Severe Guillain-Barré Syndrome Research Articles

Association of dynamic hepatic metabolism with clinical outcomes in patients with severe Guillain-Barré syndrome: A prospective cohort study from multi-centers in China.

Little is known about the ability of serological biomarkers to monitor clinical outcomes in patients with Guillain-Barré syndrome (GBS). The objective of this study was to determine the associations of liver function, easily available and convenient biomarkers, with the clinical course and outcome of severe GBS in patients. A prospective data collection was conducted in a cohort of 343 GBS patients from multi-centers between September 2019 and December 2023. Serum samples were obtained at four-time points for mechanical ventilation (MV) patients and two-time points for non-MV patients. The primary endpoint was the need for MV during hospitalization, while secondary outcomes included the ability to walk independently and the mortality at 26-week follow-up. (i) A total of 208 patients were eligible, of whom 50 required MV with a median (interquartile range) ventilation duration of 15 (8-27) days. (ii) Hypohepatia, as evidenced by reduced total protein (OR 0.913 [95% CI 0.862-0.967]) and albumin (0.775 [0.679-0.884]) 1 week after treatment, along with raised liver enzymes (2.732 [1.007-7.413]), was associated with the risk of MV after adjusting for confounders. (iii) After 26-week follow-up, patients with hypohepatia were less likely to regain independent walking and exhibited higher mortality in survival analysis (all log-rank p < .05). (iv) In a cross-sectional study spanning up to 4 years of follow-up, patients with prolonged MV (≥15 days) experienced a longer time to regain independent ambulation than those with shorter MV (167 [46-316] vs. 69 [24-106], p = .036). However, no relationships between liver function and prolonged MV were revealed. Dynamically monitoring hepatic metabolism and promptly adjusting, it can aid the improvement of GBS in patients.

Efficacy and safety of eculizumab in Guillain-Barré syndrome: A phase 3, multicenter, double-blind, randomized, placebo-controlled clinical trial.

Guillain-Barré syndrome (GBS) is an acute, self-limited, immune-mediated peripheral neuropathy. Current treatments for GBS include intravenous immunoglobulin (IVIg) and plasma exchange, which may not sufficiently benefit severely affected patients. This study evaluated the efficacy and safety of eculizumab add-on therapy to IVIg (standard-of-care treatment) in patients with severe GBS. This phase 3, multicenter, double-blind, randomized, placebo-controlled clinical trial (NCT04752566), enrolled Japanese adults (age ≥ 18 years) with severe GBS (Hughes functional grade [FG] score FG3 or FG4/FG5 within 2 weeks of onset of GBS). Participants were randomized 2:1 to receive intravenous infusion of eculizumab or placebo (once weekly for 4 weeks) with IVIg treatment with 20 weeks of follow-up. Primary efficacy endpoint was the time to first reach FG score ≤1 (able to run). Key secondary endpoints were proportion of participants achieving FG ≤1 at weeks 8 and 24 and FG improvement ≥3 at week 24. Pharmacodynamic analysis of serum free C5 concentration over time was performed. Safety was evaluated. The analysis included 57 participants (eculizumab, n = 37; placebo, n = 20). Primary endpoint was not achieved (hazard ratio, 0.9; 95% CI, 0.45-1.97; p = .89). Key secondary endpoints did not reach statistical significance. Serum C5 concentration was reduced by 99.99% at 1 h postdose and sustained to week 5 but returned to baseline at the end of follow-up period. No new safety signals for eculizumab were identified. Although well tolerated, eculizumab treatment did not show significant effects on motor function recovery compared to placebo in patients with GBS.

Management of guillain-barré syndrome patients with type 2 respiratory failure in RSUD Gambiran

Guillain-Barré syndrome (GBS) is an autoimmune disorder that affects the peripheral nervous system. Most patients with GBS are clinically characterized by tetraplegia with or without sensory disturbances. In GBS this is due to a hyperreactive immune response including the release of anti-ganglioside antibodies, the formation of antibody-dependent immune complexes, and an increase in macrophages that cause demyelination and axonal degeneration. In severe cases GBS can appear clinically progressive with respiratory muscle involvement requiring mechanical ventilation and treatment in the intensive care unit. The patient we report is a 24 year old man with clinical type 2 respiratory failure with suspicion of GBS who was initially examined at the RSUD Kilisuci, then referred to the RSUD Gambiran and treated in the ICU at the RSUD Gambiran and received immunomodulatory therapy in the form of plasma exchange (PE). The patient was treated for 10 days in the ICU at RSUD Gambiran with the outcome being that the patient was able to move to the ward.

The clinical significance of systemic immune-inflammation index and platelet/neutrophil to lymphocyte ratio in Guillain-Barré syndrome

ObjectiveSystemic immune-inflammation index (SII) and platelet/neutrophil to lymphocyte ratio (P/NLR) are two novel hematological inflammatory indices, this study invested the role of SII and P/NLR in Guillain-Barré syndrome (GBS). MethodsA total of 115 GBS patients and 120 healthy controls were enrolled in this retrospective study, SII and P/NLR were calculated from the value of complete blood counts. The Hughes Functional Grading Scale (HFGS) score on admission and at discharge was used to evaluate the severity and short-term outcome of GBS. The level of SII and P/NLR was compared between GBS patients and healthy controls, and the correlation between SII, P/NLR, and GBS’s severity as well as poor short-term outcome was analyzed. ResultsIncreased SII (p < 0.001) and decreased P/NLR (p < 0.001) were observed in patients with GBS and the level of SII (p = 0.689) and P/NLR (p = 0.879) was not different between GBS subtypes. Patients with severe GBS and poor short-term outcomes had higher levels of SII and lower levels of P/NLR (p < 0.05), SII was positively correlated and P/NLR was negatively correlated with the HFGS score on admission and at discharge (p < 0.05). To predict severe GBS, the optimal cutoff value of SII was 620.87 (AUC0.633, sensitivity 60.8 % and specificity 60.9 %, p = 0.014), the optimal cutoff value of P/NLR was 53.11 (AUC0.635, sensitivity 45.1 %, specificity 82.8 %, p = 0.013). The SII level of 620.87 was found to be optimal predictive cutoff value for the poor short-term outcome of GBS (AUC 0.728, sensitivity of 82.6 %, specificity of 59.8 %, p = 0.001), the P/NLR level of 62.80 was found to be optimal predictive cutoff value for the poor short-term outcome of GBS (AUC 0.669, sensitivity 60.9 %, specificity 71.7 %, p = 0.012). Both SII>620.87 (p = 0.005) and P/NLR<53.11 (p = 0.002) were independent risk factors for severe GBS, and SII>620.87 (p = 0.035) was independently associated with the poor short-term outcome of GBS. ConclusionSII and P/NLR may be useful biomarkers to reflect GBS patients’ severity and short-term outcome.

Current status of Guillain-Barré syndrome (GBS) in China: a 10-year comprehensive overview.

Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy; a disease involving the peripheral nervous system which is the most common cause of acute flaccid paralysis worldwide. So far, it is still lack of a comprehensive overview and understanding of the national epidemiological, clinical characteristics, and the risk factors of GBS in China, as well as differences between China and other countries and regions in these respects. With the global outbreak of the coronavirus disease 2019 (COVID-19), an epidemiological or phenotypic association between severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection and GBS has attracted great attention. In this review, we outlined the current clinical data of GBS in China by retrieving literature, extracting and synthesizing the data of GBS in China from 2010 to 2021. Besides, we compared the characteristics of epidemiology, preceding events and clinical profiles of GBS between China and other countries and regions. Furthermore, in addition to conventional intravenous immunoglobulin (IVIG) and plasma exchange (PE) therapy, the potential therapeutic effects with novel medications in GBS, such as complement inhibitors, etc., have become the research focus in treatments. We found that epidemiological and clinical findings of GBS inChina are approximately consistent with those in the International GBS Outcome Study (IGOS) cohort. We provided an overall picture of the present clinical status of GBS in China and summarized the global research progress of GBS, aiming to further understand the characteristics of GBS and improve the future work of GBS worldwide, especially in countries with the middle and low incomes.

Postcovid Guillain-Barré syndrome with severe course - case series two patients including clinical evaluation of smell and examination of olfactory event-related potentials (OERPs).

We report a case series two patients of Guillain-Barré syndrome (GBS) associated with previous COVID-19 that both patients survived. GBS is an immune-mediated disease that affects peripheral nerves and can cause life-threatening complications. In both cases (53-year-old female and 59-year-old male) with severe GBS with complications, the smell of sense was investigated subjectively using Sniffin' sticks identification tests and objectively using objective olfactometry by the evaluation of olfactory event-related potentials (OERPs). Both patients had good results of the subjective Sniffin' sticks identification test without patholgical findings. Results of objective examination of OERPs: the P2-N1 wave complex was equipotent. No olfactory disturbance could be detected in either case, OERPs were plentiful in both cases. The presentation of a case series two patients of post-covid GBS are an example of one of the many complications of COVID-19 that can cause prolonged recovery. Despite the severe course of GBS and the long recovery time, both patients returned to normal life. An expanded prospective study is planned for the future to investigate post-covid olfactory impairment. The prevalence of GBS associated with COVID-19 is still unknown but it is evident that both mild and severe forms of GBS have been described in patients.

Tumor Necrosis Factor-α, CD1A and CD1E Genetic Polymorphisms in Guillain-Barré Syndrome: A Study from India.

Guillain-Barré Syndrome (GBS) is an acute acquired autoimmune inflammatory disorder of peripheral nerves and roots. The pathogenesis is essentially an aberrant post-infectious immune response in a genetically susceptible host milieu. Single nucleotide polymorphisms (SNP) in genes encoding the inflammatory mediators like TNF-α, CD1A and CD1E can influence their expression and level and the susceptibility and clinical course of disease in GBS. We tried to study the susceptibility of single nucleotide polymorphisms of TNF-α and CD1 genes in Guillain-Barré Syndrome in Indian population and determine the association in terms of genotype, allele and haplotype distribution along with individual subtype, severity and clinical outcome. In this case-control study, we investigated the single nucleotide polymorphism pattern in the promoter region of TNF-α (-308 G/A), TNF-α (-863C/A), CD1A and CD1E genes using real-time polymerase chain reaction in 75 GBS patients and analysed in comparison with 75 age and sex-matched healthy controls. The findings revealed that the allelic distribution of TNF-α (-308 G/A) *A allele was associated with GBS (P value 0.04, Odds Ratio 2.03, 95% Confidence Interval 1.01-4.07). There was no association found with genotype, haplotype combination and other allele distribution for GBS in the study. CD1A and CD1E SNPs did not reveal any susceptibility for GBS. The subtype analysis did not reveal any statistical significance, except for CD1A *G allele with AMAN subtype (P value 0.026). The haplotypic combinations and mutant allele of TNF-α (-308 G/A), TNF-α (-863C/A), CD1A and CD1E were significantly associated with severe GBS in the study. However, there was no association of any SNP for mortality and survival of GBS in the study. TNF-α (-308 G/A)*A allele might confer genetic susceptibility for GBS in Indian population. CD1 genetic polymorphism could not be considered for susceptibility to GBS. TNF-α and CD1 genetic polymorphism did not affect mortality in GBS.

Use of functional magnetic resonance imaging to assess cognition and consciousness in severe Guillain-Barré syndrome

ObjectiveFunctional neuroimaging may provide a viable means of assessment and communication in patients with Guillain-Barré Syndrome (GBS) mimicking the complete locked-in state. Functional neuroimaging has been used to assess residual cognitive function and has allowed for binary communication with other behaviourally non-responsive patients, such as those diagnosed with unresponsive wakefulness syndrome. We evaluated the potential application of functional neuroimaging using a clinical-grade scanner to determine if individuals with severe GBS retained auditory function, command following, and communication. MethodsFourteen healthy participants and two GBS patients were asked to perform motor imagery and spatial navigation imagery tasks while being scanned using functional magnetic resonance imaging. The GBS patients were also asked to perform additional functional neuroimaging scans to attempt communication. ResultsThe motor imagery and spatial navigation task elicited significant activation in appropriate regions of interest for both GBS patients, indicating intact command following. Both patients were able to use the imagery technique to communicate in some instances. Patient 1 was able to use one of four communication tasks to answer a question correctly. Patient 2 was able to use three of seven communication tasks. However, two questions were incorrectly answered while a third was non-verifiable. ConclusionsGBS patients can respond using mental imagery and these responses can be detected using functional neuroimaging. Furthermore, these patients may also be able to use mental imagery to provide answers to ‘yes’ or ‘no’ questions in some instances. We argue that the most appropriate use of neuroimaging-based communication in these patients is to allow them to communicate wishes or preferences and assent to previously expressed decisions, rather than to facilitate decision-making.

Long-Term Neurological, Behavioral, Functional, Quality of Life, and School Performance Outcomes in Children With Guillain-Barré Syndrome Admitted to PICU

BackgroundMost children with Guillain-Barré syndrome (GBS) recover but may suffer from long-term sequelae, interfering with development and quality of life. Owing to the lack of published data, we aimed to assess the long-term neurological, behavioral, functional, quality of life, and school performance outcomes them. MethodsDesign: Cross-sectional observational. Setting: Pediatric intensive care unit. Patients: Children, aged one to 12 years, with GBS admitted over five years (July 2012 to June 2017) were enrolled during one year (July 2017 to June 2018). These children were assessed for the following outcomes: neurological (Hughes disability score, Pediatric Cerebral Performance Category [PCPC], Pediatric Overall Performance Category [POPC], and Glasgow Outcome Scale-Extended Pediatric version [GOS-E Peds] scales), behavioral (Childhood Psychopathology Measurement Schedule [CPMS]), functional (Vineland Social Maturity Scale [VSMS]-Indian Adaptation), quality of life (Pediatric Quality of Life [PedsQL]), and school performance (Parent-Directed Questionnaire). ResultsEighty children were enrolled after a median of 3.0 (1.3-4.2) years from discharge. The majority (95%) had favorable neurological recovery (Hughes disability score 0 to 1). Favorable outcome was noted in 95% of children on PCPC, 87.5% on POPC, 60% on GOS-E Peds, 86.2% on CPMS, 92.5% on VSMS, and 98% on PedsQL. The majority (97.5%) of childre were attending schools, and 57.7% had satisfactory school performance. The presence of quadriparesis at admission, mechanical ventilation, tracheostomy requirement, poor ambulatory status at discharge, and longer pediatric intensive care unit and hospital stay predicted unfavorable neurological outcome on different tools. Absence of quadriparesis at admission and no requirement of mechanical ventilation predicted a favorable result on all outcome measures. ConclusionsOn long-term follow-up, most children with severe GBS showed favorable neurological, behavioral, functional, and quality of life outcomes. Severe clinical presentation and prolonged intensive care unit stay predict poor long-term outcome.

Risk factors for the severity of Guillain-Barr\xe9 syndrome and predictors of short-term prognosis of severe Guillain-Barr\xe9 syndrome

Guillain-Barré syndrome (GBS) is a neurological disorder characterized by paralysis. Identifying the severity, appropriate therapeutic method, and prognosis of GBS at an early stage is highly important. This study aimed to investigate the modifiable risk factors for the severity of GBS and consequent need for mechanical ventilation (MV) and to identify clinical predictive factors for poor short-term outcomes of severe GBS. 155 GBS patients who were admitted to the Affiliated Yantai Yuhuangding Hospital of Qingdao University during 2014–2020 were enrolled. Demographic, clinical, therapeutic and evolutionary data were collected and were then analyzed using univariate and multivariate regression analyses. Our analytic data demonstrated that the significant clinical predictors of severe GBS were recent history of surgery, older age, cranial nerve impairment, and elevated levels of liver enzymes (p < 0.05). Furthermore, autonomic dysfunction, lower Medical Research Council (MRC) score at nadir, and elevated levels of liver enzymes were significantly associated with MV for severe GBS (p < 0.05), and lower MRC score at nadir and autonomic dysfunction remained significant predictors of MV in severe GBS (p < 0.05). Lastly, recent history of surgery, lower MRC score at admission and at nadir, requirement for MV, and pneumonia during hospitalization were significantly associated with the short-term outcome of severe GBS and that lower MRC score at admission and need for MV were confirmed to be predictors of poor short-term prognosis (p < 0.05). Of note, this study suggested that recent history of surgery is a predictor of severity in GBS patients and is associated with the poor short-term prognosis of severe GBS.

Intensive Care and Treatment of Severe Guillain-Barré Syndrome.

Guillain–Barré syndrome (GBS) is an acute polyneuropathy mostly characterized by acute flaccid paralysis with or without sensory/autonomous nerve dysfunction. Current immuno therapies including intravenous immunoglobulin (IVIg), plasma exchange (PE), and newly developed biological drugs benefit patients by alleviating hyperreactive immune responses. Up to 30% of patients develop respiratory failure during hospitalization and require mechanical ventilation and intensive care. Immunotherapies, mechanical ventilation, supportive care, and complication management during the intensive care unit (ICU) stay are equally emphasized. The most important aspect of intensive care and treatment of severe GBS, that is, mechanical ventilation, has been extensively reviewed elsewhere. In contrast to immunotherapies, care and treatment of GBS in the ICU setting are largely empirical. In this review, we intend to stress the importance of intensive care and treatment, other than mechanical ventilation in patients with severe GBS. We summarize the up-to-date knowledge of pharmacological therapies and ICU management of patients with severe GBS. We aim to answer some key clinical questions related to the management of severe GBS patients including but not limited to: Is IVIg better than PE or vice versa? Whether combinations of immune therapies benefit more? How about the emerging therapies promising for GBS? When to perform tracheal intubation or tracheostomy? How to provide multidisciplinary supportive care for severe cases? How to avert life-threatening complications in severe cases?

Acute Polyneuropathy in an Outpatient Context During the SARS-CoV-2 Pandemic: A Brief Case Serie Report.

Guillain-Barré syndrome (GBS) is a well-established complication of infectious disease. So it is not surprising that several cases have been described during the actual SARS-CoV-2 infection pandemic. Most of the descriptions are patients suffering a severe GBS in the setting of a severe SARS-CoV-2 infection. We described five patients with mild forms of COVID-19. After 2–4 weeks, these patients develop mild neurological symptoms. The clinical and neurophysiological studies supported a diagnosis of an acute polyneuropathy. Symptoms resolved without specific treatment and primary care physicians managed all patients outpatiently. Mild SARS-CoV-2 infection could associate mild neurological complications too. So patients complaining about mild neurological symptoms, a SARS-CoV-2 infection may be excluded.Supplementary InformationThe online version contains supplementary material available at 10.1007/s42399-021-00855-x.

Relationship of Glycemic Status with Disease Severity in Guillain-Barré Syndrome

Background: Guillain-Barré syndrome (GBS) is an acute autoimmune polyneuroradiculopathy characterized by flaccid paralysis which may lead to respiratory failure requiring intensive care.&#x0D; Objective: The purpose of the present study was to explore the relationship between the fasting plasma glucose (FPG), hemoglobin A1c (HbA1c) and disease severity of GBS patients who are not known to have DM.&#x0D; Methodology: This cross-sectional study included adult GBS patients without having DM [age 35 (22-48) years, median (interqurtile range, IQR); 39 male 22 female] who were admitted to Neurology department, National Institute of Neurosciences and Hospital, Dhaka, Bangladesh from July 2018 to June 2019. Demographics, clinical data were noted and FPG, HbA1c were measured. Disease severity were assessed by the GBS disability scale ranging from 0 to 6 with increasing score reflecting increased disability.&#x0D; Results: Patients with more severe GBS (disability score ≥4, unable to walk) had higher frequency of elevated FPG &gt;5.5 mmol/L (61.2%; 30/49) in comparison to those with less severe GBS (disability score ≤3, able to walk; FPG &gt;5.5 mmol/L in 16.7%, 2/12; p=0.006). But distribution of HbA1c category was not different across the groups (disability score ≥4 vs. ≤3: HbA1c &lt;5.7: 40% vs. 58%; 5.7-6.4: 50% vs. 25%; &gt;6.4: 10% vs. 17%; p=0.296). Participants with elevated FPG were elder [elevated vs. normal FPG: 40 (28-54) vs. 25 (19-43) years; median (IQR), p=0.012] and had higher CSF glucose (p=0.002) than those with normal FPG, but there was no difference in respct of gender, MRC sum score, requirement of assisted ventilation, CSF protein, GBS subtypes and duration of hospital stay (p=not significant for all).&#x0D; Conclusions: Patients with severe GBS have higher frequency of elevated FPG but not HbA1c. An acute change in glucose metabolism may occur in GBS which needs further study.&#x0D; Journal of National Institute of Neurosciences Bangladesh, 2020;6(2): 96-100

Ankle-Foot Orthoses Improve Standing and Walking in Severe Guillain-Barré Syndrome: A Case Report

ABSTRACT Introduction Ankle-foot orthoses (AFOs) are a well-recognized aspect of the rehabilitation of walking in Guillain-Barré syndrome (GBS), but there is little evidence for the effectiveness of orthoses in GBS and little information on which orthotic designs should be used. This study reports on the use of flexible, semirigid, and rigid AFOs in a subject with GBS. Materials and Methods This study presents data from a single subject in the first 9 months after severe GBS. Outcome measures used include walking speed, timed independent standing, and self-reported confidence in standing and walking. Results Improvements in standing and walking were demonstrated with the use of custom rigid AFOs. Later on, similar performance was achieved using less rigid prefabricated AFOs. Self-reported confidence remained higher using custom rigid AFOs. Conclusion In one subject with severe GBS, different designs of AFOs improved the performance of standing and walking. Custom rigid AFOs may be a more appropriate prescription in the presence of severe weakness.

Axonal degeneration in Guillain-Barré syndrome: a reappraisal.

The aim of this review was to analyse the pathophysiology of axonal degeneration in Guillain-Barré syndrome (GBS) with emphasis on early stages (≤ 10days after onset). An overview of experimental autoimmune neuritis (EAN) models is provided. Originally GBS and acute inflammatory demyelinating polyneuropathy were equated, presence of axonal degeneration being attributed to a "bystander" effect. Afterwards, primary axonal GBS forms were reported, designated as acute motor axonal neuropathy/acute motor-sensory axonal neuropathy. Revision of the first pathological description of axonal GBS indicates the coexistence of active axonal degeneration and demyelination in spinal roots, and pure Wallerian-like degeneration in peripheral nerve trunks. Nerve conduction studies are essential for syndrome subtyping, though their sensitivity is scanty in early GBS. Serum markers of axonal degeneration include increased levels of neurofilament light chain and presence of anti-ganglioside reactivity. According to nerve ultrasonographic features and autopsy studies, ventral rami of spinal nerves are a hotspot in early GBS. In P2-induced EAN models, the initial pathogenic change is inflammatory oedema of spinal roots and sciatic nerve, which is followed by demyelination, and Wallerian-like degeneration in nerve trunks possessing epi-perineurium; a critical elevation of endoneurial fluid pressure is a pre-requisite for inducing ischemic axonal degeneration. Similar lesion topography may occur in GBS. The repairing role of adaxonal Schwann cytoplasm in axonal degeneration is analysed. A novel pathophysiological mechanism for nerve trunk pain in GBS, including pure motor forms, is provided. The potential therapeutic role of intravenous boluses of methylprednisolone for early severe GBS and intractable pain is argued.

Pharmacological treatment other than corticosteroids, intravenous immunoglobulin and plasma exchange for Guillain-Barré syndrome.

Plasma exchange and intravenous immunoglobulin, but not corticosteroids, are beneficial in Guillain-Barré syndrome (GBS). The efficacy of other pharmacological agents is unknown. This review was first published in 2011 and previously updated in 2013, and 2016. To assess the effects of pharmacological agents other than plasma exchange, intravenous immunoglobulin and corticosteroids for GBS. On 28 October 2019, we searched the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, and Embase for treatments for GBS. We also searched clinical trials registries. We included all randomised controlled trials (RCTs) or quasi-RCTs of acute GBS (within four weeks from onset) of all types and degrees of severity, and in individuals of all ages. We discarded trials that investigated only corticosteroids, intravenous immunoglobulin or plasma exchange. We included other pharmacological treatments or combinations of treatments compared with no treatment, placebo or another treatment. We followed standard Cochrane methodology. We found six trials of five different interventions eligible for inclusion in this review. The trials were conducted in hospitals in Canada, China, Germany, Japan and the UK, and included 151 participants in total. All trials randomised participants aged 16 years and older (mean or median age in the trials ranged from 36 to 57 years in the intervention groups and 34 to 60 years in the control groups) with severe GBS, defined by the inability to walk unaided. One trial also randomised patients with mild GBS who were still able to walk unaided. We identified two new trials at this update.The primary outcome measure for this review was improvement in disability grade four weeks after randomisation. Four of six trials had a high risk of bias in at least one respect. We assessed all evidence for the outcome mean improvement in disability grade as very low certainty, which means that we were unable to draw any conclusions from the data. One RCT with 19 participants compared interferon beta-1a (IFNb-1a) and placebo. It is uncertain whether IFNb-1a improves disability after four weeks (mean difference (MD) -0.1; 95% CI -1.58 to 1.38; very low-certainty evidence). A trial with 10 participants compared brain-derived neurotrophic factor (BNDF) and placebo. It is uncertain whether BDNF improves disability after four weeks (MD 0.75; 95% CI -1.14 to 2.64; very low-certainty evidence). A trial with 37 participants compared cerebrospinal fluid (CSF) filtration and plasma exchange. It is uncertain whether CSF filtration improves disability after four weeks (MD 0.02; 95% CI -0.62 to 0.66; very low-certainty evidence). One trial that compared the Chinese herbal medicine tripterygium polyglycoside with corticosteroids with 43 participants did not report the risk ratio (RR) for an improvement by one or more disability grade after four weeks, but did report improvement after eight weeks. It is uncertain whether tripterygium polyglycoside improves disability after eight weeks (RR 1.47; 95% CI 1.02 to 2.11; very low-certainty evidence). We performed a meta-analysis of two trials comparing eculizumab and placebo with 41 participants. It is uncertain whether eculizumab improves disability after four weeks (MD -0.23; 95% CI -1.79 to 1.34; very low-certainty evidence). Serious adverse events were uncommon in each of the trials and evidence was graded as either low or very low. It is uncertain whether serious adverse events were more common with IFNb-1a versus placebo (RR 0.92, 95% CI 0.23 to 3.72; 19 participants), BNDF versus placebo (RR 1.00, 95% CI 0.28 to 3.54; 10 participants) or CSF filtration versus plasma exchange (RR 0.13, 95% CI 0.01 to 2.25; 37 participants). The trial of tripterygium polyglycoside did not report serious adverse events. There may be no clear difference in the number of serious adverse events after eculizumab compared to placebo (RR 1.90, 0.34 to 10.50; 41 participants). We found no clinically important differences in any of the outcome measures selected for this review in any of the six trials. However, sample sizes were small and therefore clinically important benefit or harm cannot be excluded. All six RCTs were too small to exclude clinically important benefit or harm from the assessed interventions. The certainty of the evidence was low or very low for all interventions and outcomes.

Diagnosis and treatment of Guillain-Barré Syndrome in childhood and adolescence: An evidence- and consensus-based guideline.

This evidence- and consensus-based practical guideline for the diagnosis and treatment of Guillain-Barré Syndrome (GBS) in childhood and adolescence has been developed by a group of delegates from relevant specialist societies and organisations; it is the result of an initiative by the German-Speaking Society of Neuropediatrics (GNP), and is supported by the Association of Scientific Medical Societies (AWMF, Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften). A systematic analysis of the literature revealed that only a few adequately-controlled studies exist for this particular age group, while none carries a low risk of bias. For this reason, the diagnostic and therapeutic recommendations largely rely on findings in adult patients with GBS, for which there are a higher number of suitable studies available. Consensus was established using a written, multi-step Delphi process. A high level of consensus could be reached for the crucial steps in diagnosis and treatment. We recommend basing the diagnostic approach on the clinical criteria of GBS and deriving support from CSF and electrophysiological findings. Repetition of invasive procedures that yield ambiguous results is only recommended if the diagnosis cannot be ascertained from the other criteria. For severe or persistently-progressive GBS treatment with intravenous immunoglobulin (IVIG) is recommended, whereas in cases of IVIG intolerance or inefficacy we recommended treatment with plasmapheresis. Corticosteroids are ineffective for GBS but can be considered when acute onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) is suspected due to a prolonged disease course. The full German version of the Guideline is available on the AWMF website (https://www.awmf.org/leitlinien/detail/ll/022-008.html).

The prognosis and prognostic factor of Guillain-Barré Syndrome

Guillain-Barré Syndrome (GBS) is an acute monophasic immune-mediated neuropathy, generally considered to be of good prognosis. However, 15-20% of GBS patients cannot walk independently at six months from onset. Poor prognostic factors for long-term functional disability included old age, preceding diarrhea, muscle weakness on admission and on day 7 from admission, severe GBS disability score at two weeks from admission and IgG antibody against GD1a/GD1b ganglioside complex. Factors related with requirement of mechanical ventilation included the time from onset to admission <7 days, muscle weakness on admission, facial and/or bulbar weakness and IgG antibody against GQ1b. Recently modified Erasmus GBS outcome score (mEGOS) and Erasmus GBS respiratory insufficiency score (EGRIS) were reported as prognostic factors for the long-term functional disability and respiratory insufficiency. Those were designed on Dutch patients. The usefulness of these tools in Japan or other countries remained unknown. The authors validated mEGOS and EGRIS on Japanese GBS patients in Japanese GBS outcome study, which revealed that these tools were also adaptable on Japanese GBS patients. To identify clinical and biological factors of GBS in more detail, such a large scale prospective study as International GBS outcome study (IGOS) is warranted.

Prolonged Ventilatory Support for Patients Recovering From Guillain-Barré Syndrome.

Recovery from Guillain-Barré syndrome (GBS) may be protracted, and patients may need prolonged ventilatory support. We present clinical data from a tertiary referral weaning center managing patients with GBS requiring prolonged ventilatory support. A retrospective review of patients managed in a 34-bed specialist ventilator weaning facility in London, United Kingdom, between 2006 and 2017. Data including demographics, initial presentation, and ventilatory support were collected. Functional recovery and outcome data were collected between 12 months and 3 years following disease onset. Twenty-nine patients with severe GBS requiring prolonged ventilation were included. In several patients, coexisting conditions or complications affected the course. Seventy-six percent (n = 22) were successfully weaned from invasive ventilation with a median time to tracheostomy decannulation of 193 days (range: 49-527 days). Use of noninvasive ventilation (NIV), as part of the weaning program, was applied in 59% (13/22), with 14% (3/22) requiring long-term nocturnal NIV. Twenty-four percent (7/29) were not decannulated, with 14% (4/29) supported on long-term invasive ventilation. Forty-five percent (10/22) weaned from invasive ventilation were able to achieve short distance-assisted ambulation. Mortality at 36 months was 17% (5/29), with 3 of these deaths occurring in patients invasively ventilated during their acute admission. GBS with severe respiratory muscle weakness and bulbar dysfunction may require prolonged invasive ventilation. However, there is potential for complete weaning from invasive mechanical ventilatory support with associated function recovery. These data highlight the importance of maintaining ongoing support and rehabilitation for patients with GBS requiring prolonged ventilation.

Successful treatment of a 12-year-old boy with Guillain-Barré syndrome requiring tracheostomy due to respiratory muscle paralysis: A case report.

Childhood Guillain-Barré syndrome (GBS) occasionally leads to respiratory failure early after onset, requiring long-term ventilation management after tracheal intubation. However, patients requiring tracheostomy management are rare. In the present study, a case of a 12-year-old boy with GBS who required artificial respiration management due to rapid progression of respiratory muscle paralysis is reported. Intravenous immunoglobulin (IVIg) and pulse steroid therapy were provided; however, both were ineffective and tracheostomy was necessary 26 days after onset. A second course of IVIg and pulse steroid therapy was administered on day 34. With continued rehabilitation, the patient was able to walk long distances on day 74 and was subsequently discharged on day 89. In cases of severe GBS, when IVIg and pulse steroid therapy do not improve the respiratory muscle strength of the patient, early tracheostomy may improve the patient's quality of life during artificial respiration management.