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1.
J Peripher Nerv Syst ; 28(4): 564-577, 2023 12.
Artículo en Inglés | MEDLINE | ID: mdl-37698165

RESUMEN

BACKGROUND AND AIMS: Considerable variation in clinical practice for management of Guillain-Barré syndrome (GBS) has been observed worldwide. Diagnosis and treatment are challenging in low- and middle-income countries (LMIC) due to lack of facilities and treatment availability. We aimed to evaluate current clinical practice and limitations and to provide recommendation for GBS management in low-resource settings. METHODS: We conducted an explanatory-sequential mixed-methods survey among neurologists and internists working in tertiary and secondary government hospitals in Bangladesh. There were two phases: (1) quantitative (cross-sectional survey to evaluate clinical practice and limitations); (2) qualitative (key informant interview to explain certain clinical practice and provide recommendations for GBS management in LMIC). Data were analyzed by frequencies, χ2 test and thematic analysis. RESULTS: Among 159 physicians (65 neurologists and 94 internists), 11% and 8% physicians used Brighton and NINDS criteria respectively to diagnose GBS. Specific treatment protocols of GBS were used by 12% physicians. Overcrowding of patients, inadequate diagnostic facilities, high costs of standard therapy, and inadequate logistics and trained personnel for intensive care unit and rehabilitation services were considered major challenges for GBS management. In qualitative part, respondents recommended regular training for the physicians, development of cost-effective treatment strategies and appropriate patients' referral and management guideline considering existing limitations in health service delivery and socio-economic status of the country. INTERPRETATION: Current study design and recommendations might be applied for other LMIC. Such data can assist policymakers to identify areas requiring urgent attention and take required action to improve GBS management in LMIC.


Asunto(s)
Síndrome de Guillain-Barré , Humanos , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/epidemiología , Síndrome de Guillain-Barré/terapia , Países en Desarrollo , Bangladesh/epidemiología , Estudios Transversales , Neurólogos
2.
Ann Clin Transl Neurol ; 9(8): 1264-1275, 2022 08.
Artículo en Inglés | MEDLINE | ID: mdl-35908170

RESUMEN

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


Asunto(s)
Síndrome de Guillain-Barré , Bangladesh , Niño , Estudios de Cohortes , Humanos , Pronóstico , Estudios Prospectivos
3.
Sci Rep ; 12(1): 5791, 2022 04 06.
Artículo en Inglés | MEDLINE | ID: mdl-35388043

RESUMEN

Complement activation plays a critical role in the pathogenesis of Guillain-Barré syndrome (GBS), a debilitating immune-mediated neuropathy. Mannose-binding lectin (MBL) is a complement activation factor of lectin pathway which as genetic host factor may influence the susceptibility or severity of GBS. We investigated the frequency of MBL2 promoter (- 550H/L and - 221X/Y) and functional region (exon 1 A/O) polymorphisms and their association with disease susceptibility, clinical features and serum MBL among GBS patients (n = 300) and healthy controls (n = 300) in Bangladesh. The median patient age was 30 years (IQR: 18-42; males, 68%). MBL2 polymorphisms were not significantly associated with GBS susceptibility compared to healthy controls. HL heterozygosity in GBS patients was significantly associated with mild functional disability at enrolment (P = 0.0145, OR, 95% CI 2.1, 1.17-3.82). The HY, YA, HA and HYA heterozygous haplotypes were more common among mildly affected (P = 0.0067, P = 0.0086, P = 0.0075, P = 0.0032, respectively) than severely affected patients with GBS. Reduced serum MBL was significantly associated with the LL, OO and no HYA variants and GBS disease severity. No significant association was observed between MBL2 polymorphisms and electrophysiological variants, recent Campylobacter jejuni infection or anti-ganglioside (GM1) antibody responses in GBS. In conclusion, MBL2 gene polymorphisms are related to reduced serum MBL and associated with the severity of GBS.


Asunto(s)
Síndrome de Guillain-Barré , Lectina de Unión a Manosa , Adolescente , Adulto , Activación de Complemento , Exones , Predisposición Genética a la Enfermedad , Genotipo , Síndrome de Guillain-Barré/genética , Haplotipos , Humanos , Masculino , Lectina de Unión a Manosa/genética , Polimorfismo Genético , Adulto Joven
4.
Nat Rev Neurol ; 17(5): 285-296, 2021 05.
Artículo en Inglés | MEDLINE | ID: mdl-33649531

RESUMEN

The epidemiology, clinical characteristics, management and outcome of Guillain-Barré syndrome (GBS) differ between low-income and middle-income countries (LMIC) and high-income countries (HIC). At present, limited data are available on GBS in LMIC and the true incidence of GBS in many LMIC remains unknown. Increased understanding of GBS in LMIC is needed because poor hygiene and high exposure to infections render populations in LMIC vulnerable to GBS outbreaks. Furthermore, insufficient diagnostic and health-care facilities in LMIC contribute to delayed diagnosis in patients with severe presentations of GBS. In addition, the lack of national clinical guidelines and absence of affordable, effective treatments contribute to worse outcomes and higher mortality in LMIC than HIC. Systematic population-based surveillance studies, cohort and case-control studies are required to understand the incidence and risk factors for GBS. Novel, targeted and cost-effective treatment strategies need to be developed in the context of health system challenges in LMIC. To ensure integrative rehabilitation services in LMIC, existing prognostic models must be validated, and responsive outcome measures that are cross-culturally applicable must be developed. Therefore, fundamental and applied research to improve the clinical management of GBS in LMIC should become a critical focus of future research programmes.


Asunto(s)
Países en Desarrollo/economía , Salud Global/economía , Síndrome de Guillain-Barré/economía , Síndrome de Guillain-Barré/epidemiología , Pobreza/economía , Salud Global/tendencias , Síndrome de Guillain-Barré/terapia , Humanos , Inmunoterapia/economía , Inmunoterapia/tendencias , Pobreza/tendencias
5.
Muscle Nerve ; 63(4): 546-552, 2021 04.
Artículo en Inglés | MEDLINE | ID: mdl-33452679

RESUMEN

BACKGROUND: The most common subtypes of Guillain-Barré syndrome (GBS) are acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). In the first days after the onset of weakness, standard nerve conduction studies (NCS) may not distinguish GBS subtypes. Reduced nerve excitability may be an early symptom of nerve dysfunction, which can be determined with the compound muscle action potential (CMAP) scan. The aim of this study was to explore whether early changes in motor nerve excitability in GBS patients are related to various subtypes. METHODS: Prospective case-control study in 19 GBS patients from The Netherlands and 22 from Bangladesh. CMAP scans were performed within 2 days of hospital admission and NCS 7-14 days after onset of weakness. CMAP scans were also performed in age- and country-matched controls. RESULTS: CMAP scan patterns of patients who were classified as AMAN were distinctly different compared to the CMAP scan patterns of the patients who were classified as AIDP. The most pronounced differences were found in the stimulus intensity parameters. CONCLUSIONS: CMAP scans made at hospital admission demonstrate several characteristics that can be used as an early indicator of GBS subtype.


Asunto(s)
Síndrome de Guillain-Barré , Tejido Nervioso , Conducción Nerviosa , Sistema Nervioso Periférico , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios de Casos y Controles , Neuronas Motoras/fisiología , Tejido Nervioso/fisiopatología , Países Bajos , Conducción Nerviosa/fisiología , Examen Neurológico/métodos , Sistema Nervioso Periférico/diagnóstico por imagen , Sistema Nervioso Periférico/fisiopatología , Síndrome de Guillain-Barré/fisiopatología
6.
Clin Neurophysiol Pract ; 6: 155-163, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-35112034

RESUMEN

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.

7.
Ann Clin Transl Neurol ; 6(4): 708-715, 2019 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-31019995

RESUMEN

OBJECTIVE: TLR4 plays an important role in the pathogenesis of Guillain-Barré 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.


Asunto(s)
Síndrome de Guillain-Barré/etiología , Síndrome de Guillain-Barré/genética , Polimorfismo de Nucleótido Simple/genética , Receptor Toll-Like 4/genética , Adolescente , Adulto , Anciano , Alelos , Bangladesh , Femenino , Frecuencia de los Genes/genética , Genotipo , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven
8.
Ann Clin Transl Neurol ; 6(2): 324-332, 2019 02.
Artículo en Inglés | MEDLINE | ID: mdl-30847364

RESUMEN

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-Barré 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, χ2 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 log-rank 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.


Asunto(s)
Síndrome de Guillain-Barré/complicaciones , Síndrome de Guillain-Barré/fisiopatología , Insuficiencia Respiratoria/etiología , Insuficiencia Respiratoria/fisiopatología , Adolescente , Adulto , Bangladesh , Femenino , Humanos , Modelos Logísticos , Masculino , Oportunidad Relativa , Estudios Prospectivos , Respiración Artificial/métodos , Factores de Riesgo , Adulto Joven
9.
Ann Clin Transl Neurol ; 5(5): 606-615, 2018 May.
Artículo en Inglés | MEDLINE | ID: mdl-29761123

RESUMEN

OBJECTIVE: 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. METHODS: 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. RESULTS: 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, P = 0.14, 95% CI 0.77-6.53). Serological evidence for prior ZIKV infection in patients with GBS was associated with more frequent cranial, sensory, and autonomic nerve involvement compared to GBS patients with Campylobacter jejuni, the predominant preceding infection in GBS worldwide. Nerve-conduction studies revealed that ZIKV antibodies were associated with a demyelinating subtype of GBS, while C. jejuni infections were related to an axonal subtype. INTERPRETATION: No significant association was found between ZIKV infection and GBS in Bangladesh, but GBS following ZIKV infection was characterized by a distinct clinical and electrophysiological subtype compared to C. jejuni infection. These findings indicate that ZIKV may precede a specific GBS subtype but the risk is low.

10.
PLoS One ; 13(2): e0192703, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-29432441

RESUMEN

Guillain-Barré syndrome (GBS) is an autoimmune disorder of the peripheral nervous system triggered by molecular mimicry between pathogen lipopolysaccharides and host nerve gangliosides. Polymorphisms in the Fas receptor (FAS) and Fas ligand (FASL) genes may potentially alter the elimination of autoreactive immune cells and affect disease susceptibility or disease severity in GBS. We detected single nucleotide polymorphisms (SNPs) in FAS (-1377G/A and -670A/G) and FASL (-843C/T) in a prospective cohort of 300 patients with GBS and 300 healthy controls from the Bangladeshi population. Genotype distributions were not significantly different between patients with GBS and healthy controls. The FAS -670 AG heterozygous (P = 0.0005, OR = 2.5, 95% CI = 1.5-4.2) and GG homozygous (P = 0.0048, OR = 2.6, 95% CI = 1.3-5.0) genotypes were more common in patients with anti-GM1 antibodies than patients without anti-GM1 antibodies. The FAS -670 G allele was more prevalent in anti-GM1 antibody-positive than -negative patients (P = 0.0002, OR = 1.9, 95% CI = 1.4-2.7) and also in patients with the axonal subtype than demyelinating subtype (P < 0.0001, OR = 4.8, 95% CI = 2.3-10.1). The 1377G/-670G GG haplotype was significantly associated with the axonal subtype (P < 0.0001) and anti-ganglioside antibody-positivity (P = 0.0008) in GBS. Serum sFas (237.5 pg/mL vs. 159.5 pg/mL; P < 0.0001) and sFasL (225.1 pg/mL vs. 183.4 pg/mL; P = 0.0069) were elevated in patients with GBS compared to healthy controls, and among patients with high serum sFas was associated with severe GBS (P = 0.0406). In conclusion, this study indicates FAS-FASL promoter SNPs may promote the production of cross-reactive anti-ganglioside antibodies in GBS.


Asunto(s)
Proteína Ligando Fas/genética , Síndrome de Guillain-Barré/sangre , Sistema Nervioso Periférico/patología , Polimorfismo de Nucleótido Simple , Regiones Promotoras Genéticas , Receptor fas/genética , Adolescente , Adulto , Bangladesh , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Gangliósido G(M1)/inmunología , Síndrome de Guillain-Barré/patología , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Adulto Joven , Receptor fas/sangre
11.
Eur J Clin Microbiol Infect Dis ; 37(3): 511-518, 2018 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-29411189

RESUMEN

We describe the frequency, clinical features, and electrophysiological and immunological phenotypes of Guillain-Barré Syndrome (GBS) patients treated at a single institution in Bangladesh who had preceding chicken pox (primary Varicella-zoster virus [VZV] infection) within 4 weeks of GBS onset. A literature review of GBS cases preceding VZV infection is also provided. Diagnosis of GBS was based on the National Institute of Neurological Disorders and Stroke criteria for GBS. Serum anti-VZV IgM and IgG antibodies were quantified by indirect chemiluminescence immunoassay (CLIA); anti-Campylobacter jejuni IgG, IgM, and IgA antibodies and anti-ganglioside GM1 IgM and IgG antibodies, by enzyme-linked immunosorbent assays. Neurophysiologic subtypes were categorized following the Hadden criteria. Of 536 patients with GBS, 7 (1.3%) had chicken pox within 4 weeks before GBS onset. Four of the seven cases were male (age range, 23 to 40 years old). All seven patients were bed-bound, six had sensory symptoms, and three required mechanical ventilation for respiratory failure. All seven patients had CSF albuminocytologic dissociation and evidence of demyelination in nerve conduction studies. Anti-VZV IgM antibodies were present and anti-GM1 and anti-Campylobacter jejuni lipo-oligosaccharides (LOS) were negative in all cases. All patients had excellent outcome at 1 year (able to run). A systematic literature review of GBS cases related to VZV revealed 39 previously reported patients with comparable clinical presentations and outcomes, of which 36 had neurophysiologic evidence of demyelination. VZV infection is associated with the demyelinating subtype of GBS, clearly distinct from the axonal form of GBS that predominate in countries like Bangladesh.


Asunto(s)
Síndrome de Guillain-Barré/epidemiología , Síndrome de Guillain-Barré/virología , Herpes Zóster/complicaciones , Herpes Zóster/epidemiología , Herpesvirus Humano 3 , Adolescente , Adulto , Anciano , Niño , Femenino , Síndrome de Guillain-Barré/fisiopatología , Síndrome de Guillain-Barré/terapia , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Adulto Joven
12.
Artículo en Inglés | MEDLINE | ID: mdl-28975040

RESUMEN

BACKGROUND: 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. METHODS: 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) are defined as the number of patients developing severe sepsis associated with the central venous catheter or deep venous thrombosis in the limb where the catheter is placed for SVPE. Based upon a predictive success rate of 75%, the SVPE procedure will be considered safe if less than 5 of 20 SVPE-treated GBS patients have a SAE. The procedure will be considered feasible if 8 L of plasma can be removed in at least 15 of 20 patients with GBS who receive SVPE. In addition, detailed clinical and neurological outcome assessments will be performed until discharge of the patient from the hospital and up to 4 weeks after study entry. DISCUSSION: This is the first clinical study to evaluate the feasibility and safety of SVPE as a potential alternative low-cost treatment for the patients with GBS in resource poor settings. TRIAL REGISTRATION: Clinicaltrials.gov NCT02780570.

13.
J Peripher Nerv Syst ; 22(2): 121-126, 2017 06.
Artículo en Inglés | MEDLINE | ID: mdl-28447405

RESUMEN

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.


Asunto(s)
Síndrome de Guillain-Barré/epidemiología , Síndrome de Guillain-Barré/mortalidad , Adolescente , Adulto , Distribución por Edad , Bangladesh/epidemiología , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Modelos de Riesgos Proporcionales , Estudios Retrospectivos , Factores de Riesgo , Adulto Joven
14.
Neurol Neuroimmunol Neuroinflamm ; 3(6): e284, 2016 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-27790627

RESUMEN

OBJECTIVE: To characterize the patterns of autoantibodies to glycolipid complexes in a large cohort of Guillain-Barré syndrome (GBS) and control samples collected in Bangladesh using a newly developed microarray technique. METHODS: Twelve commonly studied glycolipids and lipids, plus their 66 possible heteromeric complexes, totaling 78 antigens, were applied to polyvinylidene fluoride-coated slides using a microarray printer. Arrays were probed with 266 GBS and 579 control sera (2 µL per serum, diluted 1/50) and bound immunoglobulin G detected with secondary antibody. Scanned arrays were subjected to statistical analyses. RESULTS: Measuring antibodies to single targets was 9% less sensitive than to heteromeric complex targets (49.2% vs 58.3%) without significantly affecting specificity (83.9%-85.0%). The optimal screening protocol for GBS sera comprised a panel of 10 glycolipids (4 single glycolipids GM1, GA1, GD1a, GQ1b, and their 6 heteromeric complexes), resulting in an overall assay sensitivity of 64.3% and specificity of 77.1%. Notable heteromeric targets were GM1:GD1a, GM1:GQ1b, and GA1:GD1a, in which exclusive binding to the complex was observed. CONCLUSIONS: Rationalizing the screening protocol to capture the enormous diversity of glycolipid complexes can be achieved by miniaturizing the screening platform to a microarray platform, and applying simple bioinformatics to determine optimal sensitivity and specificity of the targets. Glycolipid complexes are an important category of glycolipid antigens in autoimmune neuropathy cases that require specific analytical and bioinformatics methods for optimal detection.

15.
J Peripher Nerv Syst ; 21(4): 345-351, 2016 12.
Artículo en Inglés | MEDLINE | ID: mdl-27616152

RESUMEN

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%) cases and abnormal nerve physiology in 258/259 (>99%) cases. Only 27 (8%) patients received either intravenous immunoglobulin (IVIg) or plasmapheresis. In total, 200 (58%) patients met level 1 of the Brighton criteria; 97 (28%) patients met level 2; 42 (12%) patients met level 3; and 5 (2%) patients met level 4. This analysis showed that despite the heterogeneity of GBS in Bangladesh, the Brighton criteria showed a high sensitivity in the diagnosis of GBS.


Asunto(s)
Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/epidemiología , Examen Neurológico/métodos , Adulto , Bangladesh/epidemiología , Electrofisiología , Femenino , Síndrome de Guillain-Barré/líquido cefalorraquídeo , Síndrome de Guillain-Barré/terapia , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Masculino , Persona de Mediana Edad , Examen Neurológico/normas , Plasmaféresis , Reflejo de Estiramiento/efectos de los fármacos , Reflejo de Estiramiento/fisiología , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Estadísticas no Paramétricas
17.
PLoS One ; 7(8): e43976, 2012.
Artículo en Inglés | MEDLINE | ID: mdl-22952833

RESUMEN

BACKGROUND: Campylobacter jejuni is the predominant antecedent infection in Guillain-Barré syndrome (GBS). Molecular mimicry and cross-reactive immune responses to C. jejuni lipo-oligosaccharides (LOS) precipitate the development of GBS, although this mechanism has not been established in patients from developing countries. We determined the carbohydrate mimicry between C. jejuni LOS and gangliosides, and the cross-reactive antibody response in patients with GBS in Bangladesh. METHODOLOGY: Sera from 97 GBS patients, and 120 neurological and family controls were tested for antibody reactivity against LOS from C. jejuni isolates from GBS patients in Bangladesh (BD-07, BD-39, BD-10, BD-67 and BD-94) by enzyme-linked immunosorbent assay (ELISA). Cross-reactivity to LOS was determined by ELISA. The LOS outer core structures of C. jejuni strains associated with GBS/MFS were determined by mass spectrometry. PRINCIPLE FINDINGS: IgG antibodies to LOS from C. jejuni BD-07, BD-39, BD-10, and BD-67 IgG antibodies were found in serum from 56%, 58%, 14% and 15% of GBS patients respectively, as compared to very low frequency (<3%) in controls (p<0.001). Monoclonal antibodies specific for GM1 and GD1a reacted strongly with LOS from the C. jejuni strains (BD-07 and BD-39). Mass spectrometry analysis confirmed the presence of GM1 and GD1a carbohydrate mimics in the LOS from C. jejuni BD-07 and BD-39. Both BD-10 and BD-67 express the same LOS outer core, which appears to be a novel structure displaying GA2 and GD3 mimicry. Up to 90-100% of serum reactivity to gangliosides in two patients (DK-07 and DK-39) was inhibited by 50 µg/ml of LOS from the autologous C. jejuni isolates. However, patient DK-07 developed an anti-GD1a immune response while patient DK-39 developed an anti-GM1 immune response. CONCLUSION: Carbohydrate mimicry between C. jejuni LOS and gangliosides, and cross-reactive serum antibody precipitate the majority of GBS cases in Bangladesh.


Asunto(s)
Anticuerpos Antibacterianos/inmunología , Campylobacter jejuni/inmunología , Reacciones Cruzadas , Gangliósidos/química , Gangliósidos/inmunología , Síndrome de Guillain-Barré/microbiología , Adolescente , Adulto , Anticuerpos Antibacterianos/sangre , Anticuerpos Monoclonales/inmunología , Bangladesh , Campylobacter jejuni/patogenicidad , Secuencia de Carbohidratos , Niño , Femenino , Síndrome de Guillain-Barré/sangre , Humanos , Inmunoglobulina G/sangre , Inmunoglobulina G/inmunología , Lipopolisacáridos/inmunología , Masculino , Datos de Secuencia Molecular
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