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Published Article by Badrul Islam
Guillain-Barré syndrome (GBS) is a post-infectious autoimmune polyneuropathy regulated by pro- an... more Guillain-Barré syndrome (GBS) is a post-infectious autoimmune polyneuropathy regulated by pro- and anti-inflammatory cytokines; TNFA polymorphisms may exert immune pathogenic roles in GBS. We assessed TNFA promoter region polymorphisms (-238G/A, -308G/A, -857C/T, -863C/A) in Bangladeshi patients with GBS (n = 300) and healthy controls (n = 300) by PCR-RFLP and ASO-PCR. TNFA -863CA was significantly associated with GBS disease susceptibility (P = 0.0154) and disease severity (P = 0.0492). TNFA -238A allele was more frequent among anti-ganglioside (GM1) antibody-positive patients (P = 0.0092) and -863AA associated with AMAN subtype of GBS (P = 0.0398). TNFA -863C/A may contribute to GBS severity and pathogenesis in Bangladeshi patients.
Papers by Badrul Islam
Research Square (Research Square), Nov 10, 2020
Background: SARS-CoV2 virus could be potentially myopathic. Serum creatinine phosphokinase (CPK) ... more Background: SARS-CoV2 virus could be potentially myopathic. Serum creatinine phosphokinase (CPK) is frequently found elevated in severe SARS-CoV2 infection, which indicates skeletal muscle damage precipitating limb weakness or even ventilatory failure. Case presentation: We addressed such a patient in his forties presented with features of severe SARS-CoV2 pneumonia and high serum CPK. He developed severe sepsis and acute respiratory distress syndrome (ARDS) and received intravenous high dose corticosteroid and Tocilizumab to counter SARS-CoV2 associated cytokine surge. After 10 days of mechanical ventilation (MV) weaning was unsuccessful albeit apparently clear lung fields, having additionally severe and symmetric limb muscle weakness. Ancilliary investigations in addition with serum CPK, including electromyogram, muscle biopsy and muscle magnetic resonance imaging (MRI) suggested acute myopathy possibly due to skeletal myositis. Conclusion: We wish to stress that myopathogenic medication in SARS-CoV2 pneumonia should be used with caution. Additionally serum CPK could be a potential marker to predict respiratory failure in SARS-CoV2 pneumonia as skeletal myopathy affecting chest muscles may contribute ventilatory failure on top of oxygenetion failure due to SARS-CoV2 pneumonia.
Journal of neuroimmunology, Jan 15, 2017
Guillain-Barré syndrome (GBS) is a post-infectious autoimmune polyneuropathy regulated by pro- an... more Guillain-Barré syndrome (GBS) is a post-infectious autoimmune polyneuropathy regulated by pro- and anti-inflammatory cytokines; TNFA polymorphisms may exert immune pathogenic roles in GBS. We assessed TNFA promoter region polymorphisms (-238G/A, -308G/A, -857C/T, -863C/A) in Bangladeshi patients with GBS (n=300) and healthy controls (n=300) by PCR-RFLP and ASO-PCR. TNFA -863CA was significantly associated with GBS disease susceptibility (P=0.0154) and disease severity (P=0.0492). TNFA -238A allele was more frequent among anti-ganglioside (GM1) antibody-positive patients (P=0.0092) and -863AA associated with AMAN subtype of GBS (P=0.0398). TNFA -863C/A may contribute to GBS severity and pathogenesis in Bangladeshi patients.
OBJECTIVE: To assess the safety and feasibility of small volume plasma exchange (SVPE) as an alte... more OBJECTIVE: To assess the safety and feasibility of small volume plasma exchange (SVPE) as an alternative to standard plasma exchange (PE) or intravenous immunoglobulin (IVIg) for Guillain-Barré syndrome (GBS) patients. DESIGN: Non-randomized, single arm, interventional trial. SETTING: National Institute of Neurosciences and Hospital, Dhaka, Bangladesh. PARTICIPANTS: Twenty adult (>18 years) patients with GBS presented within 2 weeks of onset of weakness who were unable to walk unaided for more than 10 meters. INTERVENTIONS: SVPE involves blood cell sedimentation in a blood bag and removal of supernatant plasma after blood cells are re-transfused. This procedure was repeated three to six times a day, for eight consecutive days. OUTCOME MEASURES: Serious adverse events (SAE) were defined as severe sepsis and deep venous thrombosis related to the central vein catheter (CVC) used during SVPE. SVPE was considered safe if less than 5/20 patients experienced a SAE, and feasible if 8 L plasma could be removed within 8 days in at least 15/20 patients. RESULTS Median patient age 33 years (IQR 23-46; range 18-55); 13 (65%) were male. Median MRC sum score was 20 (IQR 0-29; range 0-36); three (15%) patients required mechanical ventilation. One patient developed SAE (severe sepsis, possibly related to CVC). Minor adverse effects were transient hypotension in 10 (50%) patients; CVC-associated bleeding in 10 (50%); transfusion reaction to fresh frozen plasma in 4 (20%); and hypo-albuminemia, anaemia or electrolyte imbalance in 4 (20%). Removal of 8 L plasma was possible in 15 (75%) patients. GBS disability score improved by at least one grade in 14 (70%) patients four weeks after SVPE started. No patients died. CONCLUSION: SVPE seems a safe and feasible alternative treatment to standard PE or IVIg for GBS; further studies of clinical efficacy in low-resource developing countries are warranted. TRIAL REGISTRATION: Clinicaltrials.gov NCT02780570 on May 23, 2016. Strength and limitations of the study: 1. The strength of this study underlies the novel and simple technique of SVPE, which is much less expensive than conventional immunotherapies (plasma exchange and intravenous immunoglobulin). 2. SVPE is corroborated as safe and feasible for the first time in a prospective and standardized cohort of patients with Guillain-Barré syndrome (GBS). 3. The intrinsic limitations of this study are its non-randomized, single arm nature, which is conducted in a single center with a limited sample size of GBS patients. 4. Clinical efficacy of SVPE on patients with GBS was a secondary end-point assessment and therefore deserves a randomized controlled trial in future to assess the clinical efficacy of SVPE for the patients with GBS
Clinical Neurophysiology Practice, 2021
Objective: To describe the electrophysiological features in relation to clinical and serological ... more Objective: To describe the electrophysiological features in relation to clinical and serological findings of Guillain-Barré syndrome (GBS) in the national neuroscience hospital in Bangladesh. This is one of the few studies that investigated GBS patients using standardized electrophysiology in low-income countries. Methods: In a prospective and observational study, we investigated 312 GBS patients by standardized clinical, serological and electrophysiological methods. Unilateral motor and sensory nerve conduction studies (NCS) were performed within two weeks of onset of weakness. Follow up NCS were performed in 189 patients and classified according to eight sets of established GBS criteria. Serology included assessment of anti-GM1 antibodies and anti-campylobacter jejuni lipo-oligosaccharide (LOS) antibodies. Results: Depending on the criteria used, 44-59% patients had axonal GBS with anti-GM1 antibodies being present in 55-58% and 9-42% patients had demyelinating GBS with anti-GM1 antibodies being present in 7-35%. Conduction block (CB) with demyelinative slowing in the same nerve segment was found in 24% (74/312) patients, and CB without demyelinative slowing in the same nerve segment was found in 18% (56/312) patients, of whom anti-GM1 antibodies were found in 27% and 57% patients respectively. Follow-up NCS showed a change in GBS classification in 11-26% of patients, mainly from demyelinating to axonal GBS. Conclusions: The predominant subtype of GBS in Bangladesh is axonal but demyelinating GBS also occurs with classification being strongly dependent on the applied criteria. Significance: The present study demonstrates the importance of reaching international agreement on GBS criteria that should be based on the best evidence.
Skeletal Muscle
Background SARS-CoV2 virus could be potentially myopathic. Serum creatinine phosphokinase (CPK) i... more Background SARS-CoV2 virus could be potentially myopathic. Serum creatinine phosphokinase (CPK) is frequently found elevated in severe SARS-CoV2 infection, which indicates skeletal muscle damage precipitating limb weakness or even ventilatory failure. Case presentation We addressed such a patient in his forties presented with features of severe SARS-CoV2 pneumonia and high serum CPK. He developed severe sepsis and acute respiratory distress syndrome (ARDS) and received intravenous high dose corticosteroid and tocilizumab to counter SARS-CoV2 associated cytokine surge. After 10 days of mechanical ventilation (MV), weaning was unsuccessful albeit apparently clear lung fields, having additionally severe and symmetric limb muscle weakness. Ancillary investigations in addition with serum CPK, including electromyogram, muscle biopsy, and muscle magnetic resonance imaging (MRI) suggested acute myopathy possibly due to skeletal myositis. Conclusion We wish to stress that myopathogenic medic...
Journal of the Peripheral Nervous System
Journal of the Peripheral Nervous System
Journal of the Peripheral Nervous System
Nature Reviews Neurology
Guillain–Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the pe... more Guillain–Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and 2015 in Latin America. Diagnosis and management of GBS can be complicated as its clinical presentation and disease course are heterogeneous, and no international clinical guidelines are currently available. To support clinicians, especially in the context of an outbreak, we have developed a globally applicable guideline for the diagnosis and management of GBS. The guideline is based on current literature and expert consensus, and has a ten-step structure to facilitate its use in clinical practice. We first provide an introduction to the diagnostic criteria, clinical variants and differential diagnoses of GBS. The ten steps then cover early recognition and di...
Journal of Neurology, Neurosurgery & Psychiatry
Chemotherapy-induced peripheral neurotoxicity (CIPN) is a common dose-limiting side effect of sev... more Chemotherapy-induced peripheral neurotoxicity (CIPN) is a common dose-limiting side effect of several anticancer medications. CIPN may involve multiple areas of the peripheral nervous system from the autonomic and dorsal root ganglia (DRG) to the axon and any peripheral nerve fibre type. Large diameter sensory myelinated (Aβ) fibres are more frequently involved, but motor, small myelinated (Aδ), unmyelinated (C) or autonomic fibres may also be affected. Here, we review the current evidence on techniques for the CIPN assessment in the clinical and experimental settings. Nerve conduction studies (NCS) may be used at the subclinical and early CIPN stage, to assess the extent of large nerve fibre damage and to monitor long-term outcomes, with the sural or dorsal sural nerve as the most informative. The quantitative sensory neurological examination provides valuable data alongside NCS. Quantitative sensory testing and nerve excitability studies add information regarding pathophysiology. ...
Therapeutic Apheresis and Dialysis
Annals of Clinical and Translational Neurology
Objective: TLR4 plays an important role in the pathogenesis of Guillain-Barr e syndrome (GBS). Th... more Objective: TLR4 plays an important role in the pathogenesis of Guillain-Barr e syndrome (GBS). The relationships between TLR4 polymorphisms and susceptibility to GBS are poorly understood. We investigated the frequency and assessed the association of two single nucleotide polymorphisms (SNPs) in the extracellular domain of TLR4 (Asp299Gly and Thr399Ile) with disease susceptibility and the clinical features of GBS in a Bangladeshi cohort. Methods: A total of 290 subjects were included in this study: 141 patients with GBS and 149 unrelated healthy controls. The TLR4 polymorphisms Asp299Gly and Thr399Ile were genotyped using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) assay. Results: The minor 299Gly allele was significantly associated with GBS susceptibility (P = 0.0137, OR = 1.97, 95% CI = 1.17-3.31), and was present at a significantly higher frequency in patients with the acute motor axonal neuropathy (AMAN) subtype of GBS (P = 0.0120, OR = 2.37, 95% CI = 1.26-4.47) than acute inflammatory demyelinating polyneuropathy (AIDP) subtype (P = 0.961, OR = 1.15, 95% CI = 0.38-3.48); when compared to healthy controls. The genotype frequency of the Asp299Gly polymorphism was not significantly different between patients with GBS and healthy controls. The Asp299-Thr399 haplotype was associated with a significantly lower risk of developing GBS (P = 0.0451, OR = 0.63, 95% CI = 0.40-0.99). No association was observed between the Thr399Ile polymorphism and GBS disease susceptibility. Interpretation: The TLR4 minor 299Gly allele was associated with increased susceptibility to GBS and the axonal GBS subtype in the Bangladeshi population. However, no associations were observed between the genotypes of the Asp299Gly and Thr399Ile SNPs and antecedent C. jejuni infection or disease severity in Bangladeshi patients with GBS.
Annals of Clinical and Translational Neurology
Objective: We investigated clinical, biological, and electrophysiological risk factors for mechan... more Objective: We investigated clinical, biological, and electrophysiological risk factors for mechanical ventilation (MV) and patient outcomes in Bangladesh using one of the largest, prospective Guillain-Barr e syndrome (GBS) cohorts in developing world. Methods: A total of 693 GBS patients were included in two GBS studies conducted between 2006 and 2016 in Dhaka, Bangladesh. Associations between baseline characteristics and MV were tested using Fisher's exact test, v 2 test, or Mann-Whitney U-test, as appropriate. Risk factors for MV were assessed using multivariate logistic regression. Survival analysis was performed using Kaplan-Meier method; comparisons between groups performed using logrank test. Results: Of 693 patients, 155 (23%) required MV (median age, 26 years; interquartile range [IQR] 17-40). Among the ventilated patients, males were predominant (68%) than females. The most significant risk factor for MV was bulbar involvement (adjusted odds ratio [AOR]:19.07; 95% CI = 89.00-192.57, P = 0.012). Other independently associated factors included dysautonomia (AOR:4.88; 95% CI = 1.49-15.98, P = 0.009) and severe muscle weakness at study entry (AOR:6.12; 95% CI = 0.64-58.57, P = 0.048). At 6 months after disease onset, 20% of ventilated and 52% of non-ventilated patients (P < 0.001) had recovered completely or with minor symptoms. Mortality rate was significantly higher among ventilated patients than non-ventilated patients (41% vs. 7%, P < 0.001). Interpretation: Bulbar involvement, dysautonomia and severe muscle weakness were identified as the most important risk factors for MV among GBS patients from Bangladesh. The findings may help to develop predictive models for MV in GBS in developing countries to identify impending respiratory failure and proper clinical management of GBS patients.
Brain : a journal of neurology, 2018
Guillain-Barré syndrome is a heterogeneous disorder regarding the clinical presentation, electrop... more Guillain-Barré syndrome is a heterogeneous disorder regarding the clinical presentation, electrophysiological subtype and outcome. Previous single country reports indicate that Guillain-Barré syndrome may differ among regions, but no systematic comparative studies have been conducted. Comparative studies are required to identify factors determining disease susceptibility, variation and prognosis, and to improve diagnostic criteria. The International Guillain-Barré Syndrome Outcome Study is a prospective, observational cohort study including all patients within the diagnostic spectrum, aiming to describe the heterogeneity of Guillain-Barré syndrome worldwide. The current study was based on the first 1000 inclusions with a follow-up of at least 1 year and confirmed the variation in clinical presentation, course and outcome between patients. The full clinical spectrum of Guillain-Barré syndrome was observed in patients from all countries participating in the International Guillain-Barr...
BMJ open, Jan 17, 2018
To assess the safety and feasibility of small volume plasma exchange (SVPE) for patients with Gui... more To assess the safety and feasibility of small volume plasma exchange (SVPE) for patients with Guillain-Barré syndrome (GBS). Non-randomised, single-arm, interventional trial. National Institute of Neurosciences and Hospital, Dhaka, Bangladesh. Twenty adult (>18 years) patients with GBS presented within 2 weeks of onset of weakness who were unable to walk unaided for more than 10 m. SVPE involves blood cell sedimentation in a blood bag and removal of supernatant plasma after blood cells are retransfused. This procedure was repeated three to six times a day, for eight consecutive days. Fresh frozen plasma (FFP) and normal saline were used as replacement fluid. Serious adverse events (SAEs) were defined as severe sepsis and deep venous thrombosis related to the central venous catheter (CVC) used during SVPE. SVPE was considered safe if less than 5/20 patients experienced an SAE, and feasible if 8 L plasma could be removed within 8 days in at least 15/20 patients. Median patient age ...
Annals of clinical and translational neurology, 2018
Previous studies have associated Guillain-Barré syndrome (GBS) with Zika virus (ZIKV) outbreaks i... more Previous studies have associated Guillain-Barré syndrome (GBS) with Zika virus (ZIKV) outbreaks in South America and Oceania. In Asia, ZIKV is known to circulate widely, but the association with Guillain-Barré syndrome is unclear. We investigated whether endemic ZIKV infection is associated with the development of GBS. A prospective study was conducted from 2011 to 2015 in Bangladesh. A total of 418 patients and 418 healthy family controls were included in the study. Patients were diagnosed with GBS prior to inclusion according to established criteria. Detailed information on the epidemiology, clinical presentation, electrophysiology, diagnosis, disease severity, and clinical course were obtained during a follow-up of 1 year using a predefined protocol. ZIKV-neutralizing antibodies were detected in our study from 2013 onwards. The prevalence of ZIKV-neutralizing antibodies was not significantly higher in patients with GBS compared to healthy controls (OR 2.23, = 0.14, 95% CI 0.77-6....
Pilot and feasibility studies, 2017
In Bangladesh, most patients with Guillain-Barré syndrome (GBS) cannot afford standard treatment ... more In Bangladesh, most patients with Guillain-Barré syndrome (GBS) cannot afford standard treatment with intravenous immunoglobulin (IVIG) or a standard plasma exchange (PE) course, which partly explains the high rate of mortality and residual disability associated with GBS in this country. Small volume plasma exchange (SVPE) is an affordable and potentially effective alternative form of plasma exchange. SVPE is the repeated removal of small volumes of supernatant plasma over several days via sedimentation of patient whole blood. The aim of this study is to define the clinical feasibility and safety of SVPE in patients with GBS in resource poor settings. A total of 20 adult patients with GBS will be enrolled for SVPE at a single center in Bangladesh. Six daily sessions of whole blood sedimentation and plasma removal will be performed in all patients with GBS with a target to remove an overall volume of at least 8 liters (L) of plasma over a total of 8 days. Serious adverse events (SAE)...
Journal of the peripheral nervous system : JPNS, Dec 12, 2016
Guillain-Barré syndrome has a diverse clinical phenotype related to geographical origin. To date,... more Guillain-Barré syndrome has a diverse clinical phenotype related to geographical origin. To date, the majority of large-scale studies on Guillain-Barré syndrome (GBS) have been conducted in developed countries. We aimed to evaluate the key diagnostic features and assess the suitability of the Brighton criteria in 344 adult GBS patients from Bangladesh. All patients fulfilled the National Institute of Neurological Diseases and Stroke (NINDS) diagnostic criteria. Standardized data on demographic characteristics and clinical features, cerebrospinal fluid (CSF) analysis and nerve conduction study (NCS) results were elaborated to measure the sensitivity of Brighton criteria. Most patients (88%) were admitted to hospital after the nadir weakness. Symmetrical weakness and reduced reflexes were found in 98% of patients. CSF albuminocytologic dissociation was detected in 238/269 (89%) of cases and abnormal nerve physiology in 258/259 (>99%) of cases. Only 27 (8%) patients received either ...
Nature reviews. Neurology, 2014
Guillain-Barré syndrome (GBS) and its variant, Miller Fisher syndrome (MFS), exist as several cli... more Guillain-Barré syndrome (GBS) and its variant, Miller Fisher syndrome (MFS), exist as several clinical subtypes with different neurological features and presentations. Although the typical clinical features of GBS and MFS are well recognized, current classification systems do not comprehensively describe the full spectrum of either syndrome. In this Perspectives article, GBS and MFS are classified on the basis of current understanding of the common pathophysiological profiles of each disease phenotype. GBS is subclassified into classic and localized forms (for example, pharyngeal-cervical-brachial weakness and bifacial weakness with paraesthesias), and MFS is divided into incomplete (for example, acute ophthalmoparesis, acute ataxic neuropathy) and CNS subtypes (Bickerstaff brainstem encephalitis). Diagnostic criteria based on clinical characteristics are suggested for each condition. We believe this approach to be more inclusive than existing systems, and argue that it could facili...
Guillain-Barré syndrome (GBS) is a post-infectious autoimmune polyneuropathy regulated by pro- an... more Guillain-Barré syndrome (GBS) is a post-infectious autoimmune polyneuropathy regulated by pro- and anti-inflammatory cytokines; TNFA polymorphisms may exert immune pathogenic roles in GBS. We assessed TNFA promoter region polymorphisms (-238G/A, -308G/A, -857C/T, -863C/A) in Bangladeshi patients with GBS (n = 300) and healthy controls (n = 300) by PCR-RFLP and ASO-PCR. TNFA -863CA was significantly associated with GBS disease susceptibility (P = 0.0154) and disease severity (P = 0.0492). TNFA -238A allele was more frequent among anti-ganglioside (GM1) antibody-positive patients (P = 0.0092) and -863AA associated with AMAN subtype of GBS (P = 0.0398). TNFA -863C/A may contribute to GBS severity and pathogenesis in Bangladeshi patients.
Research Square (Research Square), Nov 10, 2020
Background: SARS-CoV2 virus could be potentially myopathic. Serum creatinine phosphokinase (CPK) ... more Background: SARS-CoV2 virus could be potentially myopathic. Serum creatinine phosphokinase (CPK) is frequently found elevated in severe SARS-CoV2 infection, which indicates skeletal muscle damage precipitating limb weakness or even ventilatory failure. Case presentation: We addressed such a patient in his forties presented with features of severe SARS-CoV2 pneumonia and high serum CPK. He developed severe sepsis and acute respiratory distress syndrome (ARDS) and received intravenous high dose corticosteroid and Tocilizumab to counter SARS-CoV2 associated cytokine surge. After 10 days of mechanical ventilation (MV) weaning was unsuccessful albeit apparently clear lung fields, having additionally severe and symmetric limb muscle weakness. Ancilliary investigations in addition with serum CPK, including electromyogram, muscle biopsy and muscle magnetic resonance imaging (MRI) suggested acute myopathy possibly due to skeletal myositis. Conclusion: We wish to stress that myopathogenic medication in SARS-CoV2 pneumonia should be used with caution. Additionally serum CPK could be a potential marker to predict respiratory failure in SARS-CoV2 pneumonia as skeletal myopathy affecting chest muscles may contribute ventilatory failure on top of oxygenetion failure due to SARS-CoV2 pneumonia.
Journal of neuroimmunology, Jan 15, 2017
Guillain-Barré syndrome (GBS) is a post-infectious autoimmune polyneuropathy regulated by pro- an... more Guillain-Barré syndrome (GBS) is a post-infectious autoimmune polyneuropathy regulated by pro- and anti-inflammatory cytokines; TNFA polymorphisms may exert immune pathogenic roles in GBS. We assessed TNFA promoter region polymorphisms (-238G/A, -308G/A, -857C/T, -863C/A) in Bangladeshi patients with GBS (n=300) and healthy controls (n=300) by PCR-RFLP and ASO-PCR. TNFA -863CA was significantly associated with GBS disease susceptibility (P=0.0154) and disease severity (P=0.0492). TNFA -238A allele was more frequent among anti-ganglioside (GM1) antibody-positive patients (P=0.0092) and -863AA associated with AMAN subtype of GBS (P=0.0398). TNFA -863C/A may contribute to GBS severity and pathogenesis in Bangladeshi patients.
OBJECTIVE: To assess the safety and feasibility of small volume plasma exchange (SVPE) as an alte... more OBJECTIVE: To assess the safety and feasibility of small volume plasma exchange (SVPE) as an alternative to standard plasma exchange (PE) or intravenous immunoglobulin (IVIg) for Guillain-Barré syndrome (GBS) patients. DESIGN: Non-randomized, single arm, interventional trial. SETTING: National Institute of Neurosciences and Hospital, Dhaka, Bangladesh. PARTICIPANTS: Twenty adult (>18 years) patients with GBS presented within 2 weeks of onset of weakness who were unable to walk unaided for more than 10 meters. INTERVENTIONS: SVPE involves blood cell sedimentation in a blood bag and removal of supernatant plasma after blood cells are re-transfused. This procedure was repeated three to six times a day, for eight consecutive days. OUTCOME MEASURES: Serious adverse events (SAE) were defined as severe sepsis and deep venous thrombosis related to the central vein catheter (CVC) used during SVPE. SVPE was considered safe if less than 5/20 patients experienced a SAE, and feasible if 8 L plasma could be removed within 8 days in at least 15/20 patients. RESULTS Median patient age 33 years (IQR 23-46; range 18-55); 13 (65%) were male. Median MRC sum score was 20 (IQR 0-29; range 0-36); three (15%) patients required mechanical ventilation. One patient developed SAE (severe sepsis, possibly related to CVC). Minor adverse effects were transient hypotension in 10 (50%) patients; CVC-associated bleeding in 10 (50%); transfusion reaction to fresh frozen plasma in 4 (20%); and hypo-albuminemia, anaemia or electrolyte imbalance in 4 (20%). Removal of 8 L plasma was possible in 15 (75%) patients. GBS disability score improved by at least one grade in 14 (70%) patients four weeks after SVPE started. No patients died. CONCLUSION: SVPE seems a safe and feasible alternative treatment to standard PE or IVIg for GBS; further studies of clinical efficacy in low-resource developing countries are warranted. TRIAL REGISTRATION: Clinicaltrials.gov NCT02780570 on May 23, 2016. Strength and limitations of the study: 1. The strength of this study underlies the novel and simple technique of SVPE, which is much less expensive than conventional immunotherapies (plasma exchange and intravenous immunoglobulin). 2. SVPE is corroborated as safe and feasible for the first time in a prospective and standardized cohort of patients with Guillain-Barré syndrome (GBS). 3. The intrinsic limitations of this study are its non-randomized, single arm nature, which is conducted in a single center with a limited sample size of GBS patients. 4. Clinical efficacy of SVPE on patients with GBS was a secondary end-point assessment and therefore deserves a randomized controlled trial in future to assess the clinical efficacy of SVPE for the patients with GBS
Clinical Neurophysiology Practice, 2021
Objective: To describe the electrophysiological features in relation to clinical and serological ... more Objective: To describe the electrophysiological features in relation to clinical and serological findings of Guillain-Barré syndrome (GBS) in the national neuroscience hospital in Bangladesh. This is one of the few studies that investigated GBS patients using standardized electrophysiology in low-income countries. Methods: In a prospective and observational study, we investigated 312 GBS patients by standardized clinical, serological and electrophysiological methods. Unilateral motor and sensory nerve conduction studies (NCS) were performed within two weeks of onset of weakness. Follow up NCS were performed in 189 patients and classified according to eight sets of established GBS criteria. Serology included assessment of anti-GM1 antibodies and anti-campylobacter jejuni lipo-oligosaccharide (LOS) antibodies. Results: Depending on the criteria used, 44-59% patients had axonal GBS with anti-GM1 antibodies being present in 55-58% and 9-42% patients had demyelinating GBS with anti-GM1 antibodies being present in 7-35%. Conduction block (CB) with demyelinative slowing in the same nerve segment was found in 24% (74/312) patients, and CB without demyelinative slowing in the same nerve segment was found in 18% (56/312) patients, of whom anti-GM1 antibodies were found in 27% and 57% patients respectively. Follow-up NCS showed a change in GBS classification in 11-26% of patients, mainly from demyelinating to axonal GBS. Conclusions: The predominant subtype of GBS in Bangladesh is axonal but demyelinating GBS also occurs with classification being strongly dependent on the applied criteria. Significance: The present study demonstrates the importance of reaching international agreement on GBS criteria that should be based on the best evidence.
Skeletal Muscle
Background SARS-CoV2 virus could be potentially myopathic. Serum creatinine phosphokinase (CPK) i... more Background SARS-CoV2 virus could be potentially myopathic. Serum creatinine phosphokinase (CPK) is frequently found elevated in severe SARS-CoV2 infection, which indicates skeletal muscle damage precipitating limb weakness or even ventilatory failure. Case presentation We addressed such a patient in his forties presented with features of severe SARS-CoV2 pneumonia and high serum CPK. He developed severe sepsis and acute respiratory distress syndrome (ARDS) and received intravenous high dose corticosteroid and tocilizumab to counter SARS-CoV2 associated cytokine surge. After 10 days of mechanical ventilation (MV), weaning was unsuccessful albeit apparently clear lung fields, having additionally severe and symmetric limb muscle weakness. Ancillary investigations in addition with serum CPK, including electromyogram, muscle biopsy, and muscle magnetic resonance imaging (MRI) suggested acute myopathy possibly due to skeletal myositis. Conclusion We wish to stress that myopathogenic medic...
Journal of the Peripheral Nervous System
Journal of the Peripheral Nervous System
Journal of the Peripheral Nervous System
Nature Reviews Neurology
Guillain–Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the pe... more Guillain–Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and 2015 in Latin America. Diagnosis and management of GBS can be complicated as its clinical presentation and disease course are heterogeneous, and no international clinical guidelines are currently available. To support clinicians, especially in the context of an outbreak, we have developed a globally applicable guideline for the diagnosis and management of GBS. The guideline is based on current literature and expert consensus, and has a ten-step structure to facilitate its use in clinical practice. We first provide an introduction to the diagnostic criteria, clinical variants and differential diagnoses of GBS. The ten steps then cover early recognition and di...
Journal of Neurology, Neurosurgery & Psychiatry
Chemotherapy-induced peripheral neurotoxicity (CIPN) is a common dose-limiting side effect of sev... more Chemotherapy-induced peripheral neurotoxicity (CIPN) is a common dose-limiting side effect of several anticancer medications. CIPN may involve multiple areas of the peripheral nervous system from the autonomic and dorsal root ganglia (DRG) to the axon and any peripheral nerve fibre type. Large diameter sensory myelinated (Aβ) fibres are more frequently involved, but motor, small myelinated (Aδ), unmyelinated (C) or autonomic fibres may also be affected. Here, we review the current evidence on techniques for the CIPN assessment in the clinical and experimental settings. Nerve conduction studies (NCS) may be used at the subclinical and early CIPN stage, to assess the extent of large nerve fibre damage and to monitor long-term outcomes, with the sural or dorsal sural nerve as the most informative. The quantitative sensory neurological examination provides valuable data alongside NCS. Quantitative sensory testing and nerve excitability studies add information regarding pathophysiology. ...
Therapeutic Apheresis and Dialysis
Annals of Clinical and Translational Neurology
Objective: TLR4 plays an important role in the pathogenesis of Guillain-Barr e syndrome (GBS). Th... more Objective: TLR4 plays an important role in the pathogenesis of Guillain-Barr e syndrome (GBS). The relationships between TLR4 polymorphisms and susceptibility to GBS are poorly understood. We investigated the frequency and assessed the association of two single nucleotide polymorphisms (SNPs) in the extracellular domain of TLR4 (Asp299Gly and Thr399Ile) with disease susceptibility and the clinical features of GBS in a Bangladeshi cohort. Methods: A total of 290 subjects were included in this study: 141 patients with GBS and 149 unrelated healthy controls. The TLR4 polymorphisms Asp299Gly and Thr399Ile were genotyped using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) assay. Results: The minor 299Gly allele was significantly associated with GBS susceptibility (P = 0.0137, OR = 1.97, 95% CI = 1.17-3.31), and was present at a significantly higher frequency in patients with the acute motor axonal neuropathy (AMAN) subtype of GBS (P = 0.0120, OR = 2.37, 95% CI = 1.26-4.47) than acute inflammatory demyelinating polyneuropathy (AIDP) subtype (P = 0.961, OR = 1.15, 95% CI = 0.38-3.48); when compared to healthy controls. The genotype frequency of the Asp299Gly polymorphism was not significantly different between patients with GBS and healthy controls. The Asp299-Thr399 haplotype was associated with a significantly lower risk of developing GBS (P = 0.0451, OR = 0.63, 95% CI = 0.40-0.99). No association was observed between the Thr399Ile polymorphism and GBS disease susceptibility. Interpretation: The TLR4 minor 299Gly allele was associated with increased susceptibility to GBS and the axonal GBS subtype in the Bangladeshi population. However, no associations were observed between the genotypes of the Asp299Gly and Thr399Ile SNPs and antecedent C. jejuni infection or disease severity in Bangladeshi patients with GBS.
Annals of Clinical and Translational Neurology
Objective: We investigated clinical, biological, and electrophysiological risk factors for mechan... more Objective: We investigated clinical, biological, and electrophysiological risk factors for mechanical ventilation (MV) and patient outcomes in Bangladesh using one of the largest, prospective Guillain-Barr e syndrome (GBS) cohorts in developing world. Methods: A total of 693 GBS patients were included in two GBS studies conducted between 2006 and 2016 in Dhaka, Bangladesh. Associations between baseline characteristics and MV were tested using Fisher's exact test, v 2 test, or Mann-Whitney U-test, as appropriate. Risk factors for MV were assessed using multivariate logistic regression. Survival analysis was performed using Kaplan-Meier method; comparisons between groups performed using logrank test. Results: Of 693 patients, 155 (23%) required MV (median age, 26 years; interquartile range [IQR] 17-40). Among the ventilated patients, males were predominant (68%) than females. The most significant risk factor for MV was bulbar involvement (adjusted odds ratio [AOR]:19.07; 95% CI = 89.00-192.57, P = 0.012). Other independently associated factors included dysautonomia (AOR:4.88; 95% CI = 1.49-15.98, P = 0.009) and severe muscle weakness at study entry (AOR:6.12; 95% CI = 0.64-58.57, P = 0.048). At 6 months after disease onset, 20% of ventilated and 52% of non-ventilated patients (P < 0.001) had recovered completely or with minor symptoms. Mortality rate was significantly higher among ventilated patients than non-ventilated patients (41% vs. 7%, P < 0.001). Interpretation: Bulbar involvement, dysautonomia and severe muscle weakness were identified as the most important risk factors for MV among GBS patients from Bangladesh. The findings may help to develop predictive models for MV in GBS in developing countries to identify impending respiratory failure and proper clinical management of GBS patients.
Brain : a journal of neurology, 2018
Guillain-Barré syndrome is a heterogeneous disorder regarding the clinical presentation, electrop... more Guillain-Barré syndrome is a heterogeneous disorder regarding the clinical presentation, electrophysiological subtype and outcome. Previous single country reports indicate that Guillain-Barré syndrome may differ among regions, but no systematic comparative studies have been conducted. Comparative studies are required to identify factors determining disease susceptibility, variation and prognosis, and to improve diagnostic criteria. The International Guillain-Barré Syndrome Outcome Study is a prospective, observational cohort study including all patients within the diagnostic spectrum, aiming to describe the heterogeneity of Guillain-Barré syndrome worldwide. The current study was based on the first 1000 inclusions with a follow-up of at least 1 year and confirmed the variation in clinical presentation, course and outcome between patients. The full clinical spectrum of Guillain-Barré syndrome was observed in patients from all countries participating in the International Guillain-Barr...
BMJ open, Jan 17, 2018
To assess the safety and feasibility of small volume plasma exchange (SVPE) for patients with Gui... more To assess the safety and feasibility of small volume plasma exchange (SVPE) for patients with Guillain-Barré syndrome (GBS). Non-randomised, single-arm, interventional trial. National Institute of Neurosciences and Hospital, Dhaka, Bangladesh. Twenty adult (>18 years) patients with GBS presented within 2 weeks of onset of weakness who were unable to walk unaided for more than 10 m. SVPE involves blood cell sedimentation in a blood bag and removal of supernatant plasma after blood cells are retransfused. This procedure was repeated three to six times a day, for eight consecutive days. Fresh frozen plasma (FFP) and normal saline were used as replacement fluid. Serious adverse events (SAEs) were defined as severe sepsis and deep venous thrombosis related to the central venous catheter (CVC) used during SVPE. SVPE was considered safe if less than 5/20 patients experienced an SAE, and feasible if 8 L plasma could be removed within 8 days in at least 15/20 patients. Median patient age ...
Annals of clinical and translational neurology, 2018
Previous studies have associated Guillain-Barré syndrome (GBS) with Zika virus (ZIKV) outbreaks i... more Previous studies have associated Guillain-Barré syndrome (GBS) with Zika virus (ZIKV) outbreaks in South America and Oceania. In Asia, ZIKV is known to circulate widely, but the association with Guillain-Barré syndrome is unclear. We investigated whether endemic ZIKV infection is associated with the development of GBS. A prospective study was conducted from 2011 to 2015 in Bangladesh. A total of 418 patients and 418 healthy family controls were included in the study. Patients were diagnosed with GBS prior to inclusion according to established criteria. Detailed information on the epidemiology, clinical presentation, electrophysiology, diagnosis, disease severity, and clinical course were obtained during a follow-up of 1 year using a predefined protocol. ZIKV-neutralizing antibodies were detected in our study from 2013 onwards. The prevalence of ZIKV-neutralizing antibodies was not significantly higher in patients with GBS compared to healthy controls (OR 2.23, = 0.14, 95% CI 0.77-6....
Pilot and feasibility studies, 2017
In Bangladesh, most patients with Guillain-Barré syndrome (GBS) cannot afford standard treatment ... more In Bangladesh, most patients with Guillain-Barré syndrome (GBS) cannot afford standard treatment with intravenous immunoglobulin (IVIG) or a standard plasma exchange (PE) course, which partly explains the high rate of mortality and residual disability associated with GBS in this country. Small volume plasma exchange (SVPE) is an affordable and potentially effective alternative form of plasma exchange. SVPE is the repeated removal of small volumes of supernatant plasma over several days via sedimentation of patient whole blood. The aim of this study is to define the clinical feasibility and safety of SVPE in patients with GBS in resource poor settings. A total of 20 adult patients with GBS will be enrolled for SVPE at a single center in Bangladesh. Six daily sessions of whole blood sedimentation and plasma removal will be performed in all patients with GBS with a target to remove an overall volume of at least 8 liters (L) of plasma over a total of 8 days. Serious adverse events (SAE)...
Journal of the peripheral nervous system : JPNS, Dec 12, 2016
Guillain-Barré syndrome has a diverse clinical phenotype related to geographical origin. To date,... more Guillain-Barré syndrome has a diverse clinical phenotype related to geographical origin. To date, the majority of large-scale studies on Guillain-Barré syndrome (GBS) have been conducted in developed countries. We aimed to evaluate the key diagnostic features and assess the suitability of the Brighton criteria in 344 adult GBS patients from Bangladesh. All patients fulfilled the National Institute of Neurological Diseases and Stroke (NINDS) diagnostic criteria. Standardized data on demographic characteristics and clinical features, cerebrospinal fluid (CSF) analysis and nerve conduction study (NCS) results were elaborated to measure the sensitivity of Brighton criteria. Most patients (88%) were admitted to hospital after the nadir weakness. Symmetrical weakness and reduced reflexes were found in 98% of patients. CSF albuminocytologic dissociation was detected in 238/269 (89%) of cases and abnormal nerve physiology in 258/259 (>99%) of cases. Only 27 (8%) patients received either ...
Nature reviews. Neurology, 2014
Guillain-Barré syndrome (GBS) and its variant, Miller Fisher syndrome (MFS), exist as several cli... more Guillain-Barré syndrome (GBS) and its variant, Miller Fisher syndrome (MFS), exist as several clinical subtypes with different neurological features and presentations. Although the typical clinical features of GBS and MFS are well recognized, current classification systems do not comprehensively describe the full spectrum of either syndrome. In this Perspectives article, GBS and MFS are classified on the basis of current understanding of the common pathophysiological profiles of each disease phenotype. GBS is subclassified into classic and localized forms (for example, pharyngeal-cervical-brachial weakness and bifacial weakness with paraesthesias), and MFS is divided into incomplete (for example, acute ophthalmoparesis, acute ataxic neuropathy) and CNS subtypes (Bickerstaff brainstem encephalitis). Diagnostic criteria based on clinical characteristics are suggested for each condition. We believe this approach to be more inclusive than existing systems, and argue that it could facili...
Background: Bangladesh has achieved a remarkable success to eradicate poliomyelitis, however, Gui... more Background: Bangladesh has achieved a remarkable success to eradicate poliomyelitis, however, Guillain–Barré syndrome (GBS)is frequently diagnosed. GBS is an autoimmune mediated disease of the peripheral nervous system preceded by infections. Campylobacter jejuni has been identified as the predominant cause of antecedent infection in GBS. C. jejuni lipopolysaccharide (LPS) induces antibodies cross-reactive with gangliosides and has shown to be involved in peripheral nerve damage. Toll-like receptor 4 (TLR4) is an important pathogen recognition receptor that recognizes mainly lipopolysaccharide (LPS) of gram-negative bacteria. In this study, we investigated functional single nucleotide polymorphisms (SNPs) in the extracellular domain of TLR4 (Asp299Gly and Thr399Ile), and assessed their association for GBS susceptibility, disease pathogenesis and disease outcome. Methods & Materials: A hospital based case controlled study was conducted in Dhaka Medical College Hospital (DMCH) in Dhak...