management strategies for these complications. Recommendations: Further research with larger sample sizes is warranted to validate these findings and explore additional prognostic factors. Multicenter studies could provide broader insights into the epidemiology and outcomes of varicella-associated neurological complications. [ABSTRACT FROM AUTHOR]

Diaphragmatic dysfunction can arise from various factors, and Guillain–Barre syndrome, characterized by acute inflammatory polyradiculoneuropathy, is one such cause that may result in respiratory failure due to diaphragmatic paralysis. Prompt recognition and timely intervention, including airway protection and addressing the underlying pathology, are crucial for achieving optimal patient outcomes. Point-of-care ultrasound, specifically utilizing the M-mode function, can be employed for individuals displaying symptoms of diaphragmatic paralysis. This diagnostic approach is uncomplicated an effective tool for serial follow-up. In this context, we present a case series involving three patients with diaphragmatic paralysis in a limited-resource setting. [ABSTRACT FROM AUTHOR]

Copyright of Argentinian Journal of Respiratory & Physical Therapy (AJRPT) is the property of Asociacion Civil Cientifica de Difusion y Promocion de la Kinesiologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Scrub typhus is an acute infectious disease caused by Orientia tsutsugamushi. Guillain–Barre syndrome (GBS) is an autoimmune-mediated peripheral neuropathy with a frequent history of prodromal infections, but GBS associated with scrub typhus is very rare. Case presentation: We report a 51-year-old male patient who developed dysarthria and peripheral facial paralysis following the cure of scfrub typhus. CSF examination and electrophysiological findings suggested a diagnosis of GBS. After treatment with intravenous immunoglobulin, the patient's neurological condition improved rapidly. Conclusions: Scrub typhus infection is likely to be a potential predisposing factor in GBS, while scrub typhus-associated GBS has a favorable prognosis. [ABSTRACT FROM AUTHOR]

Introduction: Anti-sulfatide antibodies are key biomarkers for the diagnosis of Guillain-Barré syndrome (GBS). However, case reports on anti-sulfatide antibody-related GBS are rare, particularly for atypical cases. Case description, case 1: A 63 years-old man presented with limb numbness and diplopia persisting for 2 weeks, with marked deterioration over the previous 4 days. His medical history included cerebral infarction, diabetes, and coronary atherosclerotic cardiomyopathy. Physical examination revealed limited movement in his left eye and diminished sensation in his extremities. Initial treatments included antiplatelet agents, cholesterol-lowering drugs, hypoglycemic agents, and medications to improve cerebral circulation. Despite this, his condition worsened, resulting in bilateral facial paralysis, delirium, ataxia, and decreased lower limb muscle strength. Treatment with intravenous highdose immunoglobulin and dexamethasone resulted in gradual improvement. A 1 month follow-up revealed significant neurological sequelae. Case description, case 2: A 53 years-old woman was admitted for adenomyosis and subsequently experienced sudden limb weakness, numbness, and pain that progressively worsened, presenting with diminished sensation and muscle strength in all limbs. High-dose intravenous immunoglobulin, vitamin B1, and mecobalamin were administered. At the 1 month follow-up, the patient still experienced limb numbness and difficulty walking. In both patients, albuminocytologic dissociation was found on cerebrospinal fluid (CSF) analysis, positive anti-sulfatide antibodies were detected in the CSF, and electromyography indicated peripheral nerve damage. Conclusion: Anti-sulfatide antibody-related GBS can present with Miller-Fisher syndrome, brainstem encephalitis, or a combination of the two, along with severe pyramidal tract damage and residual neurological sequelae, thereby expanding the clinical profile of this GBS subtype. Anti-sulfatide antibodies are a crucial diagnostic biomarker. Further exploration of the pathophysiological mechanisms is necessary for precise treatment and improved prognosis. [ABSTRACT FROM AUTHOR]

Guillain-Barré syndrome (GBS) is a rare postoperative complication that is sometimes characterized by serious motor weakness and prolonged weaning from mechanical ventilation. Although the exact nature of the relationship between GBS and the surgical procedure is still unclear, there is a clear increased incidence of GBS in post-surgical patients compared to non-surgical patients. GBS after surgery is unique in several ways. The course of post-surgical GBS unfolds more rapidly than in other situations where GBS develops, the condition is often more severe, and respiratory muscles are more commonly involved. Prompt diagnosis and appropriate treatment are essential, and the condition can worsen if treated inappropriately. Postoperative sedation, intubation, and restraint use make the diagnosis of GBS difficult, as the onset of symptoms of weakness or numbness in those contexts are not obvious. GBS is often misdiagnosed, being attributed to other postoperative complications, and subsequently mishandled. The lack of relevant information further obscures the clinical picture. We sought to better understand post-surgical GBS by performing an analysis of the relevant literature, focusing on clearly documenting the clinical characteristics, diagnosis, and management of GBS that emerges following surgery. We underscore the importance of physicians being aware of the possibility of GBS after major surgery and of performing a variety of laboratory clinical investigations early on in suspected cases. [ABSTRACT FROM AUTHOR]

Guillain-Barré Syndrome (GBS) is an autoimmune neuropathy. Antecedent infections have been seen to be significant triggering factors for developing GBS. Among them, arboviral infections are rapidly gaining importance as significant triggers, especially in the areas where they are endemic. Chikungunya, an arboviral infection that usually causes a self-limiting acute febrile illness can lead to GBS as one its severe complications. Herein, we describe a case of a 21-year-old female who presented with weakness in all four limbs and paresthesia. Nerve conduction study and cerebrospinal fluid (CSF) analysis showed axonal, demyelinating motor and sensory neuropathy with albuminocytological dissociation indicating Acute Motor and Sensory Axonal Neuropathy (AMSAN) variant of GBS. Serum IgM antibodies against ganglioside GM1 were detected. Anti-Chikungunya IgM antibodies were found in both serum and CSF samples. The patient was initiated with Intravenous Immunoglobulin (IVIG) therapy. In view of hypoxia, she was intubated and was on mechanical ventilation. After 2 weeks of being comatose, the patient gradually improved and was discharged with no sequelae. A literature review on antecedent infections in GBS is presented alongside the case report to better understand the association of GBS with antecedent infections, especially the endemic arboviral infections like Chikungunya, Dengue and Zika. This will help in reinforcing the significance of having robust surveillance and public health control measures for infectious diseases. [ABSTRACT FROM AUTHOR]

Background and objectives. This study explores the impact of plasma exchange therapy (TPE) on muscle strength and functional outcomes in patients with acute immune-mediated neuropathy. Materials and methods. We retrospectively analyzed clinical data from patients diagnosed with acute immune-mediated neuropathy at the Department of Neurology, Bolan University of Medical Sciences, Baluchistan, from January 2016 to December 2021. Results. The study, comprising 141 patients with various neurological disorders, underscores the efficacy of plasma exchange therapy in improving muscle strength and functional outcomes, particularly in Guillain-Barré Syndrome (GBS). Notably, after four weeks of treatment, a significant proportion of patients demonstrated improved mobility, with 51.8% able to walk without assistance. However, despite the positive response to therapy, a small percentage (2.8%) experienced unfavorable outcomes marked by expiration. Additionally, the study identifies significant associations between treatment outcomes and patient characteristics, such as the number of plasma exchange cycles and diagnosis (p <0.05), emphasizing the importance of personalized treatment approaches in managing neurological disorders. Conclusions. Our findings underscore the effectiveness of plasma exchange in enhancing muscle strength and functional outcomes in acute immune-mediated neuropathy, particularly GBS. [ABSTRACT FROM AUTHOR]

Guillain Barre syndrome (GBS) following Varicella zoster is a rare presentation and has only been reported in a few cases around the world. Of the reported cases, the type of GBS is not specified in the majority, and where specified is of the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) type. We report a case of acute motor axonal neuropathy (AMAN) type GBS following herpes zoster in a 27-year-old male who presented with bilateral lower limb weakness and left sided lower motor neuron type facial nerve palsy a week after herpes zoster infection. [ABSTRACT FROM AUTHOR]

Objective The risk factors for respiratory insufficiency in children with Guillain–Barré syndrome (GBS) are poorly known. This study aimed to investigate the factors associated with respiratory insufficiency in children with GBS. Methods This retrospective study included children diagnosed with GBS by pediatric neurologists and admitted at the Wuhan Children's Hospital and other hospitals from January 2013 to October 2022. The patients were divided into the respiratory insufficiency and nonrespiratory insufficiency groups according to whether they received assist breathing during treatment. Results The median (interquartile range) age of onset of 103 patients were 5 (3.1–8.5) years, 69 (67%) were male, and 64 (62.1%) had a history of precursor infection. Compared with the nonrespiratory insufficiency group, the respiratory insufficiency group showed more facial and/or bulbar weakness (p = 0.002), a higher Hughes Functional Grading Scale (HFGS) at admission (p < 0.001), and a shorter onset-to-admission interval (p = 0.017). Compared with the acute motor axonal neuropathy (AMAN) subtype, the acute inflammatory demyelinating polyneuropathy (AIDP) subtype showed longer days from onset to lumbar (p = 0.000), lower HFGS at admission (p = 0.04), longer onset-to-admission interval (p = 0.001), and more cranial nerve involvement (p = 0.04). The incidence of respiratory insufficiency between AIDP and AMAN showed no statistical difference (p > 0.05). Conclusion In conclusion, facial and/or bulbar weakness, HFGS at admission, and onset-to-admission interval were associated with respiratory insufficiency and might be useful prognostic markers in children with GBS. [ABSTRACT FROM AUTHOR]

Introduction: Coronaviruses can cause widespread systemic infections, the most common of which are respiratory complications, which are close to the symptoms of serious acute respiratory syndrome coronavirus (SARS-CoV). Case Description: We report a case of a 16-year-old boy who developed weakness in bilateral lower limbs, difficulty in coughing, and generalized weakness for 2 days. He was diagnosed with post-COVID Guillain-Barre syndrome (GBS). Cerebrospinal fluid (CSF) analysis manifested a CSF protein of 117 mg/dL, a white blood cell count of 6-7/mm³, and a glucose of too low to comment. Magnetic resonance investigation of the brain revealed mucosal thickening in the bilateral maxillary, ethmoid, and left frontal sinuses. Nerve conduction studies concluded evidence of sensory-motor polyneuropathy. Physiotherapy intervention included patient education, breathing retraining, airway clearance techniques, positioning, a combination of chest proprioceptive neuromuscular facilitation (PNF) techniques, a walking program with supplemented oxygen, and psychological support. Results: Outcome measures have shown enhancement in functional independence and performance of activities of daily living. Conclusion: The evidence from this study suggests that pulmonary rehabilitation plays a pivotal role in managing a patient who is diagnosed with post-COVID GBS, which includes patient education, breathing retraining, airway clearing techniques, a combination of chest PNF techniques, positioning, a walking program with augmented oxygen, and psychological support. [ABSTRACT FROM AUTHOR]

This letter, published in the Journal of Neurology, discusses a case of myelitis as a side effect of tofersen therapy in a patient with SOD1-associated ALS. Tofersen is an antisense oligonucleotide that has been approved for the treatment of SOD1-associated ALS. The patient developed myelitis during therapy and experienced symptoms such as generalized myalgias and leg weakness. Treatment with prednisolone resulted in regression of symptoms, although leg weakness persisted. The case highlights the rare occurrence of myelitis as a side effect of tofersen therapy and suggests the need for closer observation of patients and attention to transient weakness after injections. The letter also mentions the potential for immunological or inflammatory responses and complications with the use of antisense oligonucleotides in neurological disorders. [Extracted from the article]

Background: Global vaccination against COVID-19 will help nations to overcome the pandemic stage as soon as possible. Guillain--Barré syndrome (GBS) is an acute immune-mediated inflammatory disease of the peripheral nerves (PNS) that is reported as a complication of both COVID-19 and vaccines. Up to now, case reports regarding the incidence of GBS have been reported after different COVID-19 vaccines worldwide. So, the aim of this systematic review and meta-analysis is to estimate the pooled incidence of GBS after COVID-19 vaccination. Methods: Two expert researchers conducted a systematic search in PubMed, Scopus, EMBASE, Web of Science, Google Scholar as well as gray literature in order to find relevant articles published before September 2022. Results: After deleting duplicates, we found 1021 articles, of which 458 studies were further evaluated. A final number of 21 studies remained for meta-analysis, with most of those being from the USA, UK and Mexico. Follow-up duration was between 21-42 days. Out of the total number of 2.35x109 patients included in the final meta-analysis, 3654 subjects developed GBS after vaccination, most of whom were males. Incidence of GBS per million ranged between 0.23 and 9.8. The pooled incidence of GBS following vaccination was 0%. [ABSTRACT FROM AUTHOR]

This article explores the use of therapeutic plasma exchange (TPE) as a treatment for neuroimmunological disorders in children. The study found that TPE was effective and well-tolerated, with a partial response observed in most cases. However, despite positive clinical response, a high rate of neurological sequelae was observed. The article emphasizes the need for further research on the efficacy and safety of TPE in pediatric patients with neurological disorders. It also discusses the use of TPE in specific conditions such as Guillain-Barré syndrome and acute disseminated encephalomyelitis, highlighting the need for more research in these areas as well. Overall, the article suggests that TPE is a promising treatment option but more studies are needed to establish standard therapeutic modalities for pediatric patients. [Extracted from the article]

In this case report, we present a 57-year-old woman, experiencing worsening attention and concentration in the past month. She had paranoid and persecutory delusions regarding her husband. In the initial evaluation, her cardiac and liver enzymes were found to be elevated, and her blood pressure was high. She was taking antihypertensives, antidepressants, and disulfiram for the treatment of her addiction to alcohol. Her initial neurological examination was nonspecific. Mental status examination revealed distractible attention, psychomotor agitation, and poor insight. A cranial MRI revealed signal intensity changes suggestive of vasogenic edema in bilateral hemispheres and the basal ganglia. The atypical presentation of Posterior Reversible Encephalopathy Syndrome (PRES) was diagnosed. The encephalopathy presentation and peripheral toxic symptomatology were resolved following hydration and antihypertensive medication; yet, during the follow-up of the patient, motor deficits in the lower extremity were noted. An EMG examination indicated motor polyneuropathy with axonal involvement. As her muscular weakness increased, IVIG was initiated, and the symptoms of polyneuropathy improved rapidly. The multisystemic pathologies developed in a short time were indicative of potential toxicity, while other infectious, autoimmune, infiltrative, and metabolic etiology were excluded upon laboratory tests and imaging. The use of disulfiram was thought to be a key element in the development of this chain of pathologies. Based on our knowledge, this is the first case to be reported in which the cardiovascular, hepatic, muscular, central, and peripheral nervous system toxicity of disulfiram was observed simultaneously, which is the reason why we consider it important to be presented. [ABSTRACT FROM AUTHOR]

Introduction: Acute presentations and emergencies in neuromuscular disorders (NMDs) often challenge clinical acumen. The objective of this review is to refine the reader's approach to history taking, clinical localization and early diagnosis, as well as emergency management of neuromuscular emergencies. Methods: An extensive literature search was performed to identify relevant studies. We prioritized meta‐analysis, systematic reviews, and position statements where possible to inform any recommendations. Summary: The spectrum of clinical presentations and etiologies ranges from neurotoxic envenomation or infection to autoimmune disease such as Guillain–Barré Syndrome (GBS) and myasthenia gravis (MG). Delayed diagnosis is not uncommon when presentations occur "de novo," respiratory failure is dominant or isolated, or in the case of atypical scenarios such as GBS variants, severe autonomic dysfunction, or rhabdomyolysis. Diseases of the central nervous system, systemic and musculoskeletal disorders can mimic presentations in neuromuscular disorders. Conclusions: Fortunately, early diagnosis and management can improve prognosis. This article provides a comprehensive review of acute presentations in neuromuscular disorders relevant for the emergency physician. [ABSTRACT FROM AUTHOR]

Background: Tough COVID-19 predominantly affects the respiratory tract, and extra-pulmonary manifestations, including neuromuscular complaints have been associated with this disorder. It is vital to monitor COVID-19 cases for the occurrence of Neuromuscular Disorders (NMDs), which could be overshadowed by severe respiratory and cardiovascular symptoms. In this study, we reported electrophysiological findings of a series of COVID-19 patients with complaints of paresthesia and weakness. Methods: In this case series, the Electrodiagnostic studies (EDX) of 36 patients with recent complaints of weakness or paresthesia and a history of COVID-19 before symptoms were reported. Results: 12 cases (33.3%) had abnormal EDX, five males and seven females, with a mean age of 51.42±11.49 years, history of hospitalization in five cases (41.7%), and ICU admission in four (33.3%). Seven cases were concluded as having a predominantly axonal type polyneuropathy (five sensory-motor and two sensory polyneuropathies). Of these seven, one was suggestive of Critical Illness Neuropathy (CIN). Three cases demonstrated a myopathic pattern with a history of ICU admission, hence the impression of Critical Illness Myopathy (CIM). In addition, one of these three, developed both myopathy and neuropathy and thus, is considered as having CIM/N. One case was diagnosed with Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). The last case demonstrated an inflammatory involvement of lumbosacral roots (COVID-19-related radiculitis). Conclusion: COVID-19 could be associated with a wide range of NMDs. In this study, the presence of axonal polyneuropathy, CIDP, and myopathy was demonstrated following SAR-COV-2 infection. Also, CIN/M was observed in COVID-19 patients with a history of ICU admission. [ABSTRACT FROM AUTHOR]

Familial Mediterranean Fever (FMF) is the most common autoinflammatory disease characterized by recurrent episodes of abdomin al pain, fever and serositis. FMF is an autosomal recessively inherited and self-limiting disease. It is more common in countries around the Mediterranean. Guillain Barre Syndrome (GBS) is an acute, immune-mediated polyneuropathy affecting peripheral nerves and nerve roots. GBS is usually characterized by progressive flaccid paralysis and decreased deep tendon reflexes. Central nervous system involvement is not common in the course of FMF. Guillain Barre Syndrome developed in a patient who was followed with colchicine treatment for 1 year due to Familial Mediterranean Fever. In the literature review, no association of these two dise ases was found. This case is presented to draw attention to the coexistence of immune-mediated Familial Mediterranean Fever and Guillain Barre Syndrome. In this case, it was thought that two inflammatory diseases may have affected each other or autoinflammatory diseases can be seen together. [ABSTRACT FROM AUTHOR]

Background: Guillain–Barré syndrome (GBS), a post-infectious, immune-mediated, acute demyelinating disease of the peripheral nerves and nerve roots, represents the most prevalent and severe acute paralyzing neuropathy. Purinergic P2X7 receptors (P2X7R) play a crucial role in central nervous system inflammation. However, little is known about their role in the immune-inflammatory response within the peripheral nervous system. Methods: Initially, we assessed the expression of purinergic P2X7R in the peripheral blood of patients with GBS using flow cytometry and qRT-PCR. Next, we explored the expression of P2 X7R in CD4+ T cells, CD8+ T cells, and macrophages within the sciatic nerves and spleens of rats using immunofluorescence labeling and flow cytometry. The P2X7R antagonist brilliant blue G (BBG) was employed to examine its therapeutic impact on rats with experimental autoimmune neuritis (EAN) induced by immunization with the P0180 − 199 peptide. We analyzed CD4+ T cell differentiation in splenic mononuclear cells using flow cytometry, assessed Th17 cell differentiation in the sciatic nerve through immunofluorescence staining, and examined the expression of pro-inflammatory cytokine mRNA using RT-PCR. Additionally, we performed protein blotting to assess the expression of P2X7R and NLRP3-related inflammatory proteins within the sciatic nerve. Lastly, we utilized flow cytometry and immunofluorescence labeling to examine the expression of NLRP3 on CD4+ T cells in rats with EAN. Results: P2X7R expression was elevated not only in the peripheral blood of patients with GBS but also in rats with EAN. In rats with EAN, inhibiting P2X7R with BBG alleviated neurological symptoms, reduced demyelination, decreased inflammatory cell infiltration of the peripheral nerves, and improved nerve conduction. BBG also limited the production of pro-inflammatory molecules, down-regulated the expression of P2X7R and NLRP3, and suppressed the differentiation of Th1 and Th17 cells, thus protecting against EAN. These effects collectively contribute to modifying the inflammatory environment and enhancing outcomes in EAN rats. Conclusions: Suppression of P2X7R relieved EAN manifestation by regulating CD4+ T cell differentiation and NLRP3 inflammasome activation. This finding underscores the potential significance of P2X7R as a target for anti-inflammatory treatments, advancing research and management of GBS. [ABSTRACT FROM AUTHOR]

Background: Specific treatment for Guillain–Barre syndrome is based on plasma exchange and intravenous immunoglobulin (IvIg). In developing countries such as Morocco, we are often confronted with constraints in terms of price and availability of substitutes. Comparative studies of these two therapeutic modalities have been conducted particularly in severely extensive forms. Results: Our study compared small-volume plasmapheresis (SVP) with intravenous Immunoglobulin over a nine-year period in the neurology department of the University Hospital Center of Marrakech in terms of efficacy and safety in Moroccan patients with GBS of varying degrees of severity. We included 76 patients who were hospitalized for GBS. Forty-six patients were treated with SVP and 30 were treated with IvIg. The therapeutic choice depended on contraindications, socioeconomic considerations, patient choice, and availability of treatment. The clinical and paraclinical evaluations of the two groups were statistically comparable, including factors that may influence the prognosis (p > 0.05). The efficacy of IvIg and SVP did not show a statistically significant difference except for a longer neurology department stay with plasmapheresis (p < 0.001). This efficacy is evaluated by the evolution of the Hughes and MRC sum scores one month after treatment, length of hospital stay, use of mechanical ventilation and its duration, and mortality rate. Conclusion: The results selected further encourage the use of SVP because of its efficacy and safety, which are comparable to those of IvIg. And the review of the literature confirms our recommendations. [ABSTRACT FROM AUTHOR]

Acute disseminated encephalomyelitis and Guillain–Barré syndrome refer to post-infectious or post-vaccination inflammatory demyelinating disorders of central and peripheral nervous system, respectively. We report the case of a 60-year-old male patient presenting with irritability, gait difficulty, asymmetric quadriparesis (mostly in his left extremities), distal sensory loss for pain and temperature in left limbs, and reduced tendon reflexes in his upper limbs and absent in his lower limbs, following an upper respiratory tract infection, 3 weeks earlier. Brain magnetic resonance imaging revealed abnormal T2 signal and peripherally enhancing lesions in hemispheres, brainstem, and cerebellum. Nerve conduction studies were compatible with acute motor and sensory axonal neuropathy. Serology revealed positive IgM and IgG antibodies for Chlamydia pneumoniae, and he also tested positive for myelin oligodendrocyte glycoprotein (MOG) and sulfatide antibodies. Treatment with intravenous immunoglobulin and methylprednisolone led to clinical and radiological recovery within weeks. Even though several cases of combined central and peripheral demyelination have been reported before, it is the first case report with seropositive anti-sulfatide and anti-MOG acute sensorimotor axonal neuropathy and disseminated encephalitis associated with C. pneumoniae. [ABSTRACT FROM AUTHOR]

To investigate the clinical characteristics of Guillain–Barré syndrome (GBS) in patients with primary Sjögren's syndrome (SS). Records of patients with positive anti-SSA antibodies hospitalized in the Beijing Tiantan Hospital between December 2011 and May 2020 were retrieved. Patients who fulfilled the criteria for diagnosis of GBS and primary SS were included, and their clinical data were analyzed. Among the 785 patients with positive anti-SSA, 52 patients were identified in this study. They were 27 males and 25 females with median age of 59 years old. Besides anti-SSA antibodies, multiple autoantibodies were detected in these patients including antinuclear antibody, anti-Ro52, anti-mitochondrial M2, anti-thyroid peroxidase and anti-thyroglobulin autoantibodies. Preceding infection was reported in 42 patients. Hyporeflexia/areflexia and limbs weakness were the most common manifestation and 35 patients presented cranial nerve injuries. GBS disability score of 3, 4 and 5 was scaled in 28 (53.8%), 15 (28.8%) and 3 (5.8%) patients respectively. Forty-six patients received intravenous immunoglobulin (IVIG) monotherapy, 5 patients were treated by IVIG plus glucocorticoids, and 51 patients improved during hospitalization. The frequency of male gender among the patients with both GBS and primary SS suggests an independent onset of GBS and the co-existence of these autoimmune diseases in patients with multiple autoantibodies. Majority of patients with GBS and primary SS experience benign disease course. [ABSTRACT FROM AUTHOR]

Background: Accumulating evidence on the neurological sequelae of COVID-19 is a serious concern, with patients possibly being at risk of permanent debilitation if not managed appropriately. We aimed to determine the prevalence and pattern of neurological manifestations and diagnostic and therapeutic findings among hospitalized COVID-19 patients consulted with the neurology service for neurological disorders. We conducted a retrospective, observational study at the Golestan Hospital of Ahvaz, Iran, between March 20, 2020, and March 19, 2021. Patients' demographic, clinical, paraclinical, and therapeutic characteristics were extracted from medical records and then subjected to statistical analysis. Results: Overall, 6.7% (157/2340) of COVID-19 patients at Golestan Hospital had a neurological disorder. Most of the patients (90/157) were men, and the mean age of patients was 62.91 ± 91 years. A total of 56.68% of patients (89/157) were SARS-CoV-2 RT-PCR positive. The mean chest CT severity score was 8.26 ± 4.4, ranging from 1 to 19. The most common neurologic disorders were cerebrovascular disease (72.6%), encephalopathy (8.9%), and Guillain–Barre syndrome (6.4%). The CSF SARS-CoV-2 PCR test was positive in one patient with Guillain–Barre syndrome. The in-hospital mortality rate was 43.9%. Definite COVID-19, ICU admission, history of stroke and dementia, and comorbidities were associated with an increased mortality risk in these patients. Conclusions: Patients with COVID-19 can present with serious neurological disorders such as cerebrovascular disease and impaired consciousness, even without typical COVID-19 symptoms. Close monitoring for neurological symptoms may help improve prognosis in hospitalized COVID-19 patients. [ABSTRACT FROM AUTHOR]

Guillain-Barré syndrome (GBS) is a rare immune-related adverse event (irAE) that can occur in solid tumors such as hepatocellular carcinoma, gastric cancer, breast cancer, and colorectal cancer. It is characterized by progressive myasthenia and mild sensory abnormalities. The emergence of immune checkpoint inhibitors (ICIs) has significantly improved cancer patients' life expectancy but can also trigger various irAEs, including GBS. We report a rare case of GBS in a 64-year-old male patient with dual primary tumors of the colon and stomach who received toripalimab and chemotherapy for liver metastases. After five treatments, the patient experienced weakness and numbness in his limbs. Lumbar puncture, electromyography, and other tests confirmed the diagnosis of GBS. Intravenous immunoglobulin (IVIG) and methylprednisolone did not improve the patient's symptoms, but rituximab, which is not a standard regimen for GBS, was effective in eliminating B cells and improving symptoms. Following this, we effectively shifted from a regimen combining immunotherapy and chemotherapy to a targeted therapy regimen, resulting in prolonged patient survival. Currently, limited studies have been undertaken to evaluate the efficacy of rituximab in managing refractory neurological adverse events associated with ICI therapy. Using this case, we reviewed similar cases and formed our views. [ABSTRACT FROM AUTHOR]

Rhabdomyolysis (RM) induced by electric blankets is exceedingly rare, with only three cases identified in our literature review. Both RM and Guillain--Barré syndrome (GBS) present with similar clinical manifestations of myalgia and muscle weakness, posing a potential challenge for accurate diagnosis in clinical settings. This report presents the case of a 22-year-old man who developed RM subsequent to the use of an electric blanket. Despite undergoing plasma exchange and renal replacement therapy, the patient continued to exhibit poor muscle strength in both lower limbs. Subsequent comprehensive evaluation revealed the presence of concurrent GBS. Following a 5-day course of intravenous gamma globulin treatment, the patient experienced rapid recovery of muscle strength and was discharged. Additionally, we reviewed seven cases from the literature of coexistent RM and GBS. This indicated that investigation of the timing of onset of muscle strength decline in RM patients could help to identify potential concurrent neurological or muscular disorders. In cases in which concurrent GBS and RM cannot be definitively ascertained during early hospitalization, prioritizing plasma exchange treatment may lead to improved patient outcomes. [ABSTRACT FROM AUTHOR]

Background and Aims: This study aimed to identify the clinical characteristics and electrodiagnostic subtypes of Guillain‐Barré syndrome (GBS) in Istanbul. Methods: Patients with GBS were prospectively recruited between April 2019 and March 2022 and two electrodiagnostic examinations were performed on each patient. The criteria of Ho et al., Hadden et al., Rajabally et al., and Uncini et al. were compared for the differentiation of demyelinating and axonal subtypes, and their relations with anti‐ganglioside antibodies were analyzed. Results: One hundred seventy‐seven patients were included, 69 before the coronavirus disease 2019 pandemic (April 2019–February 2020) and 108 during the pandemic (March 2020–March 2022), without substantial changes in monthly frequencies. As compared with the criteria of Uncini et al., demyelinating GBS subtype diagnosis was more frequent according to the Ho et al. and Hadden et al. criteria (95/162, 58.6% vs. 110/174, 63.2% and 121/174, 69.5%, respectively), and less frequent according to Rajabally et al.'s criteria (76/174, 43.7%). Fourteen patients' diagnoses made using Rajabally et al.'s criteria were shifted to the other subtype with the second electrodiagnostic examination. Of the 106 analyzed patients, 22 had immunoglobulin G anti‐ganglioside antibodies (14 with the axonal subtype). They had less frequent sensory symptoms (54.5% vs. 83.1%, p = 0.009), a more frequent history of previous gastroenteritis (54.5% vs. 22.9%, p = 0.007), and a more severe disease as compared with those without antibodies. Interpretation: Serial electrodiagnostic examinations are more helpful for accurate subtype diagnosis of GBS because of the dynamic pathophysiology of the disease. We observed no significant increase in GBS frequency during the pandemic in this metropolis. [ABSTRACT FROM AUTHOR]

Background and Aims: Scrub typhus is an endemic disease in the fall season that occurs in a limited number of places known as the Tsutsugamushi Triangle. Peripheral neuropathy is a common complication of scrub typhus. Herein, we encountered several patients with ascending paralysis after scrub typhus infection, who were diagnosed with Guillain‐Barré syndrome (GBS). We aimed to investigate the clinical and laboratory characteristics of patients who developed GBS after scrub typhus. Methods: Patients were retrospectively recruited from six nationwide tertiary centers in South Korea from January 2017 to December 2021. Patients who had been clinically diagnosed with GBS and confirmed to have scrub typhus via laboratory examination and/or the presence of an eschar before the onset of acute limb paralysis were included. The GBS‐associated clinical and electrophysiological characteristics, outcomes, and scrub typhus‐associated features were collected. Results: Of the seven enrolled patients, six were female and one was male. The median time from scrub typhus infection to the onset of limb weakness was 6 (range: 2–14) days. All patients had eschar on their bodies. Four patients (57.1%) were admitted to the intensive care unit and received artificial ventilation for respiratory distress. At 6 months, the median GBS disability score was 2 (range, 1–4) points. Interpretation: Patients with scrub typhus‐associated GBS have a severe clinical presentation and require intensive treatment with additional immunotherapies. Therefore, GBS should be included in the differential diagnosis when peripheral neuropathies develop during scrub typhus treatment. Notably, scrub typhus is associated to GBS. [ABSTRACT FROM AUTHOR]

Immune checkpoint molecules play pivotal roles in the regulation of immune homeostasis. Disruption of the immune checkpoints causes autoimmune/inflammatory as well as malignant disorders. Over the past few years, the immune checkpoint molecules with inhibitory function emerged as potential therapeutic targets in oncological conditions. The inhibition of the function of these molecules by using immune checkpoint inhibitors (ICIs) has brought paradigmatic changes in cancer therapy due to their remarkable clinical benefits, not only in improving the quality of life but also in prolonging the survival time of cancer patients. Unfortunately, the ICIs soon turned out to be a "double‐edged sword" as the use of ICIs caused multiple immune‐related adverse effects (irAEs). The development of inflammatory neuropathies such as Guillain–Barré syndrome (GBS) and Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) as the secondary effects of immunotherapy appeared very challenging as these conditions result in significant and often permanent disability. The underlying mechanism(s) through which ICIs trigger inflammatory neuropathies are currently not known. Compelling evidence suggests autoimmune reaction and/or inflammation as the independent risk mechanism of inflammatory neuropathies. There is a lack of understanding as to whether prior exposure to the risk factors of inflammatory neuropathies, the presence of germline genetic variants in immune function‐related genes, genetic variations within immune checkpoint molecules, the existence of autoantibodies, and activated/memory T cells act as determining factors for ICI‐induced inflammatory neuropathies. Herein, we highlight the available pieces of evidence, discuss the mechanistic basis, and propose a few testable hypotheses on inflammatory neuropathies as irAEs of immunotherapy. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The Zika virus (ZIKV) is a mosquito-borne virus that already poses a danger to worldwide human health. Patients infected with ZIKV generally have mild symptoms like a low-grade fever and joint pain. However, severe symptoms can also occur, such as Guillain-Barré syndrome, neuropathy, and myelitis. Pregnant women infected with ZIKV may also cause microcephaly in newborns. To date, we still lack conventional antiviral drugs to treat ZIKV infections. Marine natural products have novel structures and diverse biological activities. They have been discovered to have antibacterial, antiviral, anticancer, and other therapeutic effects. Therefore, marine products are important resources for compounds for innovative medicines. In this study, we identified a marine natural product, harzianopyridone (HAR), that could inhibit ZIKV replication with EC50 values from 0.46 to 2.63 µM while not showing obvious cytotoxicity in multiple cellular models (CC50 > 45 µM). Further, it also reduced the expression of viral proteins and protected cells from viral infection. More importantly, we found that HAR directly bound to the ZIKV RNA-dependent RNA polymerase (RdRp) and suppressed its polymerase activity. Collectively, our findings provide HAR as an option for the development of anti-ZIKV drugs. [ABSTRACT FROM AUTHOR]

Introduction: Guillain–Barré syndrome associated with Coronavirus-2-related severe acute respiratory syndrome (COV-GBS) occurs as para- or post-infectious forms, depending on the timing of disease onset. In these two forms, we aimed to compare the cerebrospinal fluid (CSF) and serum proinflammatory cytokine profiles to evaluate differences that could possibly have co-pathogenic relevance. Materials and methods: We studied a retrospective cohort of 26 patients with either post-COV-GBS (n = 15), with disease onset occurring > 7 days after SARS-CoV-2 infection, or para-COV-GBS (n = 11), with disease onset 7 days or less. TNF-α, IL-6, and IL-8 were measured in the serum with SimplePlex™ Ella™ immunoassay. In addition to the para-/post-COV-GBS patients, serum levels of these cytokines were determined in those with non-COVID-associated-GBS (NC-GBS; n = 43), paucisymptomatic SARS-CoV-2 infection without GBS (COVID, n = 20), and in healthy volunteers (HV; n = 12). CSF cytokine levels were measured in patients with para-/post-COV-GBS, in those with NC-GBS (n = 29), or with Alzheimer's disease (AD; n = 24). Results: Serum/CSF cytokine levels did not differ in para- vs post-COV-GBS. We found that SARS-CoV-2 infection raises the serum levels of TNF-α, IL-6, and IL-8, as well as an increase of IL-6 (in serum and CSF) and IL-8 (in CSF) in either NC-GBS or COV-GBS than controls. CSF and serum cytokine levels resulted independent one with another. Conclusions: The change of cytokines linked to SARS-CoV-2 in COV-GBS appears to be driven by viral infection, although it has unique characteristics in GBS as such and does not account for cases with para- or post-infectious onset. [ABSTRACT FROM AUTHOR]

Background: Case-reports/series and cohorts of Guillain–Barré syndrome (GBS) associated with COVID-19 vaccination have been reported. Methods: A systematic review and meta-analysis of cohort studies of GBS after COVID-19 vaccination was carried out. Incidence and incidence rate ratio for a number of vaccine doses and risk of GBS, also considering the specific vaccine technology, were calculated in a random-effects model. Results: Of 554 citations retrieved, 518 were discarded as irrelevant. We finally included 15 studies. The random effect model yielded, regardless of the vaccine technology, 1.25 (95%CI 0.21; 2.83) GBS cases per million of COVID-19 vaccine doses, 3.93 (2.54; 5.54) cases per million doses for adenovirus-vectored vaccines and 0.69 (0.38; 1.06) cases per million doses for mRNA vaccines. The GBS risk was 2.6 times increased with the first dose. Regardless of the vaccine technology, the GBS risk was not increased but disaggregating the data it was 2.37 (1.67; 3.36) times increased for adenovirus-vectored vaccines and 0.32 (0.23; 0.47) for mRNA vaccines. Mortality for GBS after vaccination was 0.10 per million doses and 4.6 per GBS cases. Conclusions: Adenovirus-vectored vaccines showed a 2.4 times increased risk of GBS that was about seven times higher compared with mRNA-based vaccines. The decreased GBS risk associated with mRNA vaccines was possibly due to an elicited reduction of infections, including SARS-CoV-2, associated with GBS during the vaccination period. How adenovirus-vectored COVID-19 vaccines may trigger GBS is unclear and further studies should investigate the relationship between vaccine technologies and GBS risk. [ABSTRACT FROM AUTHOR]

Guillain-Barré syndrome is the most common cause of acute flaccid paralysis in children, but several diseases mimic GBS. We aimed to identify and report the clinical pointers and battery of tests required to differentiate Guillain-Barré syndrome from its observed mimics in the pediatric population admitted to our neuro-critical care unit. We conducted a retrospective record analysis of all pediatric patients admitted over ten years from 2008-2018, whose initial presentation was compatible with a clinical diagnosis of GBS. Eighty-three patients were at first treated as GBS, of which seven (8.4%) were found to have an alternate diagnosis--three cases of paralytic rabies, one case each of acute disseminated encephalomyelitis, cervical myeloradiculopathy, neuromyelitis optica, and a case of community-acquired Staphylococcus aureus pneumonia associated sepsis. Neurophysiological and neuro-virological testing, central nervous system imaging, and sepsis screening helped to confirm the alternate diagnosis. Our case series provides knowledge of subtle clinical differences along with the mindful use of diagnostic testing to facilitate the accurate diagnosis of GBS mimics. [ABSTRACT FROM AUTHOR]

Objective: To assess the value of magnetic resonance imaging (MRI) grading scores based on lumbosacral muscle denervation edema in predicting the course of Guillain–Barré syndrome (GBS). Methods: We collected data from 354 GBS patients and developed MRI grading criteria (5‐point scale) based on the transverse area and longitudinal length of lumbosacral edema. Univariate and multivariate logistic regression analyses were conducted to identify factors associated with GBS prognosis among 12 demographic and radiological features. Clinical models and clinical‐MRI models were separately trained and validated by data from Institution 1. External test was performed using data from Institution 2. Differences between the models were assessed using the z‐test. Results: Four clinical factors (sex, albumin cytological dissociation in cerebrospinal fluid, medical research council [MRC] sum score at admission, and MRC sum score at discharge [odds ratio, 0.24–5.15; all p < 0.001]) and MRI grading scores (odds ratio, 2.44; p < 0.001) are independent prognostic factors for GBS patients. The shallow neural network achieved the best prognostic performance both clinical model (accuracy of external test cohort, 83.96%) and clinical‐MRI model (accuracy of external test cohort, 90.56%). A significant difference between clinical and clinical‐MRI model was also found (clinical model vs. clinical‐MRI model, area under the receiver operating curve, 0.84 (95% CI: [0.71, 0.91]) vs. 0.97 (95% CI: [0.86, 0.99]), p < 0.001). Interpretation: The MRI grading scores for muscle denervation edema may serve as a potential prognostic risk factor for GBS. Furthermore, they significantly improve the prognostic performance of standalone clinical model in predicting GBS prognosis. [ABSTRACT FROM AUTHOR]

Aim: Previous studies have shown that the cerebrospinal fluid (CSF) protein level correlates with the number of demyelination criteria in electromyography in adult patients with Guillain-Barré syndrome (GBS), which is a potentially life-threatening postinfectious disease. We aimed to assess the association between CSF protein level, inflammatory markers, and electrophysiological values in the diagnosis of pediatric patients to act quickly in treating GBS. Methods: In this cross-sectional study, thirty-nine children with GBS were retrospectively analyzed from the medical records of patients who were treated as inpatients between 2013 and 2021. Neutrophil-lymphocyte ratio, platelet-lymphocyte ratio, C-reactive protein, uric acid, CSF protein levels, and electrophysiological parameters of the patients on admission were recorded. Hughes disability scores (HDS) were evaluated to determine the severity of disability on admission and at the 3rd month. Results: Cerebrospinal fluid protein was positively correlated with tibial and peroneal motor nerve distal latency (DL) and negatively correlated with tibial and peroneal sensorial nerve conduction velocities (NCV). In the acute inflammatory demyelinating polyneuropathy group, 3rd-month HDS was significantly lower than in the acute motor axonal neuropathy group. A positive correlation was found between first-admission HDS and 3rd-month HDS. There was no significant difference between the electrophysiological subgroups and inflammatory markers. Conclusion: In pediatric GBS patients, well-standardized ranges of the tibial and peroneal motor nerves DL, as well as medial plantar and peroneal superficial NCV, may be sensitive markers. Early rehabilitation programs could prevent disability in immobile patients. [ABSTRACT FROM AUTHOR]

Objective：We aimed to explore the relationship between thyroid autoantibodies and anti ⁃GM1 antibody in Guillain ⁃ Barré syndrome (GBS) patients. Methods：We selected 60 GBS patients who were admitted to Nanjing Drum Tower Hospital from July 2018 to February 2023 and tested for both anti ⁃ thyroid antibodies and Antiganglioside antibody (AGA) levels. According to anti ⁃ thyroid antibodies, the patients were divided into normal group and abnormal group. The clinical characteristics, thyroid function and the proportion of AGA in the two groups were compared. Results：Compared with the normal group, GBS patients in the abnormal group had significantly increased anti ⁃GM1 antibody (Ab) (P < 0.05) and anti ⁃GM2 Ab (P < 0.05), and were accompanied by more severe clinical symptoms (P < 0.05) . Multivariate logistic regression analysis showed that the presence of abnormal anti ⁃ thyroid antibodies［OR=5.184, 95%CI：1.377-19.518, P=0.015］and increased free thyroxine (FT4) levels［OR=1.266, 95%CI：1.009-1.588, P=0.030］ were independent risk factors for increased anti ⁃ GM1 antibody positive rate in GBS patients. Receiver operation characteristic (ROC) curve analysis found that the optimal threshold for thyroperoxidase antibody (TPO ⁃Ab) to predict anti ⁃GM1 Ab positive was 47.9 U/mL, with a sensitivity of 66.7% and a specificity of 77.8%. The optimal threshold for thyroglobulin antibody (Tg⁃Ab) to predict anti ⁃ GM1 Ab positive was 20.0 U/mL, with a sensitivity of 73.3% and a specificity of 73.3% . Conclusion： Our results suggest an association between thyroid autoantibodies and anti ⁃ GM1 antibody in GBS patients, potentially explaining the poorer prognosis of GBS patients with thyroid dysfunction. [ABSTRACT FROM AUTHOR]