Guillain-Barré Syndrome Outcome Research Articles

Timing of intravenous immunoglobulin treatment and outcome in Guillain-Barré syndrome: Is time nerve?

Despite treatment, a considerable proportion of patients with Guillain-Barré syndrome (GBS) experience poor recovery, highlighting a therapeutic need. There is a lack of evidence that treatment timing affects recovery. This study aims to investigate the effects of intravenous immunoglobulin (IVIg) timing on disability and speed of recovery in GBS. We performed a retrospective study of 136 IVIg-treated GBS patients admitted to two Korean centers between 2010 and 2021. We analyzed the effect of time to IVIg on the GBS disability scale (GBS-DS) and the degree of improvement from nadir (∆GBS-DS) at 1, 3, 6, and 12 months, as well as the time to regain the ability to walk or run unaided. Time to IVIg was treated either as a continuous variable or categorized into 1-week intervals to explore critical time windows. Known prognostic factors, the modified Erasmus GBS Outcome Scores on admission and pre-treatment serum albumin levels were adjusted as covariates. Shorter time to IVIg was independently associated with better GBS-DS, greater ∆GBS-DS, and shorter time to walk or run unaided at all time points. The therapeutic effect of IVIg was notably diminished when administered beyond the first 2 weeks of onset. Our study highlights the timing of IVIg as a modifiable prognostic factor in GBS. The earlier IVIg is initiated, the better the outcomes, with the ideal time window being within the first 2 weeks. These findings underscore the importance of prompt diagnosis and early intervention to optimize recovery in GBS patients.

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.

Electrodiagnostic subtyping in Guillain-Barré syndrome patients in the International Guillain-Barré Outcome Study.

Various electrodiagnostic criteria have been developed in Guillain-Barré syndrome (GBS). Their performance in a broad representation of GBS patients has not been evaluated. Motor conduction data from the International GBS Outcome Study (IGOS) cohort were used to compare two widely used criterion sets and relate these to diagnostic amyotrophic lateral sclerosis criteria. From the first 1500 patients in IGOS, nerve conduction studies from 1137 (75.8%) were available for the current study. These patients were classified according to nerve conduction studies criteria proposed by Hadden and Rajabally. Of the 1137 studies, 68.3% (N = 777) were classified identically according to criteria by Hadden and Rajabally: 111 (9.8%) axonal, 366 (32.2%) demyelinating, 195 (17.2%) equivocal, 35 (3.1%) inexcitable and 70 (6.2%) normal. Thus, 360 studies (31.7%) were classified differently. The areas of differences were as follows: 155 studies (13.6%) classified as demyelinating by Hadden and axonal by Rajabally; 122 studies (10.7%) classified as demyelinating by Hadden and equivocal by Rajabally; and 75 studies (6.6%) classified as equivocal by Hadden and axonal by Rajabally. Due to more strictly defined cutoffs fewer patients fulfilled demyelinating criteria by Rajabally than by Hadden, making more patients eligible for axonal or equivocal classification by Rajabally. In 234 (68.6%) axonal studies by Rajabally the revised El Escorial (amyotrophic lateral sclerosis) criteria were fulfilled; in axonal cases by Hadden this was 1.8%. This study shows that electrodiagnosis in GBS is dependent on the criterion set utilized, both of which are based on expert opinion. Reappraisal of electrodiagnostic subtyping in GBS is warranted.

Risk factors and outcome of hyponatremia in patients with Guillain–Barré syndrome

The objective of the present study was to evaluate the risk factors and outcomes associated with hyponatremia in patients with Guillain-Barré syndrome (GBS). We retrospectively studied 80 consecutive patients with GBS who visited our hospital and compared clinical, laboratory, and electrophysiological findings of patients with and without hyponatremia. Disability was evaluated using the Hughes grading system. Of the 80 patients, 18 (23%) had hyponatremia. Hyponatremia was significantly associated with older age (P = 0.003), urinary retention (P < 0.0001), Hughes grade ≥ 4 at admission and nadir (P = 0.003 and P < 0.001, respectively), acute inflammatory demyelinating polyneuropathy subtype (P = 0.017), sepsis (P = 0.001), mechanical ventilator support (P = 0.013), longer hospitalization length of stay (P < 0.0001), and inability to walk independently at 6 months (P < 0.001). Multivariate analysis performed to assess the risk factors of hyponatremia revealed that urinary retention (odds ratio [OR] 30.7, 95% confidence interval [CI] 3.6–264.4; P = 0.002) and mechanical ventilator support (OR 13.8, 95% CI 1.6–118.0; P = 0.017) were significant independent risk factors of hyponatremia. In assessing the outcomes of patients with hyponatremia, multivariate analysis showed that hyponatremia was independently associated with hospitalization length of stay ≥ 60 days and inability to walk independently at 6 month, with the former showing statistical significance but the latter not (OR 9.3, 95% CI 1.8–47.7; P = 0.007 and OR 4.9, 95% CI 0.9–26.3; P = 0.066, respectively). Therefore, we demonstrate that, along with mechanical ventilator support, urinary retention—possibly indicating autonomic dysfunction—is a risk factor of hyponatremia in GBS. Moreover, we confirm that hyponatremia is associated with poor outcome in GBS.

135 Guillain-Barré Syndrome After COVID-19 Vaccination: A Systematic Review and Analysis of Case Reports

INTRODUCTION: Cases of Guillain-BarrÉ Syndrome (GBS) have been believed to be associated with the novel COVID-19 infection, and also with the following vaccines developed against the infection. METHODS: An electronic search was conducted through four databases: PubMed, Scopus, medRxiv, and Google Scholar for all case reports after COVID-19 vaccine administration. Differences between groups were assessed by Pearson chi-square test. Modified Erasmus GBS Outcome Score for the ability to walk after GBS was calculated for all cases with sufficient clinical data, and Kaplan-Meier survival analysis was performed to study the effect of vaccine type on the relationship between vaccination time and complication of GBS. RESULTS: 175 cases of GBS following COVID-19 vaccination were included. The Acute Inflammatory Demyelinating Polyradiculoneuropathy subtype was the most reported subtype with 74 cases (42.29%). The affected age group averaged around 53.59 ± 18.83 years, with AMSAN occurring in a rather older group (63.88 ± 20.87 years, p = 0.049). The AstraZeneca vaccine was associated with AIDP (n = 38, 21.71%) more than other vaccines, p = 0.02. The bilateral facial palsy subtype was mostly linked to adenoviral vector vaccinations, accounting for an average of 72% of the total BFP cases. Most GBS patients survived (96%, p = 0.036), however, most patients had low mEGOS scores (4 ± 3.57, p &lt; 0.01). On average, patients developed GBS at 13.43 ± 11.45 days from vaccination (p = 0.73), and survival analysis for complication of GBS into mechanical ventilation or walking impairment yielded a severely increased probability of complication after 25 days (p &lt; 0.01). CONCLUSIONS: Guillain-BarrÉ Syndrome can manifest after COVID-19 vaccination. Most cases occurred after receiving the AstraZeneca vaccine, and despite low mortality rates, ambulation was compromised in most patients. A higher risk of GBS complication is associated with an onset later than 12-13 days, particularly with Pfizer, AstraZeneca, and Moderna vaccines.

Dynamics and prognostic value of serum neurofilament light chain in Guillain-Barré syndrome

Neurofilament light chain (NfL) is a biomarker for axonal damage in several neurological disorders. We studied the longitudinal changes in serum NfL in patients with Guillain-Barré syndrome (GBS) in relation to disease severity, electrophysiological subtype, treatment response, and prognosis. We included patients with GBS who participated in a double-blind, randomised, placebo-controlled trial that evaluated the effects of a second course of intravenous immunoglobulin (IVIg) on clinical outcomes. Serum NfL levels were measured before initiation of treatment and at one, two, four, and twelve weeks using a Simoa HD-X Analyzer. Serum NfL dynamics were analysed using linear mixed-effects models. Logistic regression was employed to determine the associations of serum NfL with clinical outcome and the prognostic value of serum NfL after correcting for known prognostic markers included in the modified Erasmus GBS Outcome Score (mEGOS). NfL levels were tested in serum from 281 patients. Serum NfL dynamics were associated with disease severity and electrophysiological subtype. Strong associations were found between high levels of serum NfL at two weeks and inability to walk unaided at four weeks (OR=1.74, 95% CI=1.27-2.45), and high serum NfL levels at four weeks and inability to walk unaided at 26 weeks (OR=2.79, 95% CI=1.72-4.90). Baseline serum NfL had the most significant prognostic value for ability to walk, independent of predictors included in the mEGOS. The time to regain ability to walk unaided was significantly longer for patients with highest serum NfL levels at baseline (p=0.0048) and week 2 (p<0.0001). No differences in serum NfL were observed between patients that received a second IVIg course vs. IVIg and placebo. Serum NfL levels are associated with disease severity, axonal involvement, and poor outcome in GBS. Serum NfL potentially represents a biomarker to monitor neuronal damage in GBS and an intermediate endpoint to evaluate the effects of treatment. Prinses Beatrix Spierfonds W.OR19-24.

Post-marketing active surveillance of Guillain Barré Syndrome following COVID-19 vaccination in persons aged ≥12 years in Italy: A multi-database self-controlled case series study.

Recently published studies have reported association of COVID-19 vaccine ChAdOx1-S (Vaxzevria) with Guillain Barré Syndrome (GBS). Less is known about the safety of other COVID-19 vaccines with respect to GBS outcome. This study investigated the association of COVID-19 vaccines with GBS in more than 15 million persons aged ≥12 years in Italy. Study population was all individuals aged ≥12 years who received at least one dose of COVID-19 vaccines, admitted to emergency care/hospital for GBS from 27 December 2020-30 September 2021 in Italy. Identification of GBS cases and receipt of at least one dose of mRNA-1273 (Elasomeran), BNT162b2 (Tozinameran), ChAdOx1-S (Vaxzevria) and Ad26.COV2.S (Janssen) through record linkage between regional health care and vaccination registries. Relative Incidence (RI) was estimated Self-controlled case series method adapted to event-dependent exposure using in the 42-day exposure risk period after each dose compared with other observation periods. Increased risk of GBS was found after first (RI = 6.83; 95% CI 2.14-21.85) and second dose (RI = 7.41; 2.35-23.38) of mRNA-1273 and first dose of ChAdOx1-S (RI = 6.52; 2.88-14.77). Analysis by age found an increased risk among those aged≥60 years after first (RI = 8.03; 2.08-31.03) and second dose (RI = 7.71; 2.38-24.97) of mRNA-1273. The first dose of ChAdOx1-S was associated with GBS in those aged 40-59 (RI = 4.50; 1.37-14.79) and in those aged ≥ 60 years (RI = 6.84; 2.56-18.28). mRNA-1273 and ChAdOx1-S vaccines were associated with an increased risk of GBS however this risk resulted in a small number of excess cases. Limitations were loss of GBS outpatient cases and imprecision of the estimates in the subgroup analysis due to a low number of events.

An Observational Study on Factors Causing Variable Response to Therapeutic Doses of Intravenous Immunoglobulin in Acute Immune-Mediated Polyneuropathy: A Tertiary Care Center Experience

Context: Immune-mediated neuropathies, notably Guillain–Barré syndrome (GBS) and its subtypes, exhibit diverse clinical presentations. Intravenous immunoglobulin (IVIG) is a standard treatment for these conditions, but the variable clinical course complicates outcome prediction. Despite standard IVIG dosing, some patients continue to deteriorate. Aim: The aim of this study was to assess the factors responsible for variable response to therapeutic doses of immunoglobulins in acute immune-mediated polyneuropathy. Settings and Design: This was a prospective, observational study. Materials and Methods: Acute immune-mediated polyneuropathy cases within 14 days of symptom onset and receiving IVIG were recruited. Hughes disability score (HDS), modified Erasmus GBS outcome score (mEGOS), and modified Rankin score (mRS) were assessed before IVIG, immediately after IVIG, and 4 weeks thereafter. Statistical Analysis Used: Categorical variables are expressed as percentages, and continuous variables are presented as mean ± standard deviation. Relationships between parameters were assessed using analysis of variance and multivariate analysis of covariance. Correlations were measured using Pearson’s correlation, and proportions were compared using the Chi-square test or Fisher’s exact test. Results: Among 60 GBS patients, the median age was 38 years, with upper respiratory tract infection as the most common antecedent infection (60%). The most common variant was pure motor type, while demyelinating (88.33%) was the most common electrophysiological subtype. Outcome correlated with post-IVIG scores of HDS and mRS. Linear regression analysis showed a positive correlation between onset-to-treatment duration and HDS and mEGOS scores after 4 weeks of IVIG, while mRS showed minimal correlation. No significant correlations were found between antecedent infection, gender, nerve conduction study pattern, GBS variant, and scores of HDS, mEGOS, and mRS. Conclusions: The study highlights the significance of onset-to-treatment duration. Factors with unfavorable course were age, delay in receiving IVIG, respiratory involvement, dysautonomia, cranial nerve involvement, and those with higher mEGOS scores at the 7th day of admission. Seasonal trends should not be overlooked. Serial evaluation of disability scores can predict the varying response to IVIG.

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.

European Academy of Neurology/Peripheral Nerve Society Guideline on diagnosis and treatment of Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy. Symptoms may vary greatly in presentation and severity. Besides weakness and sensory disturbances, patients may have cranial nerve involvement, respiratory insufficiency, autonomic dysfunction and pain. To develop an evidence-based guideline for the diagnosis and treatment of GBS, using Grading of Recommendations, Assessment, Development and Evaluation (GRADE) methodology, a Task Force (TF) of the European Academy of Neurology (EAN) and the Peripheral Nerve Society (PNS) constructed 14 Population/Intervention/Comparison/Outcome questions (PICOs) covering diagnosis, treatment and prognosis of GBS, which guided the literature search. Data were extracted and summarised in GRADE Summaries of Findings (for treatment PICOs) or Evidence Tables (for diagnostic and prognostic PICOs). Statements were prepared according to GRADE Evidence-to-Decision (EtD) frameworks. For the six intervention PICOs, evidence-based recommendations are made. For other PICOs, good practice points (GPPs) are formulated. For diagnosis, the principal GPPs are: GBS is more likely if there is a history of recent diarrhoea or respiratory infection; CSF examination is valuable, particularly when the diagnosis is less certain; electrodiagnostic testing is advised to support the diagnosis; testing for anti-ganglioside antibodies is of limited clinical value in most patients with typical motor-sensory GBS, but anti-GQ1b antibody testing should be considered when Miller Fisher syndrome (MFS) is suspected; nodal-paranodal antibodies should be tested when autoimmune nodopathy is suspected; MRI or ultrasound imaging should be considered in atypical cases; and changing the diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) should be considered if progression continues after 8 weeks from onset, which occurs in around 5% of patients initially diagnosed with GBS. For treatment, the TF recommends intravenous immunoglobulin (IVIg) 0.4 g/kg for 5 days, in patients within 2 weeks (GPP also within 2-4 weeks) after onset of weakness if unable to walk unaided, or a course of plasma exchange (PE) 12-15 L in four to five exchanges over 1-2 weeks, in patients within 4 weeks after onset of weakness if unable to walk unaided. The TF recommends against a second IVIg course in GBS patients with a poor prognosis; recommends against using oral corticosteroids, and weakly recommends against using IV corticosteroids; does not recommend PE followed immediately by IVIg; weakly recommends gabapentinoids, tricyclic antidepressants or carbamazepine for treatment of pain; does not recommend a specific treatment for fatigue. To estimate the prognosis of individual patients, the TF advises using the modified Erasmus GBS outcome score (mEGOS) to assess outcome, and the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess the risk of requiring artificial ventilation. Based on the PICOs, available literature and additional discussions, we provide flow charts to assist making clinical decisions on diagnosis, treatment and the need for intensive care unit admission.

European Academy of Neurology/Peripheral Nerve Society Guideline on diagnosis and treatment of Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy. Symptoms may vary greatly in presentation and severity. Besides weakness and sensory disturbances, patients may have cranial nerve involvement, respiratory insufficiency, autonomic dysfunction and pain. To develop an evidence-based guideline for the diagnosis and treatment of GBS, using Grading of Recommendations, Assessment, Development and Evaluation (GRADE) methodology a Task Force (TF) of the European Academy of Neurology (EAN) and the Peripheral Nerve Society (PNS) constructed 14 Population/Intervention/Comparison/Outcome questions (PICOs) covering diagnosis, treatment and prognosis of GBS, which guided the literature search. Data were extracted and summarised in GRADE Summaries of Findings (for treatment PICOs) or Evidence Tables (for diagnostic and prognostic PICOs). Statements were prepared according to GRADE Evidence-to-Decision (EtD) frameworks. For the six intervention PICOs, evidence-based recommendations are made. For other PICOs, good practice points (GPPs) are formulated. For diagnosis, the principal GPPs are: GBS is more likely if there is a history of recent diarrhoea or respiratory infection; CSF examination is valuable, particularly when the diagnosis is less certain; electrodiagnostic testing is advised to support the diagnosis; testing for anti-ganglioside antibodies is of limited clinical value in most patients with typical motor-sensory GBS, but anti-GQ1b antibody testing should be considered when Miller Fisher syndrome (MFS) is suspected; nodal-paranodal antibodies should be tested when autoimmune nodopathy is suspected; MRI or ultrasound imaging should be considered in atypical cases; and changing the diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) should be considered if progression continues after 8 weeks from onset, which occurs in around 5% of patients initially diagnosed with GBS. For treatment, the TF recommends intravenous immunoglobulin (IVIg) 0.4 g/kg for 5 days, in patients within 2 weeks (GPP also within 2-4 weeks) after onset of weakness if unable to walk unaided, or a course of plasma exchange (PE) 12-15 L in four to five exchanges over 1-2 weeks, in patients within 4 weeks after onset of weakness if unable to walk unaided. The TF recommends against a second IVIg course in GBS patients with a poor prognosis; recommends against using oral corticosteroids, and weakly recommends against using IV corticosteroids; does not recommend PE followed immediately by IVIg; weakly recommends gabapentinoids, tricyclic antidepressants or carbamazepine for treatment of pain; does not recommend a specific treatment for fatigue. To estimate the prognosis of individual patients, the TF advises using the modified Erasmus GBS outcome score (mEGOS) to assess outcome, and the modified Erasmus GBS Respiratory Insufficiency Score (mEGRIS) to assess the risk of requiring artificial ventilation. Based on the PICOs, available literature and additional discussions, we provide flow charts to assist making clinical decisions on diagnosis, treatment and the need for intensive care unit admission.

Predicting of Mechanical Ventilation and Outcomes by Using Models and Biomarker in Guillain-Barré Syndrome.

Guillain-Barré syndrome (GBS) is an acute inflammatory neuropathy. The Erasmus GBS Respiratory Insufficiency Score (EGRIS) and the modified Erasmus GBS Outcome Score (mEGOS) are prognostic models used in the prediction of mechanical ventilation and outcome. Thus far, there are only few biomarkers for the prognosis prediction of GBS patients, and albumin level is one that is promising. Patients diagnosed with GBS from 2013 to 2022 at Renmin Hospital, Wuhan University, China, were included. Patients hospitalized between 2016 and 2022 underwent short- and long-term follow-ups. The correlations between EGRIS/mEGOS and mechanical ventilation and outcome were evaluated. Serum albumin level was examined the day after admission. Furthermore, we also investigated whether the level of serum albumin was useful in predicting disease severity or poor outcome. In all, 145 patients were enrolled. Nineteen patients (13.1%) who required mechanical ventilation had higher Hughes GBS disability score (HGDS) at admission and discharge (P < 0.05 and P < 0.0001, respectively), shorter time from onset to admission and treatment (P < 0.01 and P < 0.001, respectively) and longer hospital stays (P < 0.001) than patients who did not require mechanical ventilation. High EGRIS scores were linked with the need for mechanical ventilation (r = 0.427, P < 0.001, AUC = 0.623). Seventy-one patients were admitted between 2016 and 2022. Of these, 65 patients had a 4-week follow-up and 61 had a 6-month follow-up. Higher mEGOS scores at admission and 7days after admission significantly correlated with short- (P < 0.0001 and P < 0.0001) and long-term (P < 0.05 and P < 0.05) outcomes, respectively. No significant difference in outcome was found between different subtypes (4weeks [P = 0.099] and 6months [P = 0.172]). Patients with lower albumin level tended to have higher HGDS (at admission P < 0.05, at nadir P < 0.001, and at discharge P < 0.001) and higher properties of the need of mechanical ventilation (P < 0.05) and ICU stay (P < 0.05) than those with normal albumin levels. Those with low albumin levels were also unable to walk independently at 6months (P < 0.01). mEGOS scores predicted the outcomes of GBS patients in China, and EGRIS score predicted the need for mechanical ventilation in these patients. Albumin level at admission correlated well with disease severity and outcomes.

Guillain-Barré Syndrome.

This article summarizes the clinical features, diagnostic criteria, differential diagnosis, pathogenesis, and prognosis of Guillain-Barré syndrome (GBS), with insights into the current and future diagnostic and therapeutic interventions for this neuromuscular syndrome. GBS is an acute, inflammatory, immune-mediated polyradiculoneuropathy that encompasses many clinical variants and divergent pathogenic mechanisms that lead to axonal, demyelinating, or mixed findings on electrodiagnostic studies. The type of antecedent infection, the development of pathogenic cross-reactive antibodies via molecular mimicry, and the location of the target gangliosides affect the subtype and severity of the illness. The data from the International GBS Outcome Study have highlighted regional variances, provided new and internationally validated prognosis tools that are beneficial for counseling, and introduced a platform for discussion of GBS-related open questions. New research has been undertaken, including research on novel diagnostic and therapeutic biomarkers, which may lead to new therapies. GBS is among the most frequent life-threatening neuromuscular emergencies in the world. At least 20% of patients with GBS have a poor prognosis and significant residual deficits despite receiving available treatments. Research is ongoing to further understand the pathogenesis of the disorder, find new biomarkers, and develop more effective and specific treatments.

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

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

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.

CSF Findings in Relation to Clinical Characteristics, Subtype, and Disease Course in Patients With Guillain-Barré Syndrome.

To investigate CSF findings in relation to clinical and electrodiagnostic subtypes, severity, and outcome of Guillain-Barré syndrome (GBS) based on 1,500 patients in the International GBS Outcome Study. Albuminocytologic dissociation (ACD) was defined as an increased protein level (>0.45 g/L) in the absence of elevated white cell count (<50 cells/μL). We excluded 124 (8%) patients because of other diagnoses, protocol violation, or insufficient data. The CSF was examined in 1,231 patients (89%). In 846 (70%) patients, CSF examination showed ACD, which increased with time from weakness onset: ≤4 days 57%, >4 days 84%. High CSF protein levels were associated with a demyelinating subtype, proximal or global muscle weakness, and a reduced likelihood of being able to run at week 2 (odds ratio [OR] 0.42, 95% CI 0.25-0.70; p = 0.001) and week 4 (OR 0.44, 95% CI 0.27-0.72; p = 0.001). Patients with the Miller Fisher syndrome, distal predominant weakness, and normal or equivocal nerve conduction studies were more likely to have lower CSF protein levels. CSF cell count was <5 cells/μL in 1,005 patients (83%), 5-49 cells/μL in 200 patients (16%), and ≥50 cells/μL in 13 patients (1%). ACD is a common finding in GBS, but normal protein levels do not exclude this diagnosis. High CSF protein level is associated with an early severe disease course and a demyelinating subtype. Elevated CSF cell count, rarely ≥50 cells/μL, is compatible with GBS after a thorough exclusion of alternative diagnoses. This study provides Class IV evidence that CSF ACD (defined by the Brighton Collaboration) is common in patients with GBS.

The early clinical and laboratory predictors of GBS outcome: hospital-based study, Assiut University, Upper Egypt

BackgroundThis study was designed to identify factors that influence outcomes in a large group of well-defined Guillain–Barré syndrome (GBS) patients with a 3-month follow-up period. Sixty-two cases of GBS with a mean age of 37.15 ± 17.60 years (33 males and 29 females) were recruited in the first 2 weeks after onset. Clinical history, examination, and a variety of rating scales including Medial Research Council sum score (MRC), Erasmus Guillain-Barré respiratory insufficiency score (EGRIS), at admission and 10 days later were performed. Follow-up investigations at 3 months included the Hughes Disability Scale (HDS), and Overall Neuropathy Limitation Scale (ONLS).Results64.5% of participants had cranial nerve deficits, 45% had neck muscle weakness, 30.6% had dysautonomia, and 8.1% were mechanically ventilated. C-reactive protein was elevated in 38.7%, and hyponatremia was recorded in 30.6% of patients. Older age, antecedent events particularly diarrhea, neck muscles weakness, low MRC sum score, impaired cough reflex, dysautonomia, and hyponatremia, were all significantly associated with poor outcomes at 3 months using HDS and ONLS. Regression analysis with dependent variables of HDS outcome showed that the presence of an antecedent event particularly diarrhea, neck muscle weakness, hyponatremia and the presence cytoalbuminous dissociation of CSF at onset, and low MRC sum score at 10th day after treatment, were predictors of poor outcome.ConclusionClinical and laboratory predictors of poor outcome were older age, the presence of an antecedent event particularly diarrhea, low MRC sum score at the 10th day, elevated CRP, hyponatremia and the presence cytoalbuminous dissociation.

Guillain-Barré syndrome associated with SARS-CoV-2 vaccination: how is it different? a systematic review and individual participant data meta-analysis.

An association between Guillain-Barré syndrome (GBS) and severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) vaccination has been reported. We aimed to summarize the clinical features of GBS associated with SARS-CoV-2 vaccination and determine the contrasting features from coronavirus disease-19 (COVID-19) associated GBS and GBS following other causes. We performed PubMed search for articles published between 1 December 2020 and 27 January 2022 using search terms related to "SARS-CoV-2 vaccination" and "GBS". Reference searching of the eligible studies was performed. Sociodemographic and vaccination data, clinical and laboratory features, and outcomes were extracted. We compared these findings with post-COVID-19 GBS and International GBS Outcome Study (IGOS) (GBS from other causes) cohorts. We included 100 patients in the analysis. Mean age was 56.88 years, and 53% were males. Six-eight received non-replicating virus vector and 30 took messenger RNA (mRNA) vaccines. The median interval between the vaccination and the GBS onset was 11 days. Limb weakness, facial palsy, sensory symptoms, dysautonomia, and respiratory insufficiency were seen in 78.65%, 53.3%, 77.4%, 23.5%, and 25%, respectively. The commonest clinical and electrodiagnostic subtype were sensory-motor variant (68%) and acute inflammatory demyelinating polyneuropathy (61.4%), respectively. And 43.9% had poor outcome (GBS outcome score ≥3). Pain was common with virus vector than mRNA vaccine, and the latter had severe disease at presentation (Hughes grade ≥3). Sensory phenomenon and facial weakness were common in vaccination cohort than post-COVID-19 and IGOS. There are distinct differences between GBS associated with SARS-CoV-2 vaccination and GBS due to other causes. Facial weakness and sensory symptoms were commonly seen in the former and outcomes poor.

Intravenous immunoglobulin treatment with prognosis for the first six months of Guillain-Barré Syndrome in Somalia: Case series.

Guillain-Barré Syndrome (GBS) is an acute, immune-mediated polyneuropathy that often leads to severe weakness. Intravenous Immunoglobulin (IVIG) is a proven effective treatment for GBS (class 1 evidence). The clinical course of GBS in individual patients is highly variable and difficult to predict. It is a retrospective observational study of 10 patients diagnosed with GBS by using nerve conduction studies and lumbar puncture. Fifty percent of the patients were under 40 years old, and ninety percent were male; all but one were treated with IVIG; and forty percent of the total mEGOS obtained was less than 5. The average predicted probability of being unable to walk unaided after 4 weeks was 47.7%, the average predicted probability of being unable to walk unaided after 3 months was 17%, and the average predicted probability of being unable to walk unaided after 6 months was 8.05%. Patients presenting with acute ascending weakness should be identified early, and early IVIG treatment for GBS improves disability as measured by The Modified Erasmus GBS Outcome Scale (mEGOS).

Serum C3 complement levels predict prognosis and monitor disease activity in Guillain-Barré syndrome

ObjectiveBiomarkers are needed to predict prognosis and disease activity in patients with Guillain-Barré syndrome (GBS). The complement system is a key player in the pathogenesis of GBS. This study aimed to assess the potential utility of serum complement proteins as novel biomarkers in GBS. MethodsWe reviewed the medical records of 76 GBS patients with C3 and C4 measurements during hospitalization between 2010 and 2021. Clinical outcomes were correlated with baseline serum C3, C4, and seven additional predictors: four existing biomarkers (GM1, albumin, immunoglobulin G, neutrophil-lymphocyte ratio) and three clinical factors from the modified Erasmus GBS outcome score model. Five complement activation products (C3a, C4a, C5a, soluble C5b-9, factor Bb) were measured in 35 patients and were compared with C3 and C4 levels. Longitudinal changes in C3 and C4 levels were compared with the disease course in 12 patients. ResultsHigher C3, but not C4, was associated with poorer outcomes: lower Medical Research Council sum scores (MRCSS), higher GBS disability score (GBSDS), longer hospitalization, and more frequent treatment-related fluctuations. Age, MRCSS at admission, and baseline serum C3 were significant independent indicators of 1- and 3-month GBSDS. We found that C3 was positively correlated with C3a (r = 0.32) and C5a (r = 0.37), which indicates an activated complement cascade with high C3. Longitudinal change of C3 coincided with clinical severity of the disease course. InterpretationThis study highlights the use of serum C3 as a novel mechanistic biomarker in GBS. Larger prospective studies are needed to validate our findings.