Iranian clinical practice guideline for amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegeneration involving motor neurons. The 3-5 years that patients have to live is marked by day-to-day loss of motor and sometimes cognitive abilities. Enormous amounts of healthcare services and resources are necessary to support patients and their caregivers during this relatively short but burdensome journey. Organization and management of these resources need to best meet patients' expectations and health system efficiency mandates. This can only occur in the setting of multidisciplinary ALS clinics which are known as the gold standard of ALS care worldwide. To introduce this standard to the care of Iranian ALS patients, which is an inevitable quality milestone, a national ALS clinical practice guideline is the necessary first step. The National ALS guideline will serve as the knowledge base for the development of local clinical pathways to guide patient journeys in multidisciplinary ALS clinics. To this end, we gathered a team of national neuromuscular experts as well as experts in related specialties necessary for delivering multidisciplinary care to ALS patients to develop the Iranian ALS clinical practice guideline. Clinical questions were prepared in the Patient, Intervention, Comparison, and Outcome (PICO) format to serve as a guide for the literature search. Considering the lack of adequate national/local studies at this time, a consensus-based approach was taken to evaluate the quality of the retrieved evidence and summarize recommendations.

Efficacy and safety of decompressive craniectomy in acute ischemic stroke patients treated with intravenous thrombolysis.

INTRODUCTION: The optimal timing for decompressive hemicraniectomy (DHC) after intravenous thrombolysis (IVT) remains unclear. This study in patients with acute ischemic stroke treated with IVT aimed to assess the safety of DHC and patient outcome. METHODS: Data was extracted from the Tabriz stroke registry from June 2011 up to September 2020. In all, 881 patients were treated with IVT. Among these, 23 patients underwent DH. Six patients were excluded due to symptomatic intracranial hemorrhage (parenchymal hematoma type 2 based on SITS-MOST definition) after IVT, but other types of bleeding after venous thrombolysis, including HI1, HI2, and PH1 were not excluded; so the remaining 17 patients were enrolled in the study. Functional Outcome was defined as the proportion of patients who achieved mRS score of 2-3 (moderate disability), 4-5 (severe disability), or 6 (mortality) at 90 days after stroke. mRSwas assess by trained neurologist at the hospital clinic with direct interview Safety outcome was assessed by comparison of two scans just prior to and after craniectomy. Any new hemorrhage or worsening of previous hemorrhage was reported. Parenchymal hematoma type 2, based on ECASS II definition, was considered as major surgical complication. This study was approved by the local ethics committee of the Tabriz University of Medical Sciences (Ethics Code: IR.TBZMED.REC.1398.420). RESULTS: At the three-month mRS follow up, six patients (35%) had moderate and five (29%) had severe disability. The outcome of death was observed in six patients (35%).Nine of 15 patients (60%) underwent surgery in the first 48 hours after onset of symptoms. No patient over 60 years of age survived to the three-month follow up; 67% of those who were under60 years and underwent DH in the first 48 hours had favorable outcome. Hemorrhagic complication was seen in 64% of patients but none was major. CONCLUSION: Results of this study showed that the rate of major bleeding and outcome of acute ischemic stroke patients who underwent DHC after IVT is comparable with the reported data in the literature and intentionally waiting for the fibrinolytic effects of IVT to disappear may not outweigh the benefits of DHC. Although the findings of the study should be interpreted with caution and larger studies are needed to confirm the results.

Sleep status in multiple sclerosis: Role of vitamin D and body mass index.

Background: Sleep disorders are major but neglected symptoms in patients with multiple sclerosis (MS). This study aimed to describe the sleep status in patients with MS. Methods: We selected mildly-disabled [Expanded Disability Status Scale (EDSS) score < 4] patients with relapsing-remitting MS (RRMS). After determining the level of vitamin D in a blood sample of the patients, the validated Persian versions of Pittsburgh Sleep Quality Index (PSQI), Insomnia Severity Index (ISI), and snoring, tiredness, observed apnea, high blood pressure, body mass index (BMI), age, neck circumference, and gender (STOP-Bang) questionnaires were filled and the sleep condition was described. Besides, the impact of age, sex, disease duration, and EDSS on sleep status was determined. Results: 37.87% of 103 included patients with MS had poor sleep quality. 21.35% rate of subthreshold, 10.67% rate of moderate, and 1.94% rate of severe insomnia were also observed. Only 1.94% of patients had a high risk of obstructive sleep apnea (OSA). There was a significant relation between Beck Depression Inventory (BDI) score with ISI (r = 0.45, P < 0.01), PSQI (r = 0.53, P < 0.01), and STOP (r = 0.20, P = 0.03). A significant correlation between STOP with BMI (r = 0.24, P = 0.01) and age (r = 0.21, P = 0.03) was also observed. Sleep status was not significantly different in groups of the patients based on vitamin D, overweight, or sex. Conclusion: Poor sleep quality is a common finding among mildly-disabled patients with MS. There is also a 33.99% rate of subthreshold or clinical insomnia in different severities. Quality of sleep and insomnia is not significantly correlated to BMI, level of vitamin D, and sex in patients with MS.

Autosomal recessive cardiomyopathy and sudden cardiac death associated with variants in MYL3.

PURPOSE: Variants in genes encoding sarcomeric proteins are the most common cause of inherited cardiomyopathies. However, the underlying genetic cause remains unknown in many cases. We used exome sequencing to reveal the genetic etiology in patients with recessive familial cardiomyopathy. METHODS: Exome sequencing was carried out in three consanguineous families. Functional assessment of the variants was performed. RESULTS: Affected individuals presented with hypertrophic or dilated cardiomyopathy of variable severity from infantile- to early adulthood-onset and sudden cardiac death. We identified a homozygous missense substitution (c.170C>A, p.[Ala57Asp]), a homozygous translation stop codon variant (c.106G>T, p.[Glu36Ter]), and a presumable homozygous essential splice acceptor variant (c.482-1G>A, predicted to result in skipping of exon 5). Morpholino knockdown of the MYL3 orthologue in zebrafish, cmlc1, resulted in compromised cardiac function, which could not be rescued by reintroduction of MYL3 carrying either the nonsense c.106G>T or the missense c.170C>A variants. Minigene assay of the c.482-1G>A variant indicated a splicing defect likely resulting in disruption of the EF-hand Ca2+ binding domains. CONCLUSIONS: Our data demonstrate that homozygous MYL3 loss-of-function variants can cause of recessive cardiomyopathy and occurrence of sudden cardiac death, most likely due to impaired or loss of myosin essential light chain function.

Vitamin D receptor gene polymorphisms and the risk of the type 1 diabetes: a meta-regression and updated meta-analysis.

BACKGROUND: The association between the polymorphisms in the vitamin D receptor (VDR) gene and the risk of type 1 diabetes mellitus (T1DM) has been evaluated in several studies. However, the findings were inconclusive. Thus, we conducted a meta-analysis to comprehensively evaluate the effect of VDR gene polymorphisms on the risk of T1DM. METHODS: All relevant studies reporting the association between VDR gene polymorphisms and susceptibility to T1DM published up to May 2020 were identified by comprehensive systematic database search in ISI Web of Science, Scopus, and PubMed/MEDLINE. Strength of association were assessed by calculating of pooled odds ratios (ORs) and 95% confidence intervals (CIs). The methodological quality of each study was assessed according to the Newcastle-Ottawa Scale. To find the potential sources of heterogeneity, meta-regression and subgroup analysis were also performed. RESULTS: A total of 39 case-control studies were included in this meta-analysis. The results of overall population rejected any significant association between VDR gene polymorphisms and T1DM risk. However, the pooled results of subgroup analysis revealed significant negative and positive associations between FokI and BsmI polymorphisms and T1DM in Africans and Americans, respectively. CONCLUSIONS: This meta-analysis suggested a significant association between VDR gene polymorphism and T1DM susceptibility in ethnic-specific analysis.

Predictors of long-term mortality after first-ever stroke.

BACKGROUND AND OBJECTIVE: Data on the factors affecting long-term mortality following a stroke in Iran are scarce. The current research aimed at investigating the extent of 2-year mortality following a stroke and the factors affecting it in the northwest of Iran. MATERIALS AND METHODS: This prospective cohort study was conducted in Tabriz, Northwest of Iran. Patients with computed tomography/magnetic resonance imaging confirmed the first-ever stroke were included in this study and followed up to 2 years. Clinical examinations, including the severity of the stroke using the modified National Institutes of Health Stroke Scale (mNIHSS), were conducted by a neurologist. The general characteristics, lifestyle factors, and laboratory tests were also completed. To estimate the survival, Kaplan-Meier analysis was used; and for group comparison, the log-rank method was applied. To identify the factors predicting 2-year mortality, semiparametric Cox regression analysis was used. RESULTS: A total of 1036 first-ever stroke patients were included in the present study. The mortality rates of stroke in 6-month, 1-year, and 2-years follow-up periods were 31.6%, 34.5%, and 38%, respectively. The two-year mortality rate was 33.6% in ischemic and 58.7% in hemorrhagic stroke (P < 0.001). In the multivariate Cox model, variables age, type of stroke, diabetes, and severity of the stroke, according to the mNIHSS index, were identified as factors predicting 2-year mortality following the stroke. CONCLUSION: The 2-year mortality following acute stroke was relatively high compared to that of in developed countries. Implementation of secondary prevention is recommended to better management of modifiable predictors of mortality.

Factors related to 6-month mortality after the first-ever stroke.

BACKGROUND AND OBJECTIVE: Stroke is the second leading cause of death worldwide and the number of stroke cases has increased remarkably over the last 20 years. This study aimed at identifying predictors of with 6-month mortality of first-ever stroke patients and the factors contributing to it in East Azerbaijan province. MATERIALS AND METHODS: A closed cohort study was carried out from April 2014 to December 2014. All cases of first-ever diagnosed stroke were included in the study. Any transient ischemic attack, silent brain infarctions, and the stroke cases which were neither associated with trauma, blood disease nor with malignancy were excluded from the study. The variables of this study include participants' demographic characteristics, stroke severity National Institutes of Health Stroke Scale (NIHSS), and stroke risk factors. Patients were followed up within 6 months. To determine the survival time, the log-rank method was applied to compare intergroup differences. The tests include the univariate and multivariate analysis Cox regression. P < 0.05 were considered as statistically significant. RESULTS: A total of 576 cases of stroke were included in this study. Average age of ischemic and hemorrhagic stroke was 70.15 ± 13.0 and 67.79 ± 12.69, respectively. Case-fatality rate (CFR) of stroke patients was 49.2 and 21.7% in hemorrhagic and ischemic stroke types, respectively. Factors contributing to stroke mortality events include the severity of stroke (NIHSS categories 15-19 and ≥20), age over 65, being female, high body mass index and hyperlipidemia. In the final model, the severity of stroke (with NIHSS 15-19 with hazard ratio (HR) 4.22 (95% confidence interval [CI] 2.36-7.56) and NIHSS ≥20 with HR 5.34 (95% CI: 2.81-10.12) and age above 65 with HR 1.61 (95% CI: 1.02-2.51) were the most important predictors of 6-month mortality. CONCLUSION: Severity of stroke by NIHSS was the most prominent factor in stroke patients' mortality. By increasing the follow-up time, a better evaluation of the predictors of mortality after stroke can be achieved.

Prognostic factors in patients with acute ischemic stroke treated with intravenous tissue plasminogen activator: The first study among Iranian patients.

Background: Tissue plasminogen activator (tPA) has been long approved as an efficacious treatment in patients with acute ischemic stroke (AIS); however, due to some serious complications, particularly intracranial hemorrhage (ICH), many physicians are still reluctant to use it liberally. This study sought to find potential prognostic factors in patients with AIS treated with tPA. Methods: A retrospective, hospital-bases observational study was conducted. Consecutively, a total of 132 patients with AIS treated with intravenous tPA, form June 2011 to July 2015 were enrolled. Inclusion and exclusion criteria were based on updated guidelines. Probable prognostic variables were examined separately in three distinct groups; the occurrence of ICH within 24 hours after treatment, poor 3-month outcome on the basis of modified Rankin Scale (mRS) and 3-month mortality. Results: Patients were 83 men (62.9%) and 49 women (37.1%) with a median age of 66 years [interquartile range (IQR)of 55-72]. Any type of hemorrhage, symptomatic hemorrhage [based on the European Cooperative Acute Stroke Study III (ECASS III) definition] within 24 hours posttreatment, poor 3-month outcome (mRS 3-6), and 3-month mortality were documented in 10.6%, 4.5%, 53.2%, and 23.6% of patients, respectively. Increased baseline blood glucose was a significant but dependent predictor of hemorrhage within the first 24 hours posttreatment. Dependent predictors of a 3-month poor outcome were high age, the National Institutes of Health Stroke Scale (NIHSS) at baseline, decreased admitting glomerular filtration rate (GFR), and the presence of atrial fibrillation (AF) rhythm, and ICH within 24 hours posttreatment. Only age [Odds ratio (OR) adjusted 1.05] and initial NIHSS (OR adjusted 1.23), however, were recognized as the independent variables in this regard. The only independent predictor of 3-month mortality was the initial NIHSS (OR adjusted 1.18). Conclusion: According to the findings of the present study, advanced age and high baseline NIHSS are two independent prognostic factors in patients with AIS treated with tPA.

Renal Dysfunction Is an Independent Risk Factor for Poor Outcome in Acute Ischemic Stroke Patients Treated with Intravenous Thrombolysis: A New Cutoff Value.

Objective. This study was set to assess the effect of renal dysfunction on outcome of stroke patients treated with intravenous thrombolysis (IVT). Methods. This multicenter research involved 403 patients from January 2009 to March 2015. Patients were divided into two groups: (1) control group with GFR ≥ 45 mL/min/1.73 m2 and (2) low GFR group with GFR < 45 mL/min/1.73 m2. Outcome measurements were poor outcome (mRS 3-6) and mortality at 3 months and symptomatic intracerebral hemorrhage (SICH) within the first 24-36 hours. Univariate and multivariate regression analyses were performed, and odds ratios (ORs) were determined at 95% confidence intervals (CIs). Results. Univariate analyses determined that every decrease of GFR by 10 mL/min/1.73 m2 significantly increased the risk of poor outcome (OR 1.19, 95% CI 1.09-1.30, p < 0.001) and mortality (OR 1.18, 95% CI 1.06-1.32, p = 0.002). In multivariate regression, adjusted for all variables with p value < 0.1, low GFR (GFR < 45 versus GFR equal to or more than 45) was associated with poor outcome (OR adjusted 2.15, 95% CI 1.01-4.56, p = 0.045). Conclusion. In IVT for acute stroke, renal dysfunction with GFR < 45 mL/min/1.73 m2 before treatment determined increased odds for poor outcome compared to GFR of more than 45 mL/min/1.73 m2.

Correlation between optic nerve involvement and chronic obstructive pulmonary disease.

BACKGROUND: Chronic obstructive pulmonary disease (COPD) is a major public health problem worldwide. The aim of this study was to evaluate the rate of optic neuropathy in COPD patients. METHODS: Forty patients diagnosed with COPD and 60 healthy subjects as control group enrolled. After examination by a pulmonary subspecialist, patients were ranked by Global initiative for chronic Obstructive Lung Disease (GOLD) criteria, and patients with zero grades on GOLD criteria were excluded. Visual evoked potential by checkerboard (raster background) method with a frequency of 2 Hz were done for all participants. P-values less than 0.05 were considered as significant. RESULTS: Fifty-five percent of COPD patients had visual evoked potential abnormalities. Mean P100 latency in both eyes was significantly longer in COPD patients. Average P100/N140 amplitude in both eyes were insignificantly higher in COPD. CONCLUSION: Higher P100 latency in COPD patients shows demyelinating type of optic nerve involvement; however, further investigation in this area is needed.

Designing a Low-noise, High-resolution, and Portable Four Channel Acquisition System for Recording Surface Electromyographic Signal.

In current years, the application of biopotential signals has received a lot of attention in literature. One of these signals is an electromyogram (EMG) generated by active muscles. Surface EMG (sEMG) signal is recorded over the skin, as the representative of the muscle activity. Since its amplitude can be as low as 50 µV, it is sensitive to undesirable noise signals such as power-line interferences. This study aims at designing a battery-powered portable four-channel sEMG signal acquisition system. The performance of the proposed system was assessed in terms of the input voltage and current noise, noise distribution, synchronization and input noise level among different channels. The results indicated that the designed system had several inbuilt operational merits such as low referred to input noise (lower than 0.56 µV between 8 Hz and 1000 Hz), considerable elimination of power-line interference and satisfactory recorded signal quality in terms of signal-to-noise ratio. The muscle conduction velocity was also estimated using the proposed system on the brachial biceps muscle during isometric contraction. The estimated values were in then normal ranges. In addition, the system included a modular configuration to increase the number of recording channels up to 96.

Laryngeal ultrasonography versus cuff leak test in predicting postextubation stridor.

INTRODUCTION: Although cuff leak test has been proposed as a simple method of predicting the occurrence of postextubation stridor, cut-off point of cuff-leak volume substantially differs between previous studies. In addition, laryngeal ultrasonography including measurement of air column width could predict postextubation stridor. The aim of the present study was to evaluate the value of laryngeal ultrasonography versus cuff leak test in predicting postextubation stridor. METHODS: In a prospective study, all patients intubated for a minimum of 24 h for acute respiratory failure, airway protection and other causes were included. Patients were evaluated for postextubation stridor and need for reintubation after extubation. The cuff leak volume was defined as a difference between expiratory tidal volumes with the cuff inflated and deflated. Laryngeal air column width was defined as the width of air passed through the vocal cords as determined by laryngeal ultrasonography. The air-column width difference was the width difference between balloon-cuff inflation and deflation. RESULTS: Forty one intubated patients with the mean age of 57.16±20.07 years were included. Postextubation stridor was observed in 4 patients (9.75%). Cuff leak test (cut off point: 249 mL) showed sensitivity and specificity of 75% and 59%, respectively. In addition, laryngeal ultrasonography (cut off point for air column width: 10.95 mm) resulted in sensitivity and specificity of 50% and 54%, respectively. Positive predictive value of both methods were <20%. CONCLUSION: Both cuff leak test and laryngeal ultrasonography have low positive predictive value and sensitivity in predicting postextubation stridor and should be used with caution in this regard.

P wave duration and dispersion in Holter electrocardiography of patients with obstructive sleep apnea.

PURPOSE: The underlying mechanisms of the association between obstructive sleep apnea (OSA) and atrial fibrillation (AF) remained unclear. We investigated P wave parameters as indicators of atrial conduction status among OSA patients. METHODS: We studied 42 untreated OSA patients, categorized into mild (6), moderate (18), and severe (18) OSA based on the apnea/hypopnea index (AHI) and 18 healthy controls. Twenty-four-hour Holter electrocardiography was applied to measure P wave parameters including P wave duration and P wave dispersion; difference between the maximum (P-max) and minimum (P-min) measured P wave duration. RESULTS: Mean P wave duration ranged from 110.2 ± 9.3 ms in mild OSA patients to 121.1 ± 15.4 ms in severe OSA patients and was 113.4 ± 10.0 ms in controls with no significant difference among the groups, P = 0.281. P wave dispersion and P-max were significantly longer in those with moderate OSA (68.0 ± 9.3 and 154.2 ± 9.3 ms) and those with severe OSA (71.6 ± 13.7 and 157.2 ± 13.3 ms) than controls (52.6 ± 15.3 and 142.1 ± 15.4 ms), P < 0.05. AHI was significantly correlated with P-max (r = 0.407, P = 0.012) and P wave dispersion (r = 0.431, P = 0.008). With linear regression analysis controlling for age, gender, and BMI, the AHI was independently associated with P wave dispersion (ß = 0.482, P = 0.002). CONCLUSIONS: Using Holter monitoring for measurement of P wave parameters, this study showed an association of OSA with prolonged P-max and P wave dispersion. These results indicate that patients with OSA have disturbances in atrial conduction associated with OSA severity. Repeating this study in a larger sample of patients is warranted.

Comparing the efficacy of botulinum toxin with tizanidine in upper limb post stroke spasticity.

BACKGROUND: This study evaluated the efficacy of focal intramuscular injection of botulinum (BoNT) toxin type A in comparison with oral tizanidine (TZD) in treatment of post-stroke upper limb spasticity. METHODS: This was a double-blinded randomized clinical trial that recruited 68 patients with post-stroke upper limb spasticity. Thirty-four patients received BoNT (Dysport(®)) injections in affected muscles of upper limb at the baseline and week 12. Thirty-four patients were treated with tizanidine (Sirdalude) by gradual increase in dosage of 2mg/week to reach maximum 24mg at week 12. Modified Ashworth Scale (MAS) and Action Research Arm Test (ARAT) were evaluated at the baseline, week 12 and week 24 for all the participants. RESULTS: The mean score of MAS reduced from 3.32 and 3.13 at baseline to 1.79 and 1.56 at week 24 on elbow and wrist joints, respectively (P < 0.01). However, there were only reductions from 2.79 and 2.77 to 2.32 and 2.31 (P < 0.001) in TZD group. ARAT increased from 1.79 to 10.97 (P < 0.001) in BoNT group. ARAT increased from 11.08 to 11.35 in TZD group (P = 0.026). CONCLUSION: BoNT injection was safe and effective in reducing post-stroke upper extremity spasticity in comparison with TZD.

Diagnostic value of ultrasonography and magnetic resonance imaging in ulnar neuropathy at the elbow.

Aim. To evaluate the diagnostic value of ultrasonography and magnetic resonance imaging (MRI) in patients with ulnar neuropathy at the elbow (UNE). Methods. We prospectively performed electrodiagnostic, ultrasonographic, and MRI studies in UNE patients and healthy controls. Three cross-sectional area (CSA) measurements of the ulnar nerve at multiple levels along the arm and maximum CSA(-max) were recorded. Results. The ulnar nerve CSA measurements were different between the UNE severity grades (P < 0.05). CSA-max had the greatest sensitivity (93%) and specificity (68%). Moreover, CSA-max ≥10 mm(2) defined the severe UNE cases (sensitivity/specificity: 82%/72%). In MRI, ulnar nerve hyperintensity had the greatest sensitivity (90%) and specificity (80%). Conclusion. Ultrasonography using CSA-max is sensitive and specific in UNE diagnosis and discriminating the severe UNE cases. Furthermore, MRI particularly targeting at increased signal of the ulnar nerve can be a useful diagnostic test of UNE.

Sensitivity and specificity of median nerve ultrasonography in diagnosis of carpal tunnel syndrome.

BACKGROUND: Although controversial, recent studies have demonstrated advantages of sonographic techniques in the diagnosis of carpal tunnel syndrome (CTS). The purpose of this study was to assess the utility of median nerve ultrasonography in the diagnosis of CTS in Iranian patients. METHODS: Ninety patents with clinically suspected CTS were studied. Based on gold standard electromyography/nerve conduction velocity studies, wrists with CTS were divided into three groups on the basis of severity of CTS, ie, mild, moderate, and severe. In addition, both sides of the wrist were examined using sonography. Transverse images of the median nerve were obtained and median nerve cross-section areas were measured at three levels, ie, immediately proximal to the carpal tunnel inlet, at the carpal tunnel inlet, and at the carpal tunnel outlet. Furthermore, flexor retinaculum thickness was evaluated. RESULTS: The mean age of the studied patients was 48.52 ± 12.17 years. Median values of the median nerve cross-section at the carpal tunnel inlet, carpal tunnel outlet, and proximal carpal tunnel significantly differed between the wrists with and without CTS (P < 0.05). Comparisons between the CTS groups (mild, moderate, and severe) and non-CTS wrists demonstrated that the median cross-sections of median nerve at the carpal tunnel inlet, carpal tunnel outlet, and inlet proximal carpal tunnel were significantly greater in the severe CTS group than in the other three groups (P < 0.05). The results showed that the median nerve cross-section at the three levels of carpal tunnel could only fairly differentiate severe CTS from other cases. CONCLUSION: The present study demonstrated that median nerve ultrasonography cannot replace the gold standard test (nerve conduction velocity) for the diagnosis of CTS because of low overall sensitivity and specificity, although it might provide useful information in some patients.

Epidemiology and characteristics of Guillain-Barré syndrome in the northwest of Iran.

BACKGROUND: Little is known about the incidence of Guillain-Barré syndrome (GBS) in Iran. We determined the incidence and evaluated prognostic factors for GBS in a prospective, population-based study. PATIENTS AND METHODS: We evaluated and followed all patients with a diagnosis of GBS admitted to three referral neurology centers in East Azerbaijan province over a 1-year period (2003). Clinical and electrophysiological characteristics of cases were reviewed and analyzed. RESULTS: A total of 76 patients were found, corresponding to a crude annual incidence rate of 2.11/100 000 population. Six patients (7.9%) died acutely within 21 days from the onset of the disease. Acute mortality was due to respiratory involvement, sepsis and acute autonomic system dysfunction. The persistence of disability after the acute phase was related to axonal involvement (OR=3.19, 95%CI, 1.65 to 6.16). There was a significant correlation between a history of diarrhea and a further need for mechanical ventilation (P<0.05). Mechanically ventilated patients had a low GBS disability score on discharge compared with patients not mechanically ventilated (P=0.05). CONCLUSIONS: The incidence rates of GBS in our province is similar to that in other countries. Acute mortality in GBS was mostly due to poor respiratory care of patients and infective complications, but disability and probably late mortality were due to axonal nerve injury.