Erasmus Guillain-Barré Syndrome Outcome Score Research Articles

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 < 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 < 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.

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.

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.

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.

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.

Reliability and validity of prognostic indicators for Guillain-Barré syndrome in children.

To explore the clinical characteristics and prognostic predictors of Guillain-Barré syndrome (GBS) in Chinese paediatric patients. The clinical features of children with GBS hospitalized in the Children's Hospital of Chongqing Medical University were summarized retrospectively. The correlation between the Erasmus GBS Outcome Score (EGOS)/modified Erasmus GBS Outcome Score (mEGOS), GBS disability score (GDS)/modified Rankin Scale (MRS), Erasmus GBS Respiratory Insufficiency Score (EGRIS), and mechanical ventilation were evaluated. One hundred forty-two patients (86 males, 56 females; median 62.50 months [interquartile range 41.00-97.50]) with classic GBS were enrolled in the study. In the present GBS cohort, 134 (94.37%) patients could walk independently (GDS ≤2) and 121 (85.21%) could manage without assistance (MRS ≤2) at 6 months. Eighteen (12.68%) patients with GBS required mechanical ventilation. The performance of mEGOS on admission, mEGOS on day 7, and EGOS-predicted GDS outcome at 4 weeks, 3 months, and 6 months in the paediatric patients with GBS admitted within 2 weeks of disease onset and that of the MRS outcome were evaluated. The EGRIS in individuals who required mechanical ventilation was significantly higher than in patients without mechanical ventilation (median=6 vs median=3, p < 0.001). In Chinese paediatric patients with GBS who were admitted 2 weeks after disease onset, the mEGOS and EGOS are validated indicators for the prediction of clinical outcomes 6 months after onset. EGRIS is helpful in predicting the implementation of mechanical ventilation in the acute phase. The Erasmus Guillain-Barré syndrome (GBS) Outcome Score (EGOS) and modified EGOS are reliable prognostic predictors in paediatric patients with GBS. The Erasmus GBS Respiratory Insufficiency Score (EGRIS) is an effective predictor of mechanical ventilation in paediatric patients with GBS. An EGRIS of ≥5 indicates a high risk of mechanical ventilation in the acute phase.

Clinical features of Guillain-Barré syndrome with anti-neurofascin 155 antibody.

Anti-neurofascin 155 (NF155) antibody has been discovered in chronic demyelinating conditions. However, the positive rate and clinical description were insufficient in acute demyelinating conditions, such as Guillain-Barré syndrome (GBS). This study aimed to explore the positive rate of anti-NF155 antibody in GBS patients and determine whether there were unique clinical characteristics in these patients. Serum anti-NF155 antibody was detected from 94 GBS patients and 50 sex- and age-matched healthy controls using cell-based assay and tissue-based assay with immunostaining of mouse teased sciatic nerve fibers. Clinical characteristics, laboratory data, and electrophysiology examinations were retrospectively collected. Seven of 94 (7.45%) GBS patients were positive for anti-NF155 antibody, and the main IgG subclass was IgG1. Compared with anti-NF155 antibody-negative GBS patients, anti-NF155 antibody-positive GBS patients had a higher GBS disability score at nadirs (p=.010), higher modified Erasmus GBS outcome score (p=.022), higher rate of abnormal compound motor action potential (CMAP) amplitude (p=.002), higher frequency of prolonged F-wave latency (p < .001), lower frequency of abnormal sensory conduction velocity (p < .001) and sensory nerve action potential amplitude (p < .001), more axonal type (p=.040), and poorer therapeutic effect (p=.017). Anti-NF155 antibody exists in a small portion of GBS patients. Anti-NF155 antibody-positive GBS patients possibly have a more severe clinical course, less sensory nerves involved, higher proportion of axonal type, poorer therapeutic effect, and worse prognosis, but the pathogenicity of the anti-NF155 antibody in GBS needs further study.

Validation and adjustment of modified Erasmus GBS outcome score in Bangladesh.

ObjectiveWe have assessed and improved the performance of the modified Erasmus GBS Outcome Score (mEGOS) among patients with Guillain‐Barré syndrome (GBS) from Bangladesh.MethodsValidation cohort consisted of patients with GBS from two prospective cohort studies in Bangladesh. Poor outcome was defined as being unable to walk independently at week 4 and week 26. We excluded patients able to walk independently, patients who died within the first week, or with missing GBS disability scores. Performance of mEGOS at entry and week 1 was determined based on the discriminative ability (ability to differentiate between patients able and unable to walk independently; measured using the area under the receiver operating characteristic curves [AUC]) and calibration (observed probability versus predicted probability of poor outcome).ResultsA total of 506 patients aged ≥6‐year‐old were enrolled, with 471 and 366 patients included in mEGOS validation analysis at entry and week 1, respectively. The AUC values for predicting poor outcome (1) at week 4 were 0.69 (mEGOS entry) and 0.78 (mEGOS week 1) and (2) at week 26 were 0.67 (mEGOS entry) and 0.70 (mEGOS week 1). Mean predicted probabilities of poor outcome corresponded with observed outcomes except for the probability of poor outcome at week 4 which was overestimated by mEGOS week 1. This was resolved by updating the model intercept.InterpretationThe mEGOS shows valid outcome predictions among patients with GBS from Bangladesh. The model can aid the identification of patients at high risk of poor outcome and help to adequately allocate healthcare resources in low‐resource settings.

Ganglioside complex antibodies in an Indian cohort of Guillain-Barré syndrome.

Antibodies against ganglioside complexes (GSCs) are associated with various clinical features and subtypes of Guillain-Barré syndrome (GBS). One-hundred patients were evaluated for antibodies to GSCs formed by combination of GM1, GM2, GD1a, GD1b, GT1b, and GQ1b using manual enzyme linked immuno-sorbent assay (ELISA). Twenty-six patients were GSC antibody-positive, most frequent being against GM1-containing GSC (76.9%). Gender distribution, mean age, symptom-duration, antecedent events, electrophysiological subtypes, requirement for mechanical ventilation, and median duration of hospital stay were comparable between the GSC antibody-positive and negative groups. There was no association between specific GSC antibody and electrophysiological subtypes or clinical variants. After controlling for false discovery rate (FDR) using the Benjamini-Hochberg method, the number of subjects who improved in overall disability sum score, modified Erasmus GBS outcome score, and neuropathy symptom score at discharge was significantly higher in the GSC antibody-positive group. Improvements in Medical Research Council sum scores and Hughes Disability Scale during the hospital stay between the GSC antibody-positive and negative groups were not significantly different after controlling for FDR. The GSC antibody-positive group had better outcome at hospital discharge in some of the disability scores. Pathophysiological pathways among patients without GSC antibodies may be different and this requires further evaluation.

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.

Second IVIg course in Guillain-Barré syndrome with poor prognosis: the non-randomised ISID study

ObjectiveTo compare disease course in patients with Guillain-Barré syndrome (GBS) with a poor prognosis who were treated with one or with two intravenous immunoglobulin (IVIg) courses.MethodsFrom the International GBS Outcome...

Dysautonomia in Guillain-Barré Syndrome: Prevalence, Clinical Spectrum, and Outcomes.

Guillain-Barré syndrome (GBS), when severe, involves the autonomic nervous system; our objective was to assess the spectrum and predictors of dysautonomia, and how it may impact functional outcomes. A retrospective review of patients admitted to the Mayo Clinic in Rochester, MN between January 1, 2000, and December 31, 2017, with GBS and dysautonomia was performed. Demographics, comorbidities, parameters of dysautonomia, clinical course, GBS disability score, and Erasmus GBS Outcome Score (EGOS) at discharge were recorded. One hundred eighty seven patients were included with 71 (38%) noted to have at least one manifestation of dysautonomia. There are 72% of patients with a demyelinating form of GBS and 36% of patients with demyelination had dysautonomia. Ileus (42%), hypertension (39%), hypotension (37%), fever (29%), tachycardia or bradycardia (27%), and urinary retention (24%) were the most common features. Quadriparesis, bulbar and neck flexor weakness, and mechanical ventilation were associated with autonomic dysfunction. Patients with dysautonomia more commonly had cardiogenic complications, syndrome of inappropriate antidiuretic hormone, posterior reversible encephalopathy syndrome, and higher GBS disability score and EGOS. Mortality was 6% in patients with dysautonomia versus 2% in the entire cohort (P = 0.02). Dysautonomia in GBS is a manifestation of more severe involvement of the peripheral nervous system. Accordingly, mortality and functional outcomes are worse. There is a need to investigate if more aggressive treatment is warranted in this category of GBS.

The utility of Guillain-Barré syndrome prognostic models in Malaysian patients.

Guillain-Barré syndrome (GBS) is an acute immune-mediated neuropathy that has variable disease course and outcome. The Erasmus GBS outcome score (EGOS), modified EGOS (mEGOS), and Erasmus GBS respiratory insufficiency score (EGRIS) are prognostic models designed to predict the functional outcome of GBS patients at 6 months (EGOS and mEGOS) and the need for mechanical ventilation within a week of admission (EGRIS). The models were primarily developed in the Dutch GBS population, and thus the usefulness of these models in other GBS cohorts is less clear. In the current study, we aimed to validate mEGOS, EGOS, and EGRIS in Malaysian GBS patients. A total of 107 patients with GBS and its variants were consecutively recruited. Patients with GBS and Miller Fisher syndrome (MFS) were analysed separately. In the GBS cohort, high mEGOS and EGOS scores were significantly correlated with poor outcome at 6 months (mEGOS on admission: r = .381, P = .005; mEGOS at day 7 of admission: r = .507, P < .001; EGOS: r = .484, P < .001). However, there were no significant correlations between mEGOS or EGOS and outcome in patients with MFS (mEGOS on admission: r = .152, P = .523; mEGOS at day 7 of admission: r = .008, P = .973; EGOS: r = .110; P = .644). The score of EGRIS for GBS patients with mechanical ventilation was significantly higher than those patients without mechanical ventilation (4 ± 2 vs 3 ± 1; P < .001). We conclude that mEGOS and EGOS are clinically useful and relevant to the Malaysian GBS population but not in patients with classic MFS. EGRIS could be used to predict the need for mechanical ventilation in our local GBS patients.

Markers for Guillain-Barré syndrome with poor prognosis: a multi-center study.

Guillain-Barré syndrome (GBS) is an acute monophasic neuropathy. Prognostic tools include the modified Erasmus GBS outcome score (mEGOS), Erasmus GBS respiratory insufficiency score (EGRIS), and the increase in serum IgG levels (ΔIgG) 2 weeks after intravenous immunoglobulin (IVIg) treatment. Given that proportions of GBS subtypes differ between Western countries and Japan, the usefulness of these tools in Japan or other countries remains unknown. We enrolled 177 Japanese patients with GBS from 15 university hospitals and retrospectively obtained mEGOS and EGRIS for all and ΔIgG status for 79 of them. High mEGOS scores on admission or on day 7 were significantly associated with poorer outcomes (unable to walk independently at 6 months). High EGRIS scores (≥5 points) were associated with an increased risk for mechanical ventilation. Patients with ΔIgG <1,108 mg/dl had significantly poorer outcomes. We suggest that mEGOS, EGRIS, and ΔIgG in GBS are clinically relevant in Japan.

Reduced soluble RAGE is associated with disease severity of axonal Guillain-Barré syndrome.

Soluble receptor for advanced glycation end products (sRAGE) is an anti-inflammatory factor that mitigates the proinflammatory effects of high mobility group box 1 (HMGB1). The aim of this study was to investigate whether Guillain-Barré syndrome (GBS)-related inflammation are mediated by sRAGE and HMGB1. We measured serum sRAGE, HMGB1, IL-6, and TNF-α levels in 86 patients with GBS and analysed associations between sRAGE or HMGB1 and clinical variables in these subjects. In addition, we determined cerebrospinal fluid sRAGE and HMGB1 levels in a cross-sectional study of 50 patients with GBS who had matched serum samples. We found serum sRAGE levels in patients with the acute motor axonal neuropathy (AMAN) subtype of GBS, but not other subtypes, were significantly lower than those in healthy controls, and were significantly correlated with GBS disability score and Erasmus GBS outcome score, while serum HMGB1, IL-6, and TNF-α levels in all subtypes of GBS were significantly higher than those in healthy controls. Moreover, increased sRAGE levels and decreased HMGB1 levels after treatment were observed. Our results showed that serum sRAGE may be a useful biomarker for inflammation in the AMAN GBS subtype, while HMGB1 may be related to the inflammatory process across all types of GBS.

Residual disability 10 years after falling ill in Guillain–Barré syndrome: A prospective follow-up study

ObjectiveTo describe residual disability 10years after onset of Guillain–Barré syndrome (GBS) and longitudinal changes from 2weeks after onset until 10years afterwards. The Erasmus GBS Outcome score (EGOS) was applied for predicting prognosis at 2 and 10years. MethodsTwenty-nine patients, mean age at onset 49years, were followed prospectively from 2weeks to 10years after GBS onset. Measures included; GBS disability score, EGOS, Barthel Index, Frenchay Activity Index, Sickness Impact Profile (SIP), Overall Neuropathy Limitations Scale (ONLS), Walk-12, and Fatigue Severity Scale. ResultsAt 10years, the facial paralysis found in 5 participants at 2years was still present, 11 participants (38%) experienced paresthesia, 6 (21%) had limitations in their arms, and 15 (52%) had limitations in walking. Decreased health-related quality of life on comparison to the general population was seen in the physical dimension of SIP at 10years. The median EGOS at 2weeks was 4.5, which correlated highly only with the Barthel Index at 2years and the ONLS arm scale at 10years. ConclusionThe residual disabilities at 1–2years comprised mainly of reduced walking ability, and are still persistent 10years after GBS onset. For some individuals, facial paralysis caused major disability. The EGOS only partly predicted residual disability at 2 and 10years after onset.