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1.
Muscle Nerve ; 64(2): 140-152, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33786855

RESUMO

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


Assuntos
Doenças do Sistema Nervoso Periférico/mortalidade , Doenças do Sistema Nervoso Periférico/patologia , Polineuropatias , Sintase do Porfobilinogênio/deficiência , Porfirias Hepáticas/mortalidade , Porfirias Hepáticas/patologia , Ácido Aminolevulínico/metabolismo , Síndrome de Guillain-Barré/mortalidade , Síndrome de Guillain-Barré/patologia , Humanos , Doenças do Sistema Nervoso Periférico/diagnóstico , Polineuropatias/mortalidade , Polineuropatias/patologia , Nervo Radial/patologia
2.
Eur J Neurol ; 27(11): 2361-2370, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32757404

RESUMO

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


Assuntos
COVID-19/complicações , Síndrome de Guillain-Barré/etiologia , COVID-19/mortalidade , Doenças Desmielinizantes/etiologia , Síndrome de Guillain-Barré/mortalidade , Humanos
3.
J Peripher Nerv Syst ; 25(4): 350-355, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-32869405

RESUMO

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


Assuntos
Síndrome de Guillain-Barré/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Síndrome de Guillain-Barré/mortalidade , Hospitalização/estatística & dados numéricos , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Estudos Retrospectivos , Sérvia/epidemiologia , Centros de Atenção Terciária/estatística & dados numéricos , Adulto Jovem
4.
Neurocrit Care ; 33(3): 759-768, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-32291575

RESUMO

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


Assuntos
Síndrome de Guillain-Barré , Mortalidade Hospitalar , Respiração Artificial , Traqueostomia , Adulto , Síndrome de Guillain-Barré/mortalidade , Humanos , Unidades de Terapia Intensiva , Tempo de Internação , Masculino , Estudos Retrospectivos
5.
Biol Blood Marrow Transplant ; 25(8): 1629-1636, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31048087

RESUMO

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


Assuntos
Doença Enxerto-Hospedeiro , Síndrome de Guillain-Barré , Transplante de Células-Tronco Hematopoéticas , Fatores Imunológicos/administração & dosagem , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Tiamina/administração & dosagem , Vitamina B 12/administração & dosagem , Adolescente , Adulto , Aloenxertos , Doença Crônica , Intervalo Livre de Doença , Feminino , Doença Enxerto-Hospedeiro/tratamento farmacológico , Doença Enxerto-Hospedeiro/etiologia , Doença Enxerto-Hospedeiro/mortalidade , Síndrome de Guillain-Barré/tratamento farmacológico , Síndrome de Guillain-Barré/etiologia , Síndrome de Guillain-Barré/mortalidade , Humanos , Incidência , Masculino , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/tratamento farmacológico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/mortalidade , Fatores de Risco , Taxa de Sobrevida
6.
Semin Neurol ; 39(5): 589-595, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31639842

RESUMO

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


Assuntos
Axônios/patologia , Síndrome de Guillain-Barré/epidemiologia , Paralisia/etiologia , Doença Aguda/mortalidade , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/mortalidade , Humanos , Morbidade , Paralisia/diagnóstico
7.
J Paediatr Child Health ; 54(3): 247-253, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28960591

RESUMO

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


Assuntos
Síndrome de Guillain-Barré/complicações , Hipotonia Muscular/etiologia , Paralisia/etiologia , Respiração Artificial , Atividades Cotidianas , Pré-Escolar , Feminino , Síndrome de Guillain-Barré/mortalidade , Síndrome de Guillain-Barré/terapia , Humanos , Lactente , Modelos Logísticos , Masculino , Respiração Artificial/estatística & dados numéricos , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/terapia , Estudos Retrospectivos , África do Sul/epidemiologia
8.
J Peripher Nerv Syst ; 22(2): 121-126, 2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-28447405

RESUMO

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.


Assuntos
Síndrome de Guillain-Barré/epidemiologia , Síndrome de Guillain-Barré/mortalidade , Adolescente , Adulto , Distribuição por Idade , Bangladesh/epidemiologia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem
9.
Int J Neurosci ; 126(11): 985-8, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26335975

RESUMO

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


Assuntos
Síndrome de Guillain-Barré/mortalidade , Mortalidade/tendências , Sistema de Registros/estatística & dados numéricos , Idoso , Idoso de 80 Anos ou mais , Feminino , Síndrome de Guillain-Barré/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Espanha/epidemiologia
10.
Ideggyogy Sz ; 69(11-12): 389-395, 2016 Nov 30.
Artigo em Inglês | MEDLINE | ID: mdl-29733556

RESUMO

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


Assuntos
Síndrome de Guillain-Barré/tratamento farmacológico , Imunoglobulinas Intravenosas/uso terapêutico , Adulto , Idoso , Feminino , Síndrome de Guillain-Barré/mortalidade , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos
11.
Sci Rep ; 14(1): 15520, 2024 07 05.
Artigo em Inglês | MEDLINE | ID: mdl-38969647

RESUMO

Guillain-Barré syndrome (GBS) is an acute autoimmune polyneuropathy with substantial geographic variations in demography, antecedent events, clinical manifestations, electrophysiological sub-types, diagnostic findings, treatment modalities, and prognostic indicators. However, there is limited contemporary data on GBS patient profiles and prognostic factors from low-resource settings like Ethiopia. The objective of this study is to investigate the clinical profile, factors associated with mortality, and hospital outcomes among GBS patients admitted to Tikur Anbessa Specialized Hospital (TASH) in Addis Ababa, Ethiopia. A retrospective cross-sectional study was conducted among 60 GBS patients admitted to TASH from January 2018 to December 2022. Data on demographics, clinical features, treatments, complications, and outcomes were extracted from medical records. Bivariate and multivariate logistic regression analyses identified factors associated with mortality and poor hospital outcomes. The cohort had a mean age of 28.5 years, with 76.7% aged 14-34 years. Males comprised 61.7% of cases. Ascending paralysis (76.7%) was the predominant presentation. Absent or reduced reflexes were seen in 91.7% of patients. The most common antecedent event was gastroenteritis (26.7%), followed by upper respiratory tract infection (URTI) (15%) and vaccination (11.7%). The mean interval from symptom onset to hospital presentation was 8.77 days, and the peak symptom severity was 4.47 days. The axonal variant (75.5%) was the most common subtype, followed by the demyelinating variant (24.5%). Intravenous immunoglobulin was administered to 41.7% of patients. Respiratory failure requiring invasive mechanical ventilator (MV) support occurred in 26.7% of cases. The mortality rate was 10%, with mechanical ventilation being the only factor significantly associated with mortality (95% CI 2.067-184.858; P < 0.010). At discharge, 55% had a good outcome, and 45% had a poor outcome, according to the Hughes Functional Disability Scale (HFDS). Mechanical ventilation (AOR 0.024, 95% CI 0.001-0.607) and a GBS disability score > 3 (AOR 0.106, 95% CI 0.024-0.467) were factors significantly associated with poor hospital outcomes. GBS in this cohort primarily affected individuals of young age, commonly preceded by gastroenteritis and characterized by a high frequency of the axonal variant. Mechanical ventilation was found to be significantly linked to mortality. Alongside mechanical ventilation requirements, severe disability upon presentation emerged as a crucial determinant of poor outcomes upon discharge, underscoring the importance of early identification of high-risk patients and prompt interventions.


Assuntos
Síndrome de Guillain-Barré , Mortalidade Hospitalar , Humanos , Síndrome de Guillain-Barré/mortalidade , Síndrome de Guillain-Barré/terapia , Masculino , Feminino , Adulto , Estudos Retrospectivos , Etiópia/epidemiologia , Adolescente , Adulto Jovem , Estudos Transversais , Pessoa de Meia-Idade , Prognóstico , Fatores de Risco
12.
Semin Dial ; 25(2): 132-9, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22277020

RESUMO

Several neurologic disorders have been treated with therapeutic plasma exchange (TPE). Case reports, case series, and clinical trials have published results regarding the outcomes in such patients. The data gathered have been used to formulate evidence-based guidelines, which can be used to guide therapy in patients with these neurological disorders. Adequately designed and powered randomized controlled trials have proven the efficacy of TPE in some disease entities, while other diseases are lacking such data. In the latter, decisions for the use of TPE must be made using the limited published data available. In this review, we discuss the published evidence regarding the use of TPE in neurological disorders, focusing on the most recent guidelines published by the American Society of Apheresis in 2010 and the American Academy of Neurology in 2011.


Assuntos
Medicina Baseada em Evidências , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/terapia , Troca Plasmática/métodos , Encefalomielite Aguda Disseminada , Feminino , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/mortalidade , Síndrome de Guillain-Barré/terapia , Humanos , Masculino , Esclerose Múltipla/diagnóstico , Esclerose Múltipla/mortalidade , Esclerose Múltipla/terapia , Miastenia Gravis/diagnóstico , Miastenia Gravis/mortalidade , Miastenia Gravis/terapia , Doenças do Sistema Nervoso/mortalidade , Troca Plasmática/efeitos adversos , Polirradiculoneuropatia/diagnóstico , Polirradiculoneuropatia/mortalidade , Polirradiculoneuropatia/terapia , Prognóstico , Ensaios Clínicos Controlados Aleatórios como Assunto , Medição de Risco , Taxa de Sobrevida , Resultado do Tratamento
14.
Crit Care ; 14(4): R136, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-20646313

RESUMO

INTRODUCTION: There are only limited data on the long-term outcome of patients receiving specialized neurocritical care. In this study we analyzed survival, long-term mortality and functional outcome after neurocritical care and determined predictors for good functional outcome. METHODS: We retrospectively investigated 796 consecutive patients admitted to a non-surgical neurologic intensive care unit over a period of two years (2006 and 2007). Demographic and clinical parameters were analyzed. Depending on the diagnosis, we grouped patients according to their diseases (cerebral ischemia, intracranial hemorrhage (ICH), subarachnoid hemorrhage (SAH), meningitis/encephalitis, epilepsy, Guillain-Barré syndrome (GBS) and myasthenia gravis (MG), neurodegenerative diseases and encephalopathy, cerebral neoplasm and intoxication). Clinical parameters, mortality and functional outcome of all treated patients were analyzed. Functional outcome (using the modified Rankin Scale, mRS) one year after discharge was assessed by a mailed questionnaire or telephone interview. Outcome was dichotomized into good (mRS ≤ 2) and poor (mRS ≥ 3). Logistic regression analyses were calculated to determine independent predictors for good functional outcome. RESULTS: Overall in-hospital mortality amounted to 22.5% of all patients, and a good long-term functional outcome was achieved in 28.4%. The parameters age, length of ventilation (LOV), admission diagnosis of ICH, GBS/MG, and inoperable cerebral neoplasm as well as Therapeutic Intervention Scoring System (TISS)-28 on Day 1 were independently associated with functional outcome after one year. CONCLUSIONS: This investigation revealed that age, LOV and TISS-28 on Day 1 were strongly predictive for the outcome. The diagnoses of hemorrhagic stroke and cerebral neoplasm leading to neurocritical care predispose for functional dependence or death, whereas patients with GBS and MG are more likely to recover after neurocritical care.


Assuntos
Cuidados Críticos/estatística & dados numéricos , Estado Terminal , Doenças do Sistema Nervoso/terapia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias Encefálicas/mortalidade , Neoplasias Encefálicas/terapia , Estado Terminal/mortalidade , Epilepsia/mortalidade , Epilepsia/terapia , Feminino , Síndrome de Guillain-Barré/mortalidade , Síndrome de Guillain-Barré/terapia , Mortalidade Hospitalar , Humanos , Modelos Logísticos , Masculino , Meningoencefalite/mortalidade , Meningoencefalite/terapia , Pessoa de Meia-Idade , Análise Multivariada , Miastenia Gravis/mortalidade , Miastenia Gravis/terapia , Doenças do Sistema Nervoso/mortalidade , Estudos Retrospectivos , Estatísticas não Paramétricas , Acidente Vascular Cerebral/mortalidade , Acidente Vascular Cerebral/terapia , Hemorragia Subaracnóidea/mortalidade , Hemorragia Subaracnóidea/terapia , Adulto Jovem
16.
Eur Neurol ; 61(6): 358-63, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-19365128

RESUMO

PURPOSE: To examine the effects of age on differences in the clinical course and functional outcome of mechanically ventilated Guillain-Barré syndrome (GBS) patients. METHODS: 32 consecutive patients with GBS in need of mechanical ventilation were divided into two groups: (1) 15 patients <65 years, and (2) 17 patients >or=65 years. Length of ventilation, therapy, incidence of bulbar involvement, dysautonomia, preceding infections, need for tracheostomy, mortality, and functional outcome at 1-year follow-up using the Hughes outcome scale were analyzed. RESULTS: The groups were comparable regarding treatment, lesion type, incidence of bulbar involvement, dysautonomia, preceding infections and duration of ventilation. The majority of patients (75%) received combination therapy (intravenous immunoglobulin and plasma exchange). Tracheostomy was performed in 91% of patients. Mortality was higher in older patients (41 vs. 7%; p = 0.02) and overall outcome worse (p = 0.05). Interestingly, once surviving the early, most critical period, older patients functionally recovered as well as younger patients (80% good outcome vs. 86%; p = 0.71). CONCLUSIONS: Despite all the advances in intensive care, mortality in older, ventilated GBS patients is still substantial. However, once they have survived the early, most critical period, older patients may recover as well as younger patients. Special care should therefore be taken to prevent and, if present, to vigorously treat complications commonly associated with intensive care treatment of severe GBS in elderly patients.


Assuntos
Síndrome de Guillain-Barré/terapia , Respiração Artificial , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Seguimentos , Síndrome de Guillain-Barré/mortalidade , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Entrevistas como Assunto , Pessoa de Meia-Idade , Troca Plasmática , Recuperação de Função Fisiológica , Análise de Regressão , Traqueostomia , Resultado do Tratamento , Adulto Jovem
17.
PLoS One ; 14(5): e0216867, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31091282

RESUMO

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


Assuntos
Infecções por Campylobacter/mortalidade , Campylobacter , Efeitos Psicossociais da Doença , Gastroenterite/mortalidade , Síndrome de Guillain-Barré/mortalidade , Doença Aguda , Feminino , Alemanha/epidemiologia , Humanos , Masculino
18.
J Child Neurol ; 34(5): 277-283, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30696330

RESUMO

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


Assuntos
Síndrome de Guillain-Barré/terapia , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Troca Plasmática , Adolescente , Criança , Feminino , Síndrome de Guillain-Barré/mortalidade , Humanos , Tempo de Internação , Masculino , Troca Plasmática/métodos , Respiração Artificial , Índice de Gravidade de Doença , Resultado do Tratamento
19.
J Neurol Sci ; 264(1-2): 121-8, 2008 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-17881005

RESUMO

UNLABELLED: One third of patients with Guillain-Barré syndrome (GBS) require admission to the intensive care unit (ICU), associated with significant risk of morbidity, mortality, and incomplete recovery. METHODS: 76 adult patients with GBS admitted to the ICU at a regional referral center over a 20-year period were studied. We determined the frequency, nature, and predictors of complications they experienced while in the ICU; this morbidity was related to long-term functional recovery and time to regain independent ambulation, extracted from longitudinal follow-up data. RESULTS: ICU stay was a median 21 days and mechanical ventilation (MV) was required in 78% (median duration 28 days). Two-thirds suffered at least one major complication, most commonly pneumonia (54%). Morbidity was strongly associated with MV and male sex. Mortality occurred in only 5 patients (6.5%). Over an average 3 years follow-up, recovery of independent ambulation was seen in 75%, with advanced age being the most powerful predictor of poor outcome. Prolonged MV and severe axonal loss did not preclude a favorable recovery. Time to ambulate was a median 198 days, although recovery could occur as late as ten years after onset; slower recovery was associated with ICU complications, prolonged MV, and early axonal abnormalities. CONCLUSION: Although patients with GBS suffer significant morbidity during protracted ICU stays, with meticulous supportive care, many make gratifying functional recoveries. In severely afflicted patients, this may only be appreciated after extended follow-up.


Assuntos
Síndrome de Guillain-Barré/mortalidade , Unidades de Terapia Intensiva/estatística & dados numéricos , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Comorbidade , Feminino , Seguimentos , Síndrome de Guillain-Barré/enfermagem , Humanos , Tempo de Internação/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Nervos Periféricos/patologia , Nervos Periféricos/fisiopatologia , Pneumonia/mortalidade , Pneumonia/enfermagem , Recuperação de Função Fisiológica/fisiologia , Respiração Artificial/estatística & dados numéricos , Estudos Retrospectivos , Distribuição por Sexo , Fatores de Tempo , Degeneração Walleriana/etiologia , Degeneração Walleriana/patologia , Degeneração Walleriana/fisiopatologia
20.
West Afr J Med ; 27(3): 167-70, 2008 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-19256323

RESUMO

BACKGROUND: Guillain Barre Syndrome (GBS) is a neurological disorder that poses a great challenge to medical care. It affects all age groups, gender and socio-economic groups. OBJECTIVE: To determine the pattern of clinical presentation as well as the factors that determine morbidity and mortality in Guillain Barre Syndrome (GBS) patients. METHODS: The case records of all the patients with diagnosis of GBS from 1988-2005 were retrieved. Socio-demographic, clinical data and laboratory investigations were collated. RESULTS: There were a total of 14 patients managed during this period, which comprised seven males and seven females. The mean(SD) age was 23.6 (13.3) years. Nine (64.3%) patients were students, only one (8%) patient was a professional and the rest 4 (36%) were artisans. Five (36%) patients presented with weakness of both upper and lower limbs while another five (36%) patients presented with weakness of the lower limbs only and four (28%) patients had bulbar symptoms in addition to weakness of the upper and lower limbs. The most common prodromal symptoms were headache, fever and joint pains. The common symptoms at presentation were excessive sweating paraesthesia (43%), urinary hesitancy and retention (35%). Physical findings include Flaccid quadriparesis 13 (93%), autonomic dysfunction 9 (64.3%), sensory impairment (71.4%) and cranial neuropathies 6 (43.5%). Albuminocytological dissociation was present in the cerebrospinal fluid of five (56%) of nine patients who had lumbar puncture done and erythrocyte sedimentation rate (ESR) was elevated in five patients (38%). The sex, age presenting complaints and treatment given were found not to have correlation with clinical outcome. The pattern of motor paresis and the nature of discharge patient had were significantly correlated with clinical outcome. CONCLUSION: Guillain Barre syndrome present impotant challenge to medical care in Nigeria and it is hoped that this study would sensitize clinicians to the clinical burden of Guillain Barre syndrome among Nigerians.


Assuntos
Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/mortalidade , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Hospitais Universitários , Humanos , Masculino , Pessoa de Meia-Idade , Morbidade , Nigéria/epidemiologia , Estudos Retrospectivos , Fatores de Risco
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