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1.
Medicine (Baltimore) ; 99(16): e18638, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32311917

RESUMO

RATIONALE: Guillian-Barré syndrome (GBS) is a devastating autoimmune disorder characterized by progressive ascending weakness, areflexia with or without autonomic and sensory disturbances. Hydrocephalus is a rare but well-documented complication in patients with GBS. However, due to the rarity of this condition, no treatment guideline for GBS with hydrocephalus is currently available. PATIENT CONCERNS: We describe a 23-year old woman with a history of bilateral limbs pain followed by dysarthria, dysphagia, severe quadriplegia 0/5, areflexia, loss of consciousness and dysautonomia. Neuroimaging studies revealed enlarged lateral ventricles; while Electromyography demonstrated demyelination and nerve injury. Lumbar puncture results showed elevated protein level 2.6 g/L; normal glucose and cell count. DIAGNOSIS: GBS with hydrocephalus. INTERVENTIONS: The patient was started on intravenous immunoglobulin for 5 consecutive days followed by endotracheal intubation and supportive therapy including osmotherapy and CSF drainage. OUTCOMES: At 2 months after admission, the patient stopped choking and had a significant improvement in muscles' strength (grade 4) and pain; then was discharged. On 1 year post-discharge follow-up, CT has revealed a significant improvement of hydrocephalus, and the patient has completely returned to the normal baseline. LESSONS: Respiratory failure is the strongest predictor of concurrent hydrocephalus in patients with GBS. The prognosis of hydrocephalus in patients with GBS is usually good, and it can be medically treated; thereby shunt surgery is rarely required.


Assuntos
Síndrome de Guillain-Barré/complicações , Síndrome de Guillain-Barré/terapia , Hidrocefalia/etiologia , Hidrocefalia/terapia , Drenagem , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Intubação Intratraqueal , Adulto Jovem
2.
Medicine (Baltimore) ; 99(2): e18619, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31914040

RESUMO

BACKGROUND: Guillain-Barré syndrome (GBS) is the most common acute paralytic neuropathy. Many clinical trials indicate acupuncture provides a good effect as a complementary therapy of Western medicine for GBS. The objective of this systematic review protocol is to provide the evidence to evaluate the effectiveness and safety of acupuncture on the treatment of GBS. METHODS: We will search relevant randomized controlled trials investigating the effect of acupuncture for GBS in following databases from start to October 2019: PubMed, Embase, the Cochrane Library, CINAHL Complete, National Digital Science Library, China National Knowledge Infrastructure, and Wanfang Database without language restriction. For articles that meet our inclusion criteria, 2 researchers will extract the data information independently, and assess the risk of bias and trial quality by the Cochrane collaboration's tool. All data will be analyzed by RevMan V.5.3.3 statistical software. RESULTS: According to the Barthel index of Activities of Daily Living (ADL) and the Medical Research Council (MRC) muscle scale, the efficacy and safety of acupuncture for GBS will be determined in this study. CONCLUSION: This systemic review will provide high quality evidence to judging whether acupuncture provides benefits to treat GBS.Prospero registration number: CRD42019158710.


Assuntos
Terapia por Acupuntura/métodos , Síndrome de Guillain-Barré/terapia , Atividades Cotidianas , Terapia por Acupuntura/efeitos adversos , Humanos , Ensaios Clínicos Controlados Aleatórios como Assunto , Projetos de Pesquisa
4.
Presse Med ; 48(11 Pt 2): 338-346, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31679897

RESUMO

Therapeutic plasma exchange (TPE) has been used as a treatment modality in many autoimmune disorders, including neurological conditions, such as Guillain-Barre syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The American Society for Apheresis (ASFA) publishes its guidelines on the use of therapeutic apheresis every 3 years based on published evidence to assist physicians with both the medical and technical aspects of apheresis consults. The ASFA Guidelines included the use of TPE in both GBS and CIDP as an acceptable first-line therapy, either alone and/or in conjunction with other therapeutic modalities. In this article, we briefly reviewed GBS and CIDP, discussed the role of apheresis in these conditions as well as various technical aspects of the TPE procedure, such as apheresis calculation, number of volume exchange, replacement fluid, and management of potential complications.


Assuntos
Síndrome de Guillain-Barré/terapia , Troca Plasmática/métodos , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia , Humanos , Guias de Prática Clínica como Assunto
6.
Rinsho Shinkeigaku ; 59(9): 600-603, 2019 Sep 25.
Artigo em Japonês | MEDLINE | ID: mdl-31474642

RESUMO

An 85-year-old Japanese female was admitted with sudden onset of quadriparesis with areflexia. Preceding infection was not present. IgG anti-GT1b antibodies were prominently positive in serum. Nerve conduction study results suggested Guillain-Barré syndrome (GBS) classified as acute motor sensory axonal neuropathy (AMSAN). While intravenous immunoglobulin (IVIg) was started, bulbar palsy and respiratory failure progressed and the condition deteriorated. Although mechanical ventilation was required, second IVIg course led to gradual improvement of quadriparesis and bulbar palsy. In the present case with elderly-onset disease, the levels of anti-GT1b antibodies were elevated, which is relatively rare in GBS. It was suggested that anti-GT1b antibodies may be related to the development of axonal GBS with bulbar palsy.


Assuntos
Gangliosídeos/imunologia , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/imunologia , Imunoglobulina G/sangue , Idoso de 80 Anos ou mais , Biomarcadores/sangue , Paralisia Bulbar Progressiva/etiologia , Progressão da Doença , Feminino , Síndrome de Guillain-Barré/classificação , Síndrome de Guillain-Barré/terapia , Humanos , Imunoglobulinas Intravenosas/administração & dosagem , Resultado do Tratamento
7.
Nat Rev Neurol ; 15(11): 671-683, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31541214

RESUMO

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 diagnosis of GBS, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae.


Assuntos
Gerenciamento Clínico , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/terapia , Variação Genética/genética , Síndrome de Guillain-Barré/epidemiologia , Humanos , Infecção por Zika virus/diagnóstico , Infecção por Zika virus/epidemiologia , Infecção por Zika virus/terapia
9.
Neurology ; 93(1): e59-e76, 2019 07 02.
Artigo em Inglês | MEDLINE | ID: mdl-31175208

RESUMO

OBJECTIVE: To define the current treatment practice of Guillain-Barré syndrome (GBS). METHODS: The study was based on prospective observational data from the first 1,300 patients included in the International GBS Outcome Study. We described the treatment practice of GBS in general, and for (1) severe forms (unable to walk independently), (2) no recovery after initial treatment, (3) treatment-related fluctuations, (4) mild forms (able to walk independently), and (5) variant forms including Miller Fisher syndrome, taking patient characteristics and hospital type into account. RESULTS: We excluded 88 (7%) patients because of missing data, protocol violation, or alternative diagnosis. Patients from Bangladesh (n = 189, 15%) were described separately because 83% were not treated. IV immunoglobulin (IVIg), plasma exchange (PE), or other immunotherapy was provided in 941 (92%) of the remaining 1,023 patients, including patients with severe GBS (724/743, 97%), mild GBS (126/168, 75%), Miller Fisher syndrome (53/70, 76%), and other variants (33/40, 83%). Of 235 (32%) patients who did not improve after their initial treatment, 82 (35%) received a second immune modulatory treatment. A treatment-related fluctuation was observed in 53 (5%) of 1,023 patients, of whom 36 (68%) were re-treated with IVIg or PE. CONCLUSIONS: In current practice, patients with mild and variant forms of GBS, or with treatment-related fluctuations and treatment failures, are frequently treated, even in absence of trial data to support this choice. The variability in treatment practice can be explained in part by the lack of evidence and guidelines for effective treatment in these situations.


Assuntos
Síndrome de Guillain-Barré/terapia , Adolescente , Adulto , Criança , Pré-Escolar , Síndrome de Guillain-Barré/epidemiologia , Humanos , Estudos Prospectivos , Índice de Gravidade de Doença , Resultado do Tratamento
10.
World Neurosurg ; 128: 347-353, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31059858

RESUMO

BACKGROUND: Guillain-Barré syndrome (GBS) is an uncommon, yet life-threatening postoperative neuropathic complication that is easily neglected, and hence, timely treatment is not provided in the clinics. This review aims to summarize the clinical features of postoperative GBS, and thus, improve the understanding of postoperative GBS. METHODS: We reviewed the literature on postoperative GBS and assessed the demographic information, clinical manifestation, operation, time of onset of postoperative GBS, and prognosis. RESULTS: A total of 33 cases of postoperative GBS were included in this study. The average age of patients with postoperative GBS was 46.9 years, and there was a peak in occurrence of GBS between ages 50 and 70 years. Men seemed more likely to have postoperative GBS than did women, with a ratio of 2.67:1. Progressive muscular weakness, present in 31 of the cases, was the most common presentation. Patients with spinal surgery were at further increased risk for GBS, and 84.8% of the patients with GBS had a good prognosis after prompt treatment. CONCLUSIONS: Surgery is probably a potential risk factor for the occurrence of GBS. Early diagnosis and prompt treatment are imperative to reduce mortality.


Assuntos
Síndrome de Guillain-Barré/etiologia , Síndrome de Guillain-Barré/terapia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/terapia , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/epidemiologia , Humanos , Procedimentos Neurocirúrgicos/efeitos adversos , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia
11.
Medicine (Baltimore) ; 98(15): e15014, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30985649

RESUMO

RATIONALE: Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuropathy presumed to result from an infection-triggered autoimmune reaction. PATIENT CONCERNS: This paper describes a 53-year-old man admitted to hospital for deterioration of his general condition. DIAGNOSIS: He developed GBS, confirmed by lumbar puncture and electromyogram, which recovered after intravenous immunoglobulins. A grade 2 aortic regurgitation was detected by transthoracic echocardiography upon diagnosis of GBS, but in the absence of fever, no further investigations were conducted. A few weeks later, the patient presented with fever and infective endocarditis (IE) was diagnosed after the identification of vegetation on the aortic valve with transesophageal echocardiography. The etiologic agent was identified as Cardiobacterium hominis based on 3 positive blood cultures and DNA detection in valvular material. INTERVENTIONS: IE was cured with a 6-week course of antibiotics and aortic valve replacement. OUTCOMES: The patient completely recovered from Guillain-Baré syndrome and IE. LESSONS: This case of GBS associated with C hominis endocarditis, emphasizes the importance of blood cultures and transesophageal echocardiography for the detection of IE and highlights the insidious nature of C hominis endocarditis which is often diagnosed late.


Assuntos
Cardiobacterium , Endocardite Bacteriana/diagnóstico , Infecções por Bactérias Gram-Negativas/diagnóstico , Síndrome de Guillain-Barré/diagnóstico , Diagnóstico Diferencial , Endocardite Bacteriana/complicações , Endocardite Bacteriana/terapia , Infecções por Bactérias Gram-Negativas/complicações , Infecções por Bactérias Gram-Negativas/terapia , Síndrome de Guillain-Barré/complicações , Síndrome de Guillain-Barré/terapia , Humanos , Masculino , Pessoa de Meia-Idade
12.
BMJ Case Rep ; 12(4)2019 Apr 23.
Artigo em Inglês | MEDLINE | ID: mdl-31015248

RESUMO

Chikungunya (CHIK) viral fever is a self-limiting illness that presents with severe debilitating arthralgia, myalgia, fever and rash. Neurological complications are rare. We present a case of a 36-year-old woman who presented with acute onset progressive difficulty swallowing and left arm weakness. She was diagnosed with CHIK viral fever 4 weeks prior to admission. After investigations, she was diagnosed with a pharyngeal-cervical-brachial variant of Guillain-Barré syndrome. In hospital, she required ventilator support. Her condition improved after five sessions of intravenous immunoglobulin with almost complete resolution within 6 months of symptom onset. With frequent CHIK outbreaks, the neurological complications are increasingly seen in the emergency department. The knowledge of these associations will result in early diagnosis and treatment.


Assuntos
Febre de Chikungunya/virologia , Síndrome de Guillain-Barré/diagnóstico , Imunoglobulinas/uso terapêutico , Adulto , Febre de Chikungunya/complicações , Vírus Chikungunya/isolamento & purificação , Diagnóstico Diferencial , Diagnóstico Precoce , Eletromiografia/métodos , Feminino , Síndrome de Guillain-Barré/tratamento farmacológico , Síndrome de Guillain-Barré/fisiopatologia , Síndrome de Guillain-Barré/terapia , Humanos , Imunoglobulinas/administração & dosagem , Fatores Imunológicos/uso terapêutico , Resultado do Tratamento , Ventiladores Mecânicos/efeitos adversos
13.
PLoS One ; 14(2): e0211404, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30785890

RESUMO

BACKGROUND: Randomized controlled trials (RCTs) pose specific challenges in rare and heterogeneous neurological diseases due to the small numbers of patients and heterogeneity in disease course. Two analytical approaches have been proposed to optimally handle these issues in RCTs: covariate adjustment and ordinal analysis. We investigated the potential gain in efficiency of these approaches in rare and heterogeneous neurological diseases, using Guillain-Barré syndrome (GBS) as an example. METHODS: We analyzed two published GBS trials with primary outcome 'at least one grade improvement' on the GBS disability scale. We estimated the treatment effect using logistic regression models with and without adjustment for prognostic factors. The difference between the unadjusted and adjusted estimates was disentangled in imbalance (random differences in baseline covariates between treatment arms) and stratification (change of the estimate due to covariate adjustment). Second, we applied proportional odds regression, which exploits the ordinal nature of the GBS disability score. The standard error of the estimated treatment effect indicated the statistical efficiency. RESULTS: Both trials were slightly imbalanced with respect to baseline characteristics, which was corrected in the adjusted analysis. Covariate adjustment increased the estimated treatment effect in the two trials by 8% and 18% respectively. Proportional odds analysis resulted in lower standard errors indicating more statistical power. CONCLUSION: Covariate adjustment and proportional odds analysis most efficiently use the available data and ensure balance between the treatment arms to obtain reliable and valid treatment effect estimates. These approaches merit application in future trials in rare and heterogeneous neurological diseases like GBS.


Assuntos
Síndrome de Guillain-Barré/terapia , Doenças do Sistema Nervoso/terapia , Doenças Raras/terapia , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Modelos Logísticos , Metilprednisolona/uso terapêutico , Razão de Chances , Troca Plasmática , Ensaios Clínicos Controlados Aleatórios como Assunto/métodos , Ensaios Clínicos Controlados Aleatórios como Assunto/estatística & dados numéricos
16.
BMJ Case Rep ; 12(1)2019 Jan 14.
Artigo em Inglês | MEDLINE | ID: mdl-30642853

RESUMO

A 45-year-old man with a history of systemic lupus erythematosus presented with progressive weakness and areflexia. Electromyogram revealed reduced motor and sensory amplitudes without demyelinating features. He was clinically diagnosed with the acute motor and sensory axonal neuropathy variant of Guillain-Barré syndrome. Despite intravenous immunoglobulin therapy, he deteriorated with loss of all voluntary motor function and cranial nerve reflexes. Concomitant investigations revealed class V lupus nephritis. Therapy was initiated with plasma exchange, glucocorticoids and further immunosuppression, with gradual neurological recovery. We present the first documented case of fulminant Guillain-Barré syndrome as a neuropsychiatric manifestation of systemic lupus erythematosus, highlighting how immune-mediated polyneuropathy via diffuse deafferentation may mimic the outward appearance of brain death. While glucocorticoids are not indicated in idiopathic Guillain-Barré, when this neurological disorder is a consequence of systemic lupus erythematosus, immunomodulatory treatment should be initiated to prevent neurological deterioration.


Assuntos
Síndrome de Guillain-Barré/diagnóstico , Imunoglobulinas Intravenosas/uso terapêutico , Lúpus Eritematoso Sistêmico/complicações , Eletromiografia/métodos , Glucocorticoides/uso terapêutico , Síndrome de Guillain-Barré/etiologia , Síndrome de Guillain-Barré/fisiopatologia , Síndrome de Guillain-Barré/terapia , Humanos , Imunossupressão/métodos , Lúpus Eritematoso Sistêmico/patologia , Lúpus Eritematoso Sistêmico/terapia , Nefrite Lúpica/classificação , Nefrite Lúpica/diagnóstico , Nefrite Lúpica/terapia , Masculino , Pessoa de Meia-Idade , Plasmaferese/métodos , Resultado do Tratamento
17.
Indian J Pediatr ; 86(4): 329-334, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30637680

RESUMO

OBJECTIVES: To compare the clinical profile and short-term outcome of children with axonal and demyelinating subtypes of childhood Guillain Barré syndrome (GBS). METHODS: This is a prospective observational study conducted in a tertiary care teaching hospital in North India. Consecutive children with Guillain Barré syndrome were recruited to compare the clinical profile and short term outcome among the subtypes. RESULTS: Among 9847 children admitted to the emergency, 95 had acute flaccid paralysis; 57 of whom had GBS. Electrophysiological studies were completed in 57; of whom 20 had acute inflammatory demyelinating polyneuropathy (AIDP); 19 had acute motor axonal neuropathy (AMAN); 12 had non-reactive nerves; five were unclassifiable; 1 had acute motor sensory axonal neuropathy (AMSAN). More children in AMAN group had preceding gastroenteritis (4 vs. 2), while AIDP group had upper respiratory infections (12 vs. 7). Ataxia was only seen in AIDP subtype while wrist drop, foot drop and hyperreflexia were seen only with AMAN subtype. Respiratory muscle involvement (6 vs. 3) and artificial ventilation (5 vs. 2) was more in AMAN. At discharge, children with AIDP were less likely to be non-ambulant (12 vs. 6, p = 0.036). Mean disability scores at hospital discharge (4.9 ± 1.2 vs. 4 ± 0.9, p = 0.015) and at last follow-up (0.7 ± 1.01 vs. 0.05 ± 0.2, p = 0.016) were higher in AMAN. Children with AIDP were more likely to achieve normalcy on follow-up (19 vs. 12, p = 0.023). CONCLUSIONS: Children with AMAN appear to have a more severe clinical course; higher short-term morbidity; and slower recovery than those with AIDP.


Assuntos
Síndrome de Guillain-Barré/patologia , Pré-Escolar , Estudos Transversais , Feminino , Síndrome de Guillain-Barré/terapia , Humanos , Índia , Masculino , Neurônios Motores/patologia , Condução Nervosa/fisiologia , Estudos Prospectivos , Resultado do Tratamento
19.
J Clin Neurosci ; 62: 195-198, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30477890

RESUMO

Outcomes of Guillain Barre Syndrome (GBS), particularly those require mechanical ventilation have been reported from a number of Asian countries, albeit, scarcely from Pakistan. We conducted this study to determine the short-term outcomes of GBS and compare the results of mechanically ventilated and non-ventilated patients. Case records of patients admitted with GBS during 2011-2016 at a large tertiary care centre of Pakistan were retrospectively reviewed. 216 patients satisfying inclusion criteria were included. Patients were divided into 2 groups based on requirement of MV (MV and non-MV group). Short term outcomes were assessed by Modified Rankin Scale (MRS) at discharge, 2 weeks and 3 months and comparison done between MV and non-MV group. Outcome based on MRS score is categorized as good (MRS = 0-3) or poor (MRS = 4-6). Requirement for MV was noted in 24.5%. MV patients had severe weakness at presentation, longer length of hospital stay (LOS) and higher frequency of in-hospital complications. Overall mortality was 7.9%. Good outcomes at discharge and at 3 months were noted in significantly higher frequency in non-MV group (50.3% and 93.2% respectively) as compared to MV group (11.3% and 33.3% respectively). In MV group, increasing age, areflexia and longer LOS stay were found as independent predictors of poor outcome. Overall outcomes of GBS in our population are comparable to both regional and international studies. However, poor outcomes in MV group are seen in higher frequency in our study. Increasing age, areflexia and longer LOS may predict poor outcome in MV patients.


Assuntos
Síndrome de Guillain-Barré , Resultado do Tratamento , Adulto , Idoso , Países em Desenvolvimento/estatística & dados numéricos , Feminino , Síndrome de Guillain-Barré/terapia , Humanos , Pessoa de Meia-Idade , Paquistão , Respiração Artificial , Estudos Retrospectivos , Centros de Atenção Terciária/estatística & dados numéricos
20.
Muscle Nerve ; 59(2): 194-200, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30390307

RESUMO

INTRODUCTION: Although patients with Guillain-Barré syndrome frequently require orotracheal intubation and tracheostomy, the incidence and relevance of neurogenic dysphagia prior to intubation and risk factors for prolonged requirement for a tracheal cannula have not yet been identified. METHODS: Retrospective analysis of the medical records of 88 patients was performed. Clinical characteristics were compared between intubated and nonintubated patients and between immediately decannulated and not immediately decannulated patients. RESULTS: Thirty-five (39.7%) patients required tracheostomy. Neuromuscular weakness and related respiratory insufficiency were the main reasons for intubation. In the subgroup of tracheotomized patients, immediate decannulation after completed respiratory weaning was possible in 14 (40%) patients. The severity of dysphagia, in particular pharyngolaryngeal hypesthesia, was related to the length of cannulation. DISCUSSION: Respiratory muscle weakness is the main reason for intubation, whereas neurogenic dysphagia is the main risk factor for persisting cannulation. Dysphagia after weaning is most frequently characterized by severe laryngeal sensory deficit. Muscle Nerve 59:194-200, 2019.


Assuntos
Transtornos de Deglutição/etiologia , Síndrome de Guillain-Barré/complicações , Síndrome de Guillain-Barré/terapia , Intubação Intratraqueal/efeitos adversos , Insuficiência Respiratória/etiologia , Traqueostomia/efeitos adversos , Adulto , Idoso , Transtornos de Deglutição/terapia , Remoção de Dispositivo , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Respiração Artificial/efeitos adversos , Estudos Retrospectivos , Índice de Gravidade de Doença
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