Guillain-Barré Syndrome Patients Research Articles

Albuminocytologic Dissociation and the Impact of Age-Adjusted Cerebrospinal Fluid Protein Levels in Guillain–Barré Syndrome

Background: This study examined the impact of age-adjusted cerebrospinal fluid (CSF) protein levels on clinical characteristics, disease severity, and outcomes in Guillain–Barré Syndrome (GBS) patients. Methods: This retrospective study included 71 GBS patients at UTMB Galveston. Albuminocytologic dissociation (ACD) was defined as CSF–total protein (CSF-TP) >0.45 g/L with a cell count of <50 cells/L. Patients were grouped using the conventional cutoff (>0.45 g/L) and age-adjusted upper limits (URLs) for CSF-TP levels, comparing clinical, CSF, and electrophysiological characteristics across groups. Results: The mean age was 50 years (SD = 14.5). The mean age of patients with a CSF-TP > 45 g/L was higher (53 vs. 39 years, p = 0.000), whereas no such difference was noted using age-dependent URLs. Using age-adjusted CSF-TP URLs reduced the sensitivity for detecting ACD by 20%. CSF-TP > age-adjusted URLs were associated with lower MRC sum scores (39 vs. 47.43, p = 0.000), higher ICU admission rates (34% vs. 20%, p = 0.003), and the need for second-line treatment (41% vs. 17%, p = 0.049), and the trends were not observed with the conventional cutoff of 0.45 g/L. CSF-TP was an independent predictor of lower MRC sum scores (p = 0.009, 95% CI −0.058, −0.009) and higher GBS disability scores (p = 0.015, 95% CI 0.000, 0.004). Conclusions: ACD is a common finding in GBS, but normal protein levels do not exclude the diagnosis. Using age-adjusted URLs might improve specificity but reduce sensitivity for ACD detection, potentially increasing false negatives. CSF-TP levels exceeding age-adjusted URLs were more strongly associated with greater disease severity and poorer outcomes compared to the conventional cutoff of 0.45 g/L.

Predictors of the Short-Term Outcomes of Guillain-Barré Syndrome: Exploring Electrodiagnostic and Clinical Features.

Guillain-Barré syndrome (GBS), an acute inflammatory disorder of the peripheral nervous system, is characterized by muscle weakness and paralysis. Prompt identification of patients at a high risk of poor outcomes is crucial for timely intervention. In this study, we combined clinical data with nerve conduction study and electromyography data to identify the predictors of GBS outcomes. We retrospectively analyzed the data of patients with GBS who had received treatment at Chang Gung Memorial Hospital, Taiwan, between 1998 and 2022. Comprehensive clinical and electrophysiological data were collected. Statistical analyses were performed to identify the predictors of poor outcomes. The patients were stratified into two groups by their scores on the GBS Disability Scale: good (score ≤ 2) and poor (score > 2) outcome groups. The study finally included 24 GBS patients (mean age: 53.0 ± 20.9 years; female-to-male ratio: 2.3; good outcome group: 13; poor outcome group: 11). Compared with the good outcome group, the poor outcome group was old (43.0 ± 20.4vs. 64.0 ± 15.7, p = 0.011), had a short time-to-treatment period (12.9 ± 7.8vs. 6.5 ± 5.4 days, p = 0.033), exhibited more prevalent mechanical ventilation use (0vs. 36.4%, p = 0.017), and had a prolonged hospitalization duration (14.7 ± 10.2vs. 53.1 ± 20.0 days, p < 0.001). Poor outcomes were associated with low compound muscle action potential (CMAP), slow motor nerve conduction velocity (MNCV), abnormal F-wave latency, and more conduction block and temporal dispersion. In the subgroup of acute inflammatory demyelinating polyradiculoneuropathy (AIDP), there were 19 patients, out of which 10 had good outcomes, while nine had poor outcomes. The clinical features that differentiate between good and poor outcomes in the AIDP subgroup were similar to those observed in all GBS patients. Notably, the motor conduction features, including distal and proximal CMAP and MNCV of the median and tibial nerves (all p < 0.05), were particularly important electrodiagnostic features of outcome discrimination in the AIDP subgroup. Combining clinical data with nerve conduction study and electromyography data can assist in predicting outcomes of both GBS patients and the AIDP subgroup. Poor outcomes are associated with older age, a more abrupt onset pattern, low CMAP, and slow nerve conduction, and abnormal tibial F responses can predict poor outcomes. Early identification of high-risk patients facilitates tailored interventions. This highlights the importance of combining clinical and electrophysiological data in GBS management.

Soluble Membrane Attack Complex (sMAC) as a Potential Diagnostic Biomarker Differentiating Acute Viral Encephalitis from Guillain-Barré Syndrome, a Post-infectious Autoimmune State.

Acute encephalitis syndrome (AES) presents with the onset of fever, altered sensorium and/or seizures, known to be caused by various infectious and non-infectious aetiological agents, among which viruses are the commonest. The severity of AES prompts rapid diagnosis, which is not met by time-consuming conventional diagnostic techniques. In this study, archived cerebrospinal fluid samples of laboratory-confirmed viral AES, an acute infectious condition and Guillain-Barré Syndrome (GBS), a post-infectious, autoimmune condition was assessed for soluble membrane attack complex (sMAC) using ELISA. Statistical analysis was performed to understand the diagnostic potential of sMAC in AES versus GBS patients. sMAC levels were significantly increased in viral encephalitis compared with GBS samples (43.69ng/mL vs. 29.33ng/mL, P < 0.05). The diagnostic potential of sMAC was assessed using the receiver operating characteristic(ROC) curve, which demonstrated excellent diagnostic discrimination between viral AES and GBS (area under curve = 0.8125, 95% CI, P < 0.0001). Using Youden's index, the optimal sMAC cut-off was calculated as 33.6ng/mL for distinguishing AES from GBS. The findings of our study revealed significant increase in sMAC levels in AES patients in comparison to those with GBS. This underscores the utility of sMAC as a valuable tool in distinguishing between AES and GBS, thereby facilitating more tailored patient management strategies, which varies for acute infectious and post-infectious conditions especially those mediated by autoimmunity.

Tackling respiratory failure in Guillain Barre’ syndrome: Burdens, management and prognosis

Guillain-Barré syndrome (GBS) is a rapidly progressive, inflammatory polyradiculoneuropathy with a heterogeneous presentation and pathogenesis. Diagnosis is clinical and electrophysiological, with supportive features in cerebrospinal fluid. Respiratory insufficiency is a life-threatening manifestation occurring in 20 to 30% of GBS patients associated with poor functional outcome. Progressive weakness of the respiratory muscles is the leading cause of acute respiratory distress, leading to respiratory failure with hypoxemia and hypercapnia. Bulbar weakness may compromise airway patency and predisposes patients to aspiration pneumonia. Mechanical ventilation (MV) is often necessary in patients with severe GBS. Prediction and early recognition of high respiratory failure risk is important to triage patients to the appropriate unit, that is, general ward or intensive care unit (ICU). MV needs to be applied when patients meet respiratory failure criteria and severe clinical signs, as bulbar weakness, ineffective cough and dysautonomia. Prompt initiation of immunotherapy with intravenous immunoglobulin or plasmapheresis, together with supportive care, are effective in GBS. There are challenging clinical questions related to the use of MV in GBS: when to initiate the ventilatory support, what to use, when and how to wean and when to perform tracheostomy. In this review, the authors summarize the up-to-date knowledge of pathophysiology and general management in the real- world clinical practice of the respiratory failure in GBS, focusing on time of MV, of weaning and of tracheostomy.

Guillain-Barré Syndrome in Africa: Understanding, Addressing, and Empowering.

Guillain-Barré Syndrome (GBS) is a spectrum of peripheral neuropathies characterized by rapid symmetrical limb weakness and sensory symptoms. GBS can be life-threatening and requires intensive care, particularly for patients with imminent respiratory failure. In Africa, limited research and high therapy costs pose challenges. This literature review aims to comprehensively address GBS in Africa to improve understanding and outcomes. This literature review aims to provide an extensive overview of GBS in Africa, encompassing its clinical presentation, impact, management approaches, and challenges faced. It also highlights the need for increased research and awareness to enhance patient care and outcomes. A comprehensive review of existing literature on GBS in Africa was conducted, focusing on clinical presentation, diagnosis, management, and its impact on patients and communities. Data sources included medical databases, research articles, and reports. Data was scoured from databases such as PubMed, Medline and Embase. A total of four hundred and fifty-five articles and case studies were screened, with broader topic margins into GBS and different triggers, demographics, statistics, and variations in treatments across the world. These articles were further screened to match our inclusion criteria which focused on articles published after 2000 and which gave clearer insights into the presentations and situation of GBS in the African continent. GBS in Africa is characterized by a range of clinical presentations, with limited diagnostic resources and healthcare infrastructure. Patients often face long intervals between symptom onset and hospitalization, impacting outcomes. The syndrome's impact extends beyond physical symptoms, affecting patients' quality of life, employment, and community roles. Management involves immunotherapy, physiotherapy, and psychosocial support, but high therapy costs and incomplete recovery pose challenges. Research in Africa has grown in recent years but remains limited compared to other regions. Efforts are needed to expand research capacity, introduce early screening programs, and improve healthcare infrastructure. GBS presents a significant healthcare challenge in Africa, with the potential for severe clinical outcomes. This literature review underscores the importance of enhancing research, awareness, and healthcare infrastructure. African-led research initiatives offer hope for improved patient outcomes and healthcare system strengthening. By advocating for increased government support and resources, Africa can address the pressing needs of GBS patients and foster a brighter and healthier future for affected individuals on the continent.

Prediction of respiratory failure and prolonged mechanical ventilation in Guillain-Barré syndrome: A prospective cohort study in Bangladesh.

The aim of this study is to validate and perform a region-specific adjustment of the Erasmus GBS Respiratory Insufficiency Score (EGRIS) and identify potential predictors of prolonged mechanical ventilation (PMV) among Guillain-Barré syndrome (GBS) patients from Bangladesh. We enrolled GBS patients from four prospective observational cohort studies conducted in Bangladesh. Accuracy of EGRIS to predict the requirement of MV in <7 days of study entry was evaluated. Model performance was assessed by discrimination (ability of the model to differentiate between patients who needed MV or not) and calibration (accuracy of absolute risk estimates). PMV was defined as duration of MV >14 days. Potential predictors for PMV were evaluated by Cox regression. A total of 594 GBS patients aged ≥6 years old were enrolled; of whom 541 patients had complete EGRIS data prior to MV and were included in validation analysis. EGRIS correctly distinguished between patients requiring MV or not in 81% pairs (AUC = 0.81). EGRIS overestimated the probability of MV than the observed probability (41% vs. 20%) which was resolved by updating of the model intercept. Inability to flex hip at day 7 of start of MV was the strongest predictor for PMV with predicted probabilities of 82%. EGRIS accurately predicts the need for MV in GBS patients from Bangladesh. This study developed a region-specific version of EGRIS and identified predictors of PMV. These findings can assist clinicians to identify patients at high risk of developing respiratory failure and requiring PMV to ensure timely intubation and tracheostomy of the patients in low resource settings.

Clinical characteristics and functional outcomes of pediatric Guillain-Barré syndrome admitted to the Neuro-intensive care unit: a decade-long retrospective observational study.

Guillain-Barré Syndrome (GBS) remains a significant contributor to acute flaccid paralysis in pediatric patients worldwide. Despite its impact, studies focusing on pediatric GBS requiring intensive care unit (ICU) management are limited. This study aimed to address this gap by exploring the clinical and outcome characteristics of pediatric GBS necessitating ICU care. This retrospective observational study, spanning a decade, analyzed the records of 75 pediatric GBS patients admitted to the Neuro-ICU of a tertiary care center in South India. Data included demographics, prodromal symptoms, clinical features, investigations, treatment modalities, and outcomes. The majority (55/75) of patients were male, with a median age of 12 years. The highest incidence of GBS requiring ICU admission was in the monsoon season. Prodromal symptoms were observed in 56%. Most patients (93.33%) presented with typical GBS symptoms, and 40% had respiratory distress on ICU admission. Acute motor axonal neuropathy (AMAN) was the most common subtype. Approximately 80% required mechanical ventilation, with a median duration of 22.5 days. No in-hospital mortality was recorded. At discharge, most patients had a GBS disability score of 4, improving to 2 at a median follow -up of 228 days. Pediatric GBS patients requiring ICU care exhibit distinctive characteristics, including a higher prevalence of AMAN subtype, seasonal clustering, and favorable outcomes with intensive treatment. The absence of in-hospital mortality underscores the effectiveness of prompt ICU admission and dedicated Neuro-intensive care.

Mortality and its predictors among patients with Guillain-Barré syndrome in the intensive care unit of a low-income country, Ethiopia: a multicenter retrospective cohort study.

Guillain-Barré syndrome (GBS) is a rare autoimmune disease that affects the peripheral nervous system. It is characterized by the destruction of nerves involved in movement. This condition can lead to transient pain, changes in temperature and touch sensations, muscle weakness, loss of sensation in the legs and/or arms, and difficulty swallowing or breathing. Published data on the outcomes of critical care for patients with GBS are extremely scarce in Africa, particularly Ethiopia. Therefore, this study aimed to assess mortality and its predictors among patients with GBS in the intensive care unit (ICU) of specialized hospitals in Ethiopia, a low-income country. This retrospective cohort study was conducted at the Tibebe Ghion Specialized Hospital and the Felege Hiwot Comprehensive Specialized Hospital in Bahir Dar, Ethiopia, from 1 January 2019 to 30 December 2023. Data were collected in the medical record rooms. Cox regression analysis was performed to identify the predictors of mortality among GBS patients in the ICU. The crude and adjusted hazard ratios (AHRs) and 95% confidence intervals (CIs) were calculated using bivariable and multivariable Cox regression models. A p-value of <0.05 was considered statistically significant. Of 124 GBS patients admitted to the ICU, 120 were included in the final analysis. During the follow-up, there were 23 (19.17%) deaths. The overall incidence rate of death was 1.96 (95% CI: 1.30, 2.95) per 100 person-days of observation. Traditional medicine (AHR = 3.11, 95%: 1.12, 16.70), COVID-19 infection (AHR = 5.44, 95% CI: 1.45, 73.33), pre-ICU cardiac arrest (AHR = 6.44, 95% CI: 2.04, 84.50), and ICU readmission (AHR = 4.24, 95% CI: 1.03, 69.84) were identified as the independent predictors of mortality. The mortality rate among GBS patients admitted to the ICU was high. Traditional medicine, COVID-19 infection, pre-ICU cardiac arrest, and readmission to the ICU were the significant predictors of mortality. Conducting large-scale studies with a prospective design in the future would yield more robust evidence.

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.

Validating ICD-10 Diagnosis Codes for Guillain-Barré Syndrome in Taiwan's National Health Insurance Claims Database.

To validate the International Classification of Diseases, 10th Revision (ICD-10) codes for Guillain-Barré syndrome (GBS) in Taiwan's insurance claims database. We identified adult patients hospitalized at any Chang Gung Memorial Foundation branch hospital between January 1st, 2017, and December 31st, 2022, with ICD-10 code G61.0 in any of the five discharge diagnosis positions, indicating possible Guillain-Barré syndrome. We then validated the possible GBS diagnosis using data from electronic medical records of the identified patients, based on the diagnostic criteria established by the National Institute of Neurological Disorders and Stroke. We determined the positive predictive values (PPV) of various operational definitions, including the position (primary or other) where the code was recorded in the discharge diagnosis, nerve conduction study (NCS) claims, and / or specific GBS treatments. The final validation cohort of 484 patients with ICD-10 code for GBS in the discharge diagnosis was found to include 368 true GBS patients. Identifying inpatients using only the ICD-10 code for GBS in any of the five positions for discharge diagnosis yielded a PPV of 76.0%. With more restrictive definitions (primary diagnosis only, or requiring additional claims for NCS and/or treatments), the PPV tended to increase, but with fewer true GBS patients identified. Using ICD-10 GBS code in the primary diagnosis plus NCS and treatment claims yielded the highest PPV (98.3%); however, 140 (38.0%) of the true GBS patients were missed using this definition. In contrast, using the ICD-10 GBS code in any position, plus claims for NCS, achieved a relatively good PPV (85.8%) with minimal loss of true GBS patients (13, ie, 3.5%). In Taiwan's NHI claims data, identifying true GBS patients using only the ICD-10 code yielded a PPV of 76.0%; however, adding claims for diagnostic procedure and GBS treatment increased the PPV to 98.3%.

The Initial Clinical and Electrophysiological Characteristics of Different Subtypes of Guillain-Barré Syndrome Diagnosed Based on Serial Electrophysiological Examinations.

We aimed to identify different Guillain-Barré syndrome (GBS) subtypes, demyelination, axonal degeneration, and reversible conduction failure (RCF) as early as possible by analyzing the initial clinical and electrophysiological examinations. This study retrospectively collected GBS patients between October 2018 and December 2022 at Beijing Tiantan Hospital. The diagnostic criteria for the initial electrophysiological study were based on Rajabally's criteria, and the criteria for the serial electrophysiological study were based on Uncini's criteria. All subjects underwent clinical and electrophysiological evaluations at least twice within 8 weeks. A total of 47 eligible patients with GBS were included, comprising 19 acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 18 axonal degenerations, and 10 RCFs. In the RCF group, 40%, 30%, and 30% patients were diagnosed as AIDP, axonal, and equivocal at the initial study, respectively. The AIDP group had significantly higher cerebrospinal fluid (CSF) protein than the RCF (123.8 [106.4, 215.1] mg/dL vs. 67.1 [36.8, 85.6] mg/dL, p=0.002) and axonal degeneration (123.8 [106.4, 215.1] mg/dL vs. 60.8 [34.8, 113.0] mg/dL, p<0.001) groups. The RCF group had significantly lower Hughes functional grades at admission (3 [2, 4] vs. 4 [4, 4], p=0.012) and discharge (1.0 [1.0, 2.0] vs. 3.0 [2.0, 3.0], p<0.001) than the axonal degeneration group and showed significantly shorter distal motor latency (DML), Fmin, Fmean, Fmax, and lower F% than the AIDP group (p<0.05). The early identification of RCF from AIDP had relatively obvious features, including slightly elevated CSF protein levels and normal or slightly prolonged DML and F-wave latencies, contrasting with the apparent elevation and prolongation seen in AIDP. Differentiating RCF from axonal degeneration remains challenging. One potential distinguishing factor is that the motor function in RCF tends to be better than in the latter.

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.

Neurofilament light chain as a diagnostic and prognostic biomarker in Guillain–Barré syndrome

BackgroundElevated neurofilament light chain (NfL) levels are associated with worse prognosis in Guillain–Barré syndrome (GBS). Our objectives were to determine the utility of serum NfL (sNfL), cerebrospinal fluid (CSF)/serum NfL ratio and NfL index as prognostic and diagnostic biomarkers for GBS.MethodsWe measured NfL in serum and/or CSF obtained from 96 GBS patients between 1989 and 2014 in western Sweden. The sNfL Z-scores, NfL ratios and NfL indices were calculated. Outcome was determined with the GBS disability scale (GBSDS) at 3 and 12 months. NfL parameters in GBS were compared with healthy controls (HC), multiple sclerosis (MS), and amyotrophic lateral sclerosis (ALS).ResultsThe sNfL Z-score was higher for GBSDS > 2 at 3 months (median [IQR], 3.5 ng/L [3.2–4.0], vs 2.6 [1.7–3.4], p = 0.008) and at 12 months (3.6 ng/L [3.5–3.8] vs 2.6 [1.8–3.5], p = 0.049). NfL ratio and index were not associated with outcome. The area under the curve (AUC) for sNfL Z-score was 0.76 (95% CI 0.58–0.93, p < 0.0001) for GBSDS > 2 at 3 months. NfL ratio and index were lower in GBS than HC, MS, and ALS. The AUC for the NfL ratio was 0.66 (95% CI 0.55–0.78, p = 0.0018) and for the NfL index 0.86 (95% CI 0.78–0.93, p < 0.0001).DiscussionOur results confirm sNfL as prognostic biomarker for GBS and the precision was improved using the age-adjusted sNfL Z score. NfL index and Qalb are potential diagnostic biomarkers for GBS.

Comprehensive Microbiological and Metagenomic Analysis of the Guillain–Barré Syndrome Outbreak in Lima, 2019

In 2018/2019, two large Guillain–Barré Syndrome (GBS) outbreaks took place in Peru. Here, we report a comprehensive analysis of biological samples from GBS patients from the 2019 outbreak. We applied metagenomic, microbiologic, and serological analyses to different biological samples collected from GBS patients. Further phenotypic and genomic characterization was conducted on Campylobacter jejuni isolates from GBS samples. Microbiologic and metagenomic analyses revealed several patients with multiple co-infections, yet no common infectious agents were found other than C. jejuni. Four C. jejuni isolates were isolated from rectal swabs. Twenty-one patients had detectable IgG serum antibodies related to C. jejuni, of whom seven had IgM antibodies. Genomic analyses showed that these four strains were clonal (ST2993) and contained the class A lipooligosaccharide biosynthesis locus. These results further support the idea that that C. jejuni is the etiological agent that triggered the GBS outbreak in Peru in 2019 and that the strains are not restricted to Peru, hence could be regarded as a broad public health concern. Furthermore, though we cannot delineate the role played by co-infections in GBS development, results obtained herein highlight metagenomic analysis as a potential new tool for depicting a yet unknown area of research in GBS.

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.

Interpretable Machine Learning Model for Predicting the Prognosis of Guillain-Barré Syndrome Patients.

Machine learning (ML) is increasingly used in medical predictive modeling, but there are no studies applying ML to predict prognosis in Guillain-Barré syndrome (GBS). The medical records of 223 patients with GBS were analyzed to construct predictive models that affect patient prognosis. Least Absolute Shrinkage and Selection Operator (LASSO) was used to filter the variables. Decision Trees (DT), Random Forest (RF), Extreme Gradient Boosting (XGBoost), k-nearest Neighbour (KNN), Naive Bayes (NB), Neural Network (NN). Light Gradient Boosting Machine (LGBM) and Logistic Regression (LR) were used to construct predictive models. Clinical data from 55 GBS patients were used to validate the model. SHapley additive explanation (SHAP) analysis was used to explain the model. Single sample gene set enrichment analysis (ssGSEA) was used for immune cell infiltration analysis. The AUCs (area under the curves) of the 8 ML algorithms including DT, RF, XGBoost, KNN, NB, NN, LGBM and LR were as follows: 0.75, 0.896 0.874, 0.666, 0.742, 0.765, 0.869 and 0.744. The accuracy of XGBoost (0.852) was the highest, followed by LGBM (0.803) and RF (0.758), with F1 index of 0.832, 0.794, and 0.667, respectively. The results of the validation set data analysis showed AUCs of 0.839, 0.919, and 0.733 for RF, XGBoost, and LGBM, respectively. SHAP analysis showed that the SHAP values of blood neutrophil/lymphocyte ratio (NLR), age, mechanical ventilation, hyporeflexia and abnormal glossopharyngeal vagus nerve were 0.821, 0.645, 0.517, 0.401 and 0.109, respectively. The combination of NLR, age, mechanical ventilation, hyporeflexia and abnormal glossopharyngeal vagus used to predict short-term prognosis in patients with GBS has a good predictive value.

Expanding our understanding of Guillain-Barré syndrome: Recent advances and clinical implications.

Guillain-Barré syndrome (GBS) is a rare yet potentially life-threatening disorder of the peripheral nervous system (PNS), characterized by substantial clinical heterogeneity. Although classified as an autoimmune disease, the immune mechanisms underpinning distinct GBS subtypes remain largely elusive. Traditionally considered primarily antibody-mediated, the pathophysiology of GBS lacks clarity, posing challenges in the development of targeted and effective treatments. Nevertheless, recent investigations have substantially expanded our understanding of the disease, revealing an involvement of autoreactive T cell immunity in a major subtype of GBS patients and opening new biomedical perspectives. This review highlights these discoveries and offers a comprehensive overview of current knowledge about GBS, including ongoing challenges in disease management.

Role of prazosin in patients with Guillain-Barré syndrome with sympathetic overactivity: A cohort study.

In Guillain-Barré syndrome (GBS), patients with dysautonomia demonstrate sympathetic overactivity (SO). This study assessed the role of prazosin (α1-blocker) in the management of SO. This cohort study was conducted from January 2022 to September 2023. Thirty-two GBS patients with SO received prazosin (2.5-10 mg three times a day) (prazosin group). For comparison, we included historical controls that included 33 GBS patients having SO with similar baseline characteristics, including median age and disability, who did not receive prazosin, from a GBS registry of patients admitted during February 2018-December 2021. The primary endpoint was days to resolution of SO. Secondary endpoints were daily fluctuations in the systolic (SBP) and diastolic blood pressure (DBP), duration of hospital stay, in-hospital mortality, and disability at 3 months. The median ages of both the treatment and the control groups were 36 (IQR 25-49) years and 43 (66.2%) were males. The demographic and clinical parameters were comparable. Prazosin resulted in significantly earlier normalization of SO compared to the control group (median 15 vs. 20 days; p = .01). The mean fluctuations in the SBP and DBP at 15 days were significantly lower in the prazosin group. However, the duration of hospital stay and good recovery at 3 months were comparable. Three patients developed hypotension, while two patients died (ventilator-associated pneumonia) in the prazosin group. This study provides new evidence supporting the role of prazosin in SO, and needs randomized trials to confirm our findings.

Prevalence and Prognostic Impact of Hyponatremia in Guillain-Barré Syndrome: A Systematic Review and Meta-Analysis.

This study aims to systematically review the existing literature and perform a meta-analysis to evaluate the prevalence of hyponatremia among Guillain Barre Syndrome (GBS) patients and its relationship with disease prognosis.We comprehensively searched PubMed, Embase, Medline, Web of Science, Science Direct, and the Cochrane Library databases from 1995 to 2024 for observational studies on the prevalence of hyponatremia in GBS. The meta-analysis followedthe Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA 2020) guidelines. Heterogeneity among the included studies was calculated with the I2 for each analysis. We used the Comprehensive Meta-Analysis software (Version 3.3.070; Biostat, Englewood, USA). Eight observational studies met our inclusion criteria. The meta-analysis showed that the pooled prevalence of hyponatremia among GBS patients was 12% (95% CI: 0.107-0.149). The results exhibited high heterogeneity (I² = 99%), indicating significant variability among the studies. Hyponatremia rates reported in these eight studies ranged from 11.5% to 48% in GBS patients. The prevalence of hyponatremia was found to be 12% in GBS patients, which is relatively lower compared to some reports. Hyponatremia was found to be associated with prolonged hospital stay, mortality, and mechanical ventilation as poor prognostic factors. Further prospective studies are needed to elucidate the mechanisms underlying hyponatremia in GBS and to develop targeted interventions to address this issue.

A nationwide Guillain-Barré syndrome epidemiological study in Spain during the COVID-19 years.

The purpose was to perform a nationwide epidemiological study of Guillain-Barré syndrome (GBS) in Spain, analysing background incidences and seasonal variation and trying to identify incidence changes during the coronavirus disease 2019 (COVID-19) years. This was an observational study collecting all GBS diagnoses from the National Epidemiological Surveillance Network collected by the Ministry of Health. Patients discharged with GBS as the main diagnosis and admitted during 2018-2021 were included. Data on the incidence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections were obtained from the National Epidemiology Centre. In total, 3147 cases were included, 832 in 2018, 861 in 2019, 670 in 2020 and 784 in 2021. Nationwide hospital incidence was 1.78 in 2018, 1.71 in 2019, 1.41 in 2020 and 1.66 in 2021, with an increased frequency in males, the elderly population and in the winter season. Eleven per cent of GBS patients needed ventilatory support. GBS and SARS-CoV-2 incidences did not correlate with one another (r = -0.29, p = 0.36). GBS incidence decreased during 2020 and during the COVID-19 lockdown period in comparison to the same months of 2018-2019. The incidence of GBS in Spain is similar to that of other countries. Despite prior reports describing a significant increase in COVID-19-associated GBS in Spain, a significant drop of GBS incidence during the SARS-CoV-2 pandemic was detected, probably due to prevention measures.