Porphyric neuropathy.

Acute hepatic porphyrias are inherited metabolic disorders that may present with polyneuropathy, which if not diagnosed early can lead to quadriparesis, respiratory weakness, and death. Porphyric neuropathy is an acute to subacute motor predominant axonal neuropathy with a predilection for the upper extremities and usually preceded by a predominantly parasympathetic autonomic neuropathy. The rapid progression and associated dysautonomia mimic Guillain-Barré syndrome but are distinguished by the absence of cerebrospinal fluid albuminocytologic dissociation, progression beyond 4 wk, and associated abdominal pain. Spot urine test to assess the porphyrin precursors delta-aminolevulinic acid and porphobilinogen can provide a timely diagnosis during an acute attack. Timely treatment with intravenous heme, carbohydrate loading, and avoidance of porphyrinogenic medications can prevent further neurological morbidity and mortality.

Incidence and mortality rates of Guillain-Barré syndrome in Serbia.

Guillain-Barré syndrome (GBS) is an acute auto-immune polyradiculoneuropathy. A huge variety of GBS incidence and mortality rates has been noted across the world. The objective of the present multi-centric study was to assess the incidence and mortality rates of GBS during a 10-year period in Serbia. We collected data of adult GBS patients who were hospitalized from 2009 to 2018 in all five tertiary healthcare centers in Serbia. The incidence rates per 100 000 inhabitants with 95% confidence intervals (CI) were calculated and further corrected for the estimated number of patients hospitalized in secondary centers. Mortality rates were also assessed. GBS was considered severe if patients were not able to walk at least 10 m without assistance. Six hundred and forty GBS patients were registered in tertiary centers in a 10-year period. The proportion of severe cases was 75% at nadir, and 52% on discharge. GBS incidence rate in Serbia was 1.1 per 100 000 inhabitants, and estimated incidence if patients from secondary centers included 1.2 per 100 000. Peak incidence was observed during the sixth decade of life. During the acute phase, 5.6% of GBS patients died, while overall 9.7% of them died during 6-month period from disease onset. This study contributes to our knowledge about GBS epidemiology. Results will allow us to improve the diagnosis and treatment of GBS patients in Serbia.

Guillain-Barré syndrome associated with SARS-CoV-2 infection. A systematic review.

Guillain-Barré syndrome (GBS) incidence can increase during outbreaks of infectious illnesses. A few cases of GBS associated with coronavirus disease 2019 (COVID-19) infection have been reported. The aim was to identify specific clinical features of GBS associated with COVID-19. PubMed, Embase and Cochrane were searched from 1 November 2019 to 17 May 2020 and included all papers with full text in English, Spanish, French or Italian, reporting original data of patients with GBS and COVID-19. Data were extracted according to a predefined protocol. A total of 18 patients reported in 14 papers were included in this review. All the patients were symptomatic for COVID-19, with cough and fever as the most frequently reported symptoms. The interval between the onset of symptoms of COVID-19 and the first symptoms of GBS ranged from -8 to 24 days (mean 9 days; median 10 days). Most of the patients had a typical GBS clinical form predominantly with a demyelinating electrophysiological subtype. Mechanical ventilation was necessary in eight (44%) patients. Two (11%) patients died. Published cases of GBS associated with COVID-19 report a sensorimotor, predominantly demyelinating GBS with a typical clinical presentation. Clinical features and disease course seem similar to those observed in GBS related to other etiologies. These results should be interpreted with caution since only 18 cases have been heterogeneously reported so far.

Effect of Early Tracheostomy on Mortality of Mechanically Ventilated Patients with Guillain-Barré Syndrome: A Nationwide Observational Study.

BACKGROUND: Patients with Guillain-Barré syndrome (GBS) who require mechanical ventilation (MV) are regarded as candidates for early tracheostomy because of the high risk of prolonged MV; however, the association between early tracheostomy and favorable outcomes in patients with GBS remains unclear. In this study, we evaluated the association between early tracheostomy and outcomes in mechanically ventilated patients with GBS. METHODS: This retrospective observational study included adult patients with GBS identified in the Japanese Diagnosis Procedure Combination national inpatient database from July 1, 2010, to March 31, 2018, who initiated MV within the first week of admission and who received MV for more than 1 week. Early tracheostomy was defined as tracheostomy performed within 7 days of MV. The primary outcome was in-hospital mortality, and the secondary outcomes were 28-day mortality, nosocomial pneumonia, length of hospital stay, length of intensive care unit (ICU) stay, duration of MV, duration of sedation, duration of analgesia, duration of delirium, and total hospitalization costs. Propensity scores for early tracheostomy were calculated using a logistic regression model on the following variables: age; sex; body mass index; Japan Coma Scale status at admission; Charlson comorbidity index score; comorbidity of chronic pulmonary disease; complication of pneumonia at admission; complication of hyponatremia at admission; neurological presentation at admission; ambulance use; referral from other hospitals; treatment year; days from hospital admission to MV initiation; ICU admission until the day of MV initiation; and treatments until the day of MV initiation. Stabilized inverse probability of treatment weighting analyses was performed to compare the outcomes between patients with and without early tracheostomy. RESULTS: Among 919 eligible patients, 654 patients (71%) underwent tracheostomy, with 136 patients (15%) receiving early tracheostomy. Overall, the median time from initiation of MV to tracheostomy was 12 days (interquartile range 8-15 days). After stabilized inverse probability of treatment weighting, early tracheostomy was not associated with lower in-hospital mortality (risk difference 0.4%; 95% confidence interval - 5.6 to 6.7%) compared with patients without early tracheostomy. There were no significant differences in 28-day mortality (risk difference - 1.3%; 95% confidence interval - 3.5 to 0.9%) and incidence of nosocomial pneumonia (risk difference - 2.6%; 95% confidence interval - 9.1 to 4.2%) between the two groups. None of the other secondary outcomes differed significantly between the groups. CONCLUSIONS: Early tracheostomy was not significantly associated with decreased mortality or morbidity in patients with GBS requiring MV for more than 1 week.

Guillain-Barre Syndrome.

Guillain-Barre syndrome (GBS) is the leading cause of acute paralysis that can potentially affect all of the human population. GBS is believed to be an immune-mediated disease, possibly triggered by a recent infection, and driven by an immune attack targeting the peripheral nervous system. GBS can be divided into several subtypes depending on the phenotype, pathophysiology, and neurophysiological features. Unfortunately, morbidity and mortality rates are still high despite the current understanding of the pathophysiology and available treatment options. Additional research is still needed to shed more light into the pathogenesis for a better understanding and treatment of this condition.

Incidence, Risk Factors, and Outcome of Immune-Mediated Neuropathies (IMNs) following Haploidentical Hematopoietic Stem Cell Transplantation.

Immune-mediated neuropathies (IMNs) following hematopoietic stem cell transplantation have been described recently, which, excluding Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy, may present with atypical patterns. This retrospective, nested, case-control study reviewed data from 3858 patients who received haploidentical hematopoietic stem cell transplantation (haplo-HSCT) during the past 10 years at a single center, and 40 patients (1.04%) with IMN following haplo-HSCT were identified. Chronic graft-versus-host disease (cGVHD) (P = .043) and cytomegalovirus (CMV) viremia (P = .035) were recognized as independent risk factors for the development of IMN after haplo-HSCT. There were no significant differences in overall survival (P = .619), disease-free survival (P = .609), nonrelapse mortality (P = .87), or the incidence of relapse (P = .583) between patients with and without IMN after haplo-HSCT. However, patients with post-transplant IMN were at higher risk of developing cGVHD (P = .012) than patients who did not develop IMN. Twenty-four of the 40 patients with IMN (60%) attained neurologic improvement after treatments including vitamins B1 and B12 and/or immunomodulatory agents. However, 19 (47.5%) patients still had persistent motor/sensory deficits despite receiving timely treatment. More studies are needed to help develop standardized diagnostic and therapeutic strategies for patients with post-transplant IMN.

The disease burden associated with Campylobacter spp. in Germany, 2014.

Bacteria of the genus Campylobacter are an important cause of human illness worldwide. Campylobacter infections are expressed as gastroenteritis and can lead to severe sequelae like reactive arthritis, Guillain-Barré syndrome, irritable bowel syndrome and inflammatory bowel disease. In Germany, Campylobacter-associated gastroenteritis cases are notifiable but there is no reporting obligation for the sequelaes and the disease burden is clearly underestimated. The aim of our study was to quantify reliably the current disease burden of all Campylobacter spp.-associated diseases for Germany with the method of disability-adjusted life years (DALYs). DALYs combine mortality and morbidity in a single summary measure, whereby one DALY represents the loss of one year in full health. For acute gastroenteritis, we estimated 967 DALYs of which only 484 DALYs were detected within the reporting system. Overall, we estimated that 8811 DALYs were caused by the campylobacter-related diseases known so far. 98% of the DALYs were associated with morbidity and 2% with mortality. Mortality was caused by the health outcomes Gastroenteritis and Guillain-Barré syndrome exclusively.

A Novel Treatment Strategy for Severe Guillain-Barré Syndrome: Zipper Method.

OBJECTIVE: Intravenous immunoglobulin and plasma exchange are proven treatments for Guillain-Barré syndrome. Despite these treatments, the prognosis for severe Guillain-Barré syndrome is still not satisfactory. This article seeks for a logical timing for plasma exchange-intravenous immunoglobulin synergy, which may improve outcome in severe Guillain-Barré syndrome requiring mechanical ventilation. STUDY DESIGN: This study is an open-label study. Nine pediatric severe Guillain-Barré syndrome patients requiring mechanical ventilation were treated with novel treatment strategy named as "zipper method." In this method, following diagnosis of Guillain-Barré syndrome, plasma exchange was started immediately. In the first session of plasma exchange, one and a half volume of patients' plasma was removed by using 5% albumin as replacement solution. At the end of the plasma exchange session, 0.4 g/kg intravenous immunoglobulin infusion was started immediately. Second plasma exchange session was applied with one volume change after 24 hours from the end of the intravenous immunoglobulin infusion. Each plasma exchange session was followed by intravenous immunoglobulin infusions. This plasma exchange-intravenous immunoglobulin cycle was repeated for 5 times. RESULTS: Among the 9 patients, the mean mechanical ventilation duration was 7 (5-14) days and the mean hospital stay was 18 (10-30) days. Medical Research Council sum score was increased in all patients, especially after the third session. All patients survived and all patients were able to walk unaided on the 28th day of admission. CONCLUSION: The zipper method as a novel treatment modality seems to reduce mortality, speed up weaning from mechanical ventilation, and shorten hospital stay, with excellent outcome in severe Guillain-Barré syndrome patients, who require intensive care. This technique stands as a promising immunomodulation strategy for various scenarios.

Characteristics, incidence and outcome of patients admitted to intensive care unit with Guillain-Barre syndrome in Australia and New Zealand.

PURPOSE: To describe characteristics, incidence and outcome of patients with Guillain-Barre syndrome (GBS) admitted to ICU. METHODS: We conducted a binational, retrospective, observational, epidemiological study. We compared the baseline characteristics, physiological conditions and outcomes for GBS patients with or without mechanical ventilation (MV) and of survivors and non-survivors. RESULTS: We studied 711 patients admitted to 173 ICUs between 2005 and 2015. We found an increasing proportion of GBS admissions per year (P < 0.03). 237 patients required MV. These patients had higher APACHE III scores (47 vs 31), worse PaO2/FiO2 ratio (P/F 258 v 341), a significantly longer ICU LOS (25 v 4 days) (P < 0.0001, all comparisons) and a greater incidence of pre-ICU cardio-respiratory arrest (6.7% v 1.2%). Similarly, non-survivors were 5-times more likely to have experienced a pre-ICU cardio-respiratory arrest. Overall, ICU and hospital mortality were 3.9% and 6.9%, respectively and increased to 9.7% and 14.3% in the MV group. MV patients remained in hospital for almost 40 days. CONCLUSION: GBS represents a small but increasing proportion of ICU admissions with one-third of patients receiving MV. Overall in-hospital mortality is relatively low but doubles if MV is needed. These observations provide important prognostic information to clinicians involved in the care of these patients.

Acute flaccid paralysis in South African children: Causes, respiratory complications and neurological outcome.

AIM: To describe the causes, clinical presentation and neurological outcome of acute flaccid paralysis in children. METHODS: A retrospective study in a tertiary paediatric hospital in South Africa. Data on clinical presentation, respiratory complications and long-term neurological outcomes of children presenting with acute flaccid paralysis were collected. Logistic regression analysis was applied to determine predictors for the need of mechanical ventilation. RESULTS: The study included 119 patients, 99 of whom had Guillain-Barré syndrome (GBS); 47 patients (39.5%) required mechanical ventilation. Backward logistic regression analysis revealed that bulbar dysfunction (P < 0.001), autonomic dysfunction (P = 0.003) and upper limb paralysis (P = 0.038) significantly predicted the need for mechanical ventilation. EuroQol-5D scores of self-care problems and usual activities after discharge significantly declined over time. CONCLUSIONS: In this large series from Africa, GBS was the main cause of acute flaccid paralysis in children and was associated with significant morbidity. Other causes of acute flaccid paralysis mimicking GBS were not uncommon and should be excluded in this setting.

High mortality from Guillain-Barré syndrome in Bangladesh.

Although Guillain-Barré syndrome (GBS) has higher incidence and poor outcome in Bangladesh, mortality from GBS in Bangladesh has never been explored before. We sought to explore the frequency, timing, and risk factors for deaths from GBS in Bangladesh. We conducted a prospective study on 407 GBS patients who were admitted to Dhaka Medical College Hospital, Dhaka, Bangladesh from 2010 to 2013. We compared deceased and alive patients to identify risk factors. Cox regression model was used to adjust for confounders. Of the 407 GBS patients, 50 (12%) died, with the median time interval between the onset of weakness and death of 18 days. Among the fatal cases, 24 (48%) were ≥40 years, 36 (72%) had a Medical Research Council sum score ≤20 at entry, 33 (66%) had a progressive phase <8 days, and 27 (54%) required ventilation support. Ten patients (20%) died due to unavailability of ventilator. The strongest risk factor for deaths was lack of ventilator support when it was required (HR: 11.9; 95% confidence interval [CI]: 4.6-30.7). Other risk factors for death included age ≥40 years (HR: 5.9; 95% CI: 2.1-16.7), mechanical ventilation (HR: 2.3; 95% CI: 1.02-5.2), longer progressive phase (>8 days) (HR: 2.06; 95% CI: 1.1-3.8), autonomic dysfunction (HR: 1.9; 95% CI: 1.05-3.6), and bulbar nerve involvement (HR: 5.4; 95% CI: 1.5-19.2). In Bangladesh, GBS is associated with higher mortality rates, which is related to lack of ventilator support, disease severity, longer progressive phase of the disease, autonomic dysfunction, and involvement of the bulbar nerves.

Malignancy in Guillain-Barré syndrome: A twelve-year single-center study.

The relationship between Guillain-Barré syndrome (GBS) and malignancy is uncertain. We retrospectively analyzed data of 118 consecutive patients admitted with GBS from Birmingham, U.K. (2001-2012). We calculated relative cancer risk using different definitions and determined characteristics of malignancy-associated GBS. Malignancy was globally commoner in our GBS cohort compared to the general population (odds ratio: 2.08; CI: 1.06-3.71; p=0.036). However, this was unconfirmed if paraneoplastic criteria were applied. GBS patients with cancer were significantly more likely to be older (p=0.043), hyponatremic (p=0.037) and demonstrate more axonal loss (p<0.05). Cerebrospinal fluid (CSF) protein levels were lower in the malignancy group (p=0.002) and neurological improvement less likely (p=0.023). In-patient mortality was significantly higher in patients with malignancy (p<0.01). We conclude global cancer risk is higher in GBS than in the general population, although definition-dependent. Malignancy requires consideration in elderly, hyponatremic subjects with normal CSF protein, severe axonal loss, who fail to improve post-treatment.

Epidemiology and prognostic factors of inpatient mortality of Guillain-Barré syndrome: A nationwide population study over 14years in Asian country.

OBJECTIVE: The present study aimed to evaluate the characteristics of inpatient mortality from Guillain-Barré syndrome (GBS), which is a rare and potentially life-threatening polyradiculoneuropathy, in an Asian country, as there are few big-data studies regarding this topic. METHODS: We obtained data regarding patients with GBS from Taiwan's National Health Insurance Research Database admission records. We identified patients with a discharge diagnosis of GBS during 2000-2013 using the International Classification of Diseases, 9th Revision, Clinical Modification code (357.0), and evaluated their baseline characteristics, clinical complications, and risk factors. RESULTS: We identified 5469 patients with GBS, and the crude incidence of GBS was 1.71 per 100,000 person-years. The inpatient mortality rate was 1.61% (88/5469) and 55 deaths (62.5% of all deaths) occurred before day 19 of the hospitalization (mainly during the progressive phase). The predictors of inpatient mortality included older age, a greater comorbidity burden (especially catastrophic illness), endotracheal intubation, mechanical ventilation, cardiac complications, and systemic infection. In contrast, patients who were admitted to a medical center or Neurology Department exhibited a higher survival rate. CONCLUSIONS: This 14-year nationwide study included the largest analysis of Asian patients with GBS to date, and identified various prognostic factors that predicted inpatient mortality.

Patient characteristics and the effects of intravenous immunoglobulin in patients with Guillain-Barre syndrome.

Purpose - The purpose of this study is to determine the diagnosis- and treatment-related characteristics in Guillain-Barré syndrome (GBS) and to evaluate the effects of early intravenous immunoglobulin (IVIg) treatment on disability, mortality and prognosis. Materials and methods - Adult patients who were diagnosed with GBS in our clinic between January 2000 and January 2014 were retrospectively scanned. While the patients undergoing IVIg treatment were included in the study, the other neuropathic diseases were excluded. Patients were divided into two groups based on the administration time of the IVIg treatment; Group 1 (<7 days) and Group 2 (≥ 7 days) Group 1 consisted of patients undergoing IVIg treatment within 7 days after presentation of symptoms and Group 2 consisted of patients undergoing IVIg treatment on and after 7th day following presentation of symptoms. The scores from Hughes Functional Grading Scale (HFGS) on admission and one month laterwere recorded in all patients in order to evaluate the disability and prognosis in terms of demographic and clinical laboratory characteristics. Results - In this study, 49 GBS patients were included (31 patients in Group 1 and 18 patients in Group 2). Demyelinating form of GBS was determined in 22 (44.8%) patients.). While there was no difference between both groups (p: 0.288, p: 0.762, p: 0.693 respectively) in terms of intensive care and rehabilitation requirement and progression, only 2 patients in Group 1 died. While HFGS mean score on admission in all the patient groups was 3.27±0.974, their HFGS mean score at month 1 was 2.53±1.226. There was no difference between the groups in terms of HFGS mean scores on admission and at month 1. Within each groups, there was a significant improvement between initial (on admission) HFGS scores and HFGS scores acquired at month 1. Conclusion - In this study, demyelinating form was more frequent than axonal form. A total of 2 g/kg dose of IVIg treatment administered for 5 days as a standard in GBS patients ensured a significant improvement on both disability and early and late administration and early administration of the treatment does not lead to any difference in intensive care unit and rehabilitation requirements.

Trends in Guillain-Barré syndrome mortality in Spain from 1999 to 2013.

BACKGROUND: Guillain-Barré syndrome (GBS) is a rare disease that consists of a group of neuropathic conditions. Very few epidemiological studies of GBS have been carried out in Spain. The aim of this study was to determine the trends in GBS mortality in the total population of Spain for the period 1999 to 2013. METHODS: Data on GBS deaths were drawn from the National Statistics Institute of Spain. Crude and overall age-standardised GBS mortality rates were calculated and joinpoint regression models were used to describe trend changes. Mean age of deceased by GBS each year was also assessed. RESULTS: The overall age-standardised GBS mortality rate was 0.71 per million in 1999 and 0.40 in 2013. It was higher in men, 1.08 vs. 0.42 in 1999 and 0.48 vs. 0.35 in 2013. There was a statistically significant decrease in mortality during the study period. All the age-standardised mortality rates decreased (overall and by gender) from 1999 to 2013. The mean age at death increased with time, from 73 years in 1999 to 77 years in 2013. CONCLUSIONS: GBS mortality has improved in Spain during the last 15 years. The age of death has risen and the mortality rate has decreased.

Outcome of Guillain-Barre syndrome patients with respiratory paralysis.

BACKGROUND AND AIMS: To evaluate the outcome of patients with Guillain -: Barre syndrome (GBS) having respiratory failure treated with modified intubation policy. DESIGN AND METHODS: Consecutive patients with GBS having single breath count below 12 and respiratory rate >30/min were included and their clinical details noted. The patients were intubated and mechanically ventilated (MV) if their PaO2 was <60 mmHg on venturi mask, PaCo2 > 50 mmHg or pH < 7.3. Their electrophysiological subtypes and complications were noted. The hospital mortality and 3 months outcome were compared in MV and those could be managed without MV even with respiratory compromise. RESULTS: Out of 369 patients, 102 (27.6%) patients had respiratory compromise who were included in this study. Of the patients with respiratory compromise, 44 (43.1%) were intubated and mechanically ventilated after a median of 4 days of hospitalization. The median duration of MV was 21 (range 1-88) days. The patients with autonomic dysfunction (56.8% vs. 19%), facial weakness (78% vs. 36.2%), bulbar weakness (81.8% vs. 31%), severe weakness (63.8% vs. 31%) and high transaminase level (47.7% vs. 25.9%) needed MV more frequently. In our study, 6.8% patients died and 26.6% had poor outcome which was similar between MV and non-MV patients. The MV patients had longer hospitalization and more complications compared with non-MV group. CONCLUSION: In GBS patients with respiratory compromise, conservative intubation does not increase mortality and disability.

[Features of Guillain-Barre syndrome in adults: results of a university hospital]. / Características del síndrome de Guillain-Barré en adultos: resultados de un hospital universitario.

BACKGROUND: Guillain-Barré syndrome (GBS) has a cosmopolitan distribution. Its course is usually mild and tends to limit itself, but in severe cases it can cause death. The aim of this article is to describe the characteristics of a group of adults with GBS diagnosed and treated at a university hospital. METHODS: All cases of GBS that occurred between January 1, 2005 to December 31, 2009 were analyzed. The clinical records were reviewed through the implementation of a structured survey that included the following sections: patient identification, clinical data, history of infection, season of occurrence, rates of electrophysiological variants, and lethality. RESULTS: A total of 45 patients were included; the male to female ratio was 1.4: 1 and the mean age was 48.2 ± 16.0 years. The season with the highest number of cases was the summer. History of intestinal infection was present in 40 % of patients, and respiratory infection in 24.4%. The lethality associated with GBS was 11.1 % (95 % CI, 4.4-23.9), fatal cases occurred in patients older than the survivors (65.2 ± 15.0 versus 46.0 ± 14.9, p = 0.01). The most common variant was acute motor axonal neuropathy (64.4 %); there were four cases of Miller Fisher syndrome. CONCLUSIONS: The most common electromyographic variant of GBS was acute motor axonal neuropathy. The highest number of cases was observed during the summer season.

Introducción: el Síndrome de Guillain-Barré (SGB) tiene una distribución cosmopolita. Usualmente su curso es benigno y tiende a autolimitarse, pero en casos severos puede ocasionar la muerte. El objetivo de este artículo es describir las características de un grupo de adultos con SGB diagnosticados y tratados en un hospital universitario. Métodos: se analizaron todos los casos de SGB ocurridos del 1 de enero de 2005 al 31 de diciembre de 2009. Se revisaron los expedientes clínicos a través de la aplicación de una cédula estructurada que comprendió las siguientes secciones: identificación del paciente, datos clínicos, historia de infección, estación del año de ocurrencia, tipos de variantes electrofisiológicas y letalidad. Resultados: en total se incluyeron 45 pacientes; la relación hombre-mujer fue de 1.4:1 y la edad media del grupo fue 48.2 ± 16.0 años. La estación del año con mayor número de casos fue el verano. La historia de infección intestinal estuvo presente en 40 % de los pacientes, y la infección respiratoria en 24.4 %. La letalidad asociada con SGB fue de 11.1 % (IC: 95 %, 4.4-23.9), los casos fatales se presentaron en los pacientes con mayor edad que los que sobrevivieron (65.2 ± 15.0 frente a 46.0 ± 14.9, p = 0.01). La variante más frecuente fue la neuropatía axonal motora aguda (64.4 %); hubo cuatro casos de síndrome de Miller Fisher. Conclusiones: la variante electromiográfica más frecuente del SGB fue la neuropatía axonal motora aguda. Se observó mayor número de casos durante la estación de verano.

The value of comparing mortality of Guillain-Barré syndrome across different regions.

OBJECTIVE: To study the clinical profile of Guillain-Barré syndrome (GBS) patients who died in 4 Asian countries in order to understand factors underlying any variation in mortality. METHODS: Retrospectively reviewed medical records of GBS patients who died in 7 hospitals from 4 Asian countries between 2001 and 2012. Baseline characteristics, timing and causes of death were recorded. RESULTS: A total of 16 out of 261 GBS patients died. The overall mortality rate was 6%, with a range of 0 to 13%. The leading causes of death were respiratory infections, followed by myocardial infarction. The median age of our patients was 77 years. Half of the patients required mechanical ventilation and almost all had significant concomitant illnesses. A disproportionate number of patients in the Hong Kong cohort died (13%). Patients with advanced age, fewer antecedent respiratory infections and need for mechanical ventilation were at most risk. Most deaths occurred during the plateau phase of GBS and on the general ward after having initially received intensive care. CONCLUSIONS: There is considerable variability in mortality of GBS among different Asian cohorts. Although the risks factors for mortality were similar to Western cohorts, the timing and site of death differed. This allows specific measures to be implemented to improve GBS care in countries with higher mortality.