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1.
Beijing Da Xue Xue Bao Yi Xue Ban ; 51(4): 775-777, 2019 Aug 18.
Artigo em Chinês | MEDLINE | ID: mdl-31420639

RESUMO

Guillain-Barre syndrome (GBS) is an autoimmune disease on the injury of peripheral nerve myelin proteins or axon, of which the acute motor axonal neuropathy (AMAN) as a subtype is of infrequence and an extremely low incidence of post-operation. This article originally reported one case from Peking University People's Hospital on successful treatment of severe GBS (AMAN) on post-operation with renal carcinoma and meningioma. The diagnostic criteria of AMAN refer to AIDP, of which the feature of AMAN suggests a pure motor nerve dysfunction and significant damage on motor axon. It is reported that infection and surgery may induce GBS. The positive result of IgM and IgG was considered the application of ganglioside and blood-brain barrier might be damaged after meningioma surgery which eased the drug to enter the cerebrospinal fluid circulation and induced lesions, therefore the etiology on this GBS case was of high confidence of administrating ganglioside drugs. Autonomic nerve dysfunctions, such as blood pressure fluctuations and arrhythmia could be caused in GBS, of which about 3%-10% of GBS patients would die. Early use of gamma globulin or plasma exchange was recommended internationally, but recently some new ideas, to some extent, of significance on GBS treatment emerged. However, there was still no consensus on GBS treatment systematically all over the world. Till now, the general treatment program on GBS may be still gamma globulin or plasma exchange and a curious judgment of prognosis is essential in order to make a reasonable plan. That it was usually of no omen on severe autonomic nerve dysfunction must be successively monitored, the same as the management of the respiratory tract and nutrition support. The key measures taken on lung recruitment was postural drainage on this case with a low cost but a qualified effectiveness. This case report aimed to deepen the understanding of AMAN and acquaint the cutting-edge advances on the treatment of GBS, as well as providing successful treatment experience for the prevention on similar cases.


Assuntos
Carcinoma de Células Renais , Síndrome de Guillain-Barré , Neoplasias Renais , Neoplasias Meníngeas , Meningioma , Humanos
2.
Lima; Perú. Ministerio de Salud; 20190822. 37 p. tab, graf.
Monografia em Espanhol | LILACS, LIPECS | ID: biblio-1010285

RESUMO

Contribuir al adecuado tratamiento de la población afectada por el SGB, garantizando la continuidad de los servicios de salud en los departamentos con incremento inusual de casos, a fin de reducir los daños colaterales y/o secuelas neurológicas


Assuntos
Resultado do Tratamento , Assistência Integral à Saúde , Síndrome de Guillain-Barré , Planos de Emergência
3.
J Assoc Physicians India ; 67(4): 56-59, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31309800

RESUMO

Objectives: To study clinical characteristics of various forms of Guillain-Barre syndrome in Indian adults. Material and Methods: The epidemiological, clinical, cerebrospinal fluid and electrophysiological data of 65 patients of Guillain-Barre syndrome (GBS) were reviewed in a retrospective study. Results: Analysis of age distribution disclosed a high incidence (36.92%) in young adults between 18 to 29 years of age. Seasonal preponderance in winter and summer was found. Preceding events were identified in 22 (33.84%) cases. Motor weakness, areflexia, and facial weakness were the most common clinical features. Cerebrospinal fluid albuminocytological dissociation was present in 80% of patients. Utilising clinical and electrophysiological data, these 65 patients with Guillain-Barre syndrome were subclassified as acute demyelinating polyradiculoneuropathy 17 (26.15%), axonal form 17 (26.15%), Fisher's syndrome 2 (3.07%)and ataxic variant 1(1.53). The remaining 28 (43.07%) patients were unclassified. 9(13.8%) patients had recurrent GBS. Only 5 (7.7%) patients required mechanical ventilation. Follow up available on 47 patients disclosed that all of them recovered satisfactorily. No patient was persistently disabled and no mortality occurred during hospitalization. Conclusions: GBS in Indian population from northwest India showed peculiar age, seasonal distribution and high frequency of both AIDP and axonal subtypes. Both, axonal and demyelinating subtypes shared common clinical features and had good prognosis.


Assuntos
Síndrome de Guillain-Barré/epidemiologia , Adolescente , Adulto , Distribuição por Idade , Humanos , Incidência , Índia/epidemiologia , Estudos Retrospectivos , Adulto Jovem
4.
BMC Neurol ; 19(1): 145, 2019 Jun 28.
Artigo em Inglês | MEDLINE | ID: mdl-31253118

RESUMO

BACKGROUND: Fulminant Guillain-Barré syndrome (GBS) is characterized clinically by rapid progression of severe symptoms, such as the absence of brainstem reflexes, complete tetraplegia and respiratory arrest. The clinical course of fulminant GBS remains unclear. Here, we report a patient with fulminant GBS, who showed severe weakness of the pharyngeal-cervical-branchial (PCB) area in the recovery phase. CASE PRESENTATION: A 38-year-old man rapidly developed fulminant GBS. In blood examination, he was positive for a broad range of anti-ganglioside antibodies, including anti-GQ1b, GT1a, GT1b, GD1a, GD1b and GD3 IgG antibodies. We performed immunosuppressive therapies using intravenous immunoglobulin and intravenous methylprednisolone. Although disturbance of consciousness and weakness of the distal upper and lower limbs improved gradually, weakness of the oropharynx, neck, and proximal upper limbs were resistant to these therapies. Anti-GT1a IgG antibodies remained persistently positive. Consequently, mechanical ventilation and tube feeding were required for 7 and 10 months, respectively. Two years later, weakness of the proximal upper limbs and mild respiratory dysfunction remained as sequelae. CONCLUSION: Anti-GT1a IgG antibodies are known to be detected in patients with the PCB variant of GBS. In fulminant GBS, the persistent presence of anti-GT1a IgG antibodies may be associated with occurrence of severe PCB-like weakness in the recovery phase.


Assuntos
Autoanticorpos/sangue , Síndrome de Guillain-Barré/complicações , Síndrome de Guillain-Barré/imunologia , Debilidade Muscular/imunologia , Adulto , Progressão da Doença , Gangliosídeos/imunologia , Síndrome de Guillain-Barré/sangue , Humanos , Masculino , Pescoço , Orofaringe , Extremidade Superior
5.
Brain Nerve ; 71(6): 581-587, 2019 Jun.
Artigo em Japonês | MEDLINE | ID: mdl-31171755

RESUMO

Complement activation is involved in the pathogenetic mechanism of Guillain-Barré syndrome (GBS). To date, the effectiveness of complement inhibitors for GBS has been shown by in vitro and in vivo studies. A recent Japanese randomized controlled trial with eculizumab, a monoclonal antibody against the complement C5, indicated that eculizumab might improve the outcomes of GBS patients at six months from onset. In future, the prognosis of severe GBS cases may possibly be improved by a novel therapy targeting the complement.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Inativadores do Complemento/uso terapêutico , Proteínas do Sistema Complemento , Síndrome de Guillain-Barré/tratamento farmacológico , Síndrome de Guillain-Barré/patologia , Anticorpos Monoclonais , Complemento C5/antagonistas & inibidores , Humanos , Prognóstico , Ensaios Clínicos Controlados Aleatórios como Assunto
7.
Zhonghua Yi Xue Za Zhi ; 99(23): 1800-1804, 2019 Jun 18.
Artigo em Chinês | MEDLINE | ID: mdl-31207691

RESUMO

Objective: To investigate the clinical characterization, treatment and prognosis of anti-GQ1b antibody syndrome. Methods: The clinical data of 8 patients with positive serum anti-GQ1b antibody from the Department of Neurology of Nanjing Brain Hospital between June 2016 and July 2018 were analyzed retrospectively. Their serums were tested by immunoblotting. Relevant literatures were reviewed to investigate possible pathogenesis. Results: Of the 8 cases, 4 cases were male, 4 cases were female; their age ranged from 16 to 76 (47±21) years old. Seven of them were with acute onset, the time course of the disease ranged from 2 to 15 (7±4) days. Six cases had a history of influenza prior to the onset of the presenting symptoms. In terms of the clinical manifestations of the eight patients, two were affected with Guillain-Barre syndrome (GBS), two with Cavernous sinus syndrome, one with Miller Fisher syndrome, one with both GBS and spinal cord demyelination, one with Bulbar paralysis, and one with chronic inflammatory demyelinating polyneuropathy (CIDP). The anti-GQ1b antibody IgG in serum was positive in 6 patients, two of whom were combined with positive IgG of anti-GD1b antibody in serum. The anti-GQ1b antibody IgM in serum was positive in 1 patient, and the anti-GQ1b antibody IgM and anti-GT1b antibody IgM in cerebrospinal fluid (CSF) were both positive in the other patient. In terms of the treatment, 3 patients (3/8) received vitamin B treatment only, 2 patients (2/8) received steroid plus vitamin B treatment, 2 patients (2/8) received intravenous immunoglobulin (IVIG) plus vitamin B treatment, and 1 patient (1/8) received steroid plus IVIG treatment. During the 8-33 months' follow-up after discharge, 6 patients were significantly improved in their symptoms, one with mild diplopia, one with limbs weakness, numbness and difficulty in walking. The symptoms of one patient (case 3) fluctuated twice and recovered again after treatment. Conclusions: The disease spectrum of anti-GQ1b antibodies syndrome is broad, and main symptom is ophtalmoplegia. Immunotherapy with IVIG and steroid would be beneficial to prognosis.


Assuntos
Síndrome de Guillain-Barré , Síndrome de Miller Fisher , Adolescente , Adulto , Idoso , Autoanticorpos , Feminino , Gangliosídeos , Humanos , Imunoglobulina M , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
8.
Recurso na Internet em Português | LIS - Localizador de Informação em Saúde | ID: lis-LISBR1.1-46573

RESUMO

A epidemia mundial de infecção pelo Zika vírus vem preocupando, principalmente as gestantes, por causa do risco de terem bebês com microcefalia. Porém existe também uma outra preocupação relacionda com essa infecção: a possibilidade de que o vírus da Zika produza a chamada síndrome de Guillain-Barré. Essa é uma forma rara de paralisia aguda que pode levar um quarto das pessoas afetadas a precisarem de aparelhos até para respirar.


Assuntos
Síndrome de Guillain-Barré , Zika virus , Influenza Humana , Pneumonia , HIV , Infecções Bacterianas , Viroses , Paralisia
9.
Mymensingh Med J ; 28(2): 449-455, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31086165

RESUMO

Guillain-Barre syndrome (GBS) is related with significant morbidity and also mortality. Little is known about the long term outcome of GBS patients who survived. The objective of this study is to determine the lasting outcome and consequences of GBS patients. This is a cross-sectional study of patients who diagnosed GBS and managed at the Intensive Care Unit of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from January 2004 to December 2017. All survived patients were invited for a structured interview, questionnaires, and full neurologic exam to record their current clinical condition focused on complaints and symptoms, neurological deficits, disabilities, behaviour, and quality of life. Thirty-eight patients participated, with a median age of 20 years (range 4-39 years) and a median interviewed time of 7 years (range 1-13 years). Residual complaints were reported by 24(63%) patients, including paresthesias (10.5%), unsteadiness of gait (37%), painful hands or feet (29%), and severe fatigue (13%). Questionnaires identified a wide range of behavioural problems. Most Patients showed good recovery of neurological deficits after GBS, but many have persisting long-term residual complaints and symptoms that may lead to psychosocial problems interfering with participation in daily life.


Assuntos
Síndrome de Guillain-Barré/complicações , Síndrome de Guillain-Barré/psicologia , Qualidade de Vida/psicologia , Adolescente , Adulto , Bangladesh/epidemiologia , Criança , Pré-Escolar , Estudos Transversais , Fadiga/epidemiologia , Síndrome de Guillain-Barré/epidemiologia , Humanos , Unidades de Terapia Intensiva , Parestesia/epidemiologia , Inquéritos e Questionários , Resultado do Tratamento , Adulto Jovem
10.
Cell Prolif ; 52(4): e12634, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31094043

RESUMO

OBJECTIVES: Guillain-Barré syndrome (GBS) is a type of acute autoimmune disease, which occurs in peripheral nerves and their roots. There is extensive evidence that suggests many immune-associated genes have essential roles in GBS. However, the associations between immune genes and GBS have not been sufficiently examined as most previous studies have only focused on individual genes rather than their entire interaction networks. MATERIALS AND METHODS: In this study, multiple levels of data including immune-associated genes, GBS-associated genes, protein-protein interaction (PPI) networks and gene expression profiles were integrated, and an immune or GBS-directed neighbour co-expressed network (IOGDNC network) and a GBS-directed neighbour co-expressed network (GDNC network) were constructed. RESULTS: Our analysis shows the immune-associated genes are strongly related to GBS-associated genes whether at the interaction level or gene expression level. Five immune-associated modules were also identified which could distinguish between GBS and normal samples. In addition, functional analysis indicated that immune-associated genes are essential in GBS. CONCLUSIONS: Overall, these results highlight a strong relationship between immune-associated genes and GBS existed and provide a potential role for immune-associated genes as novel diagnostic and therapeutic biomarkers for GBS.


Assuntos
Síndrome de Guillain-Barré/genética , Transcriptoma/genética , Humanos , Mapas de Interação de Proteínas/genética
11.
Zhonghua Er Ke Za Zhi ; 57(5): 363-367, 2019 May 02.
Artigo em Chinês | MEDLINE | ID: mdl-31060129

RESUMO

Objective: To summarize the clinical features of Bickerstaff brainstem encephalitis (BBE) in children. Methods: In this retrospective study, data of 19 patients with BBE (11 males and 8 females) were collected from Department of Neurology, Beijing Children's Hospital from October 2015 to January 2018. The clinical features, treatment and prognosis were analyzed. Results: The onset age of BBE ranged from 1 year and 8 months to 12 years and 11 months. There were 18 cases with preceding infection. The most common infection was upper respiratory tract infection (9 cases), followed by simple fever (5 cases). The most common initial neurological symptoms were lethargy or disturbance of consciousness (8 cases), followed by limb weakness (5 cases). There were 6 cases of simple BBE and 13 cases of BBE overlapping Guillain-Barré syndrome (GBS). Besides the characteristic triad of altered mental status, ataxia, and ophthalmoplegia, there were other symptoms including convulsion (5 cases), diplopia (3 cases), nystagmus (7 cases), facial muscular weakness (7 cases),bulbar palsy (13 cases) and autonomic nerve symptoms (9 cases). Hypo or areflexia was seen in 16 cases. Positive Babinski's signs were seen in 8 cases. Hyponatremia was present in 10 cases in whom 4 showed severe hyponatremia. Albumin-cytological dissociation of cerebrospinal fluid was seen in 10 cases. The autoimmune antibodies were examined in all 19 patients. Anti-ganglioside antibodies including anti-GM1 IgG antibody was positive in 2 patients and one of whom was also found with positive anti-GD1b IgG antibody. Anti-GQ1b IgG antibody was present in 2 patients. Electromyography was performed in 14 cases and 8 cases, who were all BBE overlapping GBS, showed neurological damage. A total of 16 cases were monitored by video electroencephalography and 8 cases showed slow waves of background. In addition to, interictal focal discharge was detected in 2 cases. T2 fluid-attenuated inversion recovery (FLAIR) sequence abnormal signals were detected in 3 of 18 cases performed brain magnetic resonance imaging (MRI), and lesions involved with brainstem, basal ganglia, thalamus, cerebellum, corpus callosum and cerebral cortex. Lesions involved cervical and thoracic spinal cord were found in 1 out of 11 cases for whom spinal cord MRI was performed. All of the 4 cases who underwent enhanced MRI of spinal had partial nerve roots enhancement. All of the 19 patients received 1 to 2 courses of intravenous immunoglobulin therapy, and 2 cases also received plasma exchange. Fifteen cases received steroid therapy. The following-up period ranged from 3 months to 2.5 years. Two cases were lost to follow-up. Twelve cases achieved a full recovery within 3 months. Three cases recovered within 6 months. One case still had slight limb weakness and ataxia after 1 year and 8 months of follow-up, and another case had left autonomic nerve symptoms in the follow-up of 2 years and 3 months. Both of them were BBE overlapping GBS. Conclusions: Children's BBE is similar to that in adults, and is frequently found overlapped with GBS. Furthermore, it is sometimes accompanied by central nervous system demyelination disease. The antiganglioside antibodies are not often detectable. Immunoglobulin therapy could usually achieve good response. The prognosis of simple BBE is good in most situations. For BBE overlapping GBS, the more severe the limb weakness during the peak of disease is, the slower the recovery would be.


Assuntos
Tronco Encefálico/patologia , Encefalite/diagnóstico , Síndrome de Guillain-Barré , Adulto , Criança , Feminino , Gangliosídeos , Humanos , Masculino , Estudos Retrospectivos
12.
Brain Dev ; 41(9): 826, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31109765
13.
Zhonghua Yi Xue Za Zhi ; 99(18): 1401-1405, 2019 May 14.
Artigo em Chinês | MEDLINE | ID: mdl-31137128

RESUMO

Objective: To explore the classification, clinical features, the short-term efficacy of intravenous immunoglobulin (IVIg) for Guillain-Barré syndrome(GBS) and look for predictors of acute motor axonal neuropathy (AMAN) during pregnancy. Methods: The clinical data of 45 hospitalized pregnant patients with GBS recruited from October 2008 to October 2017 at the Tianjin Medical University general hospital, Handan City First Hospital and Nankai University Affiliated Tianjin Fourth Central Hospital, were collected and analyzed retrospectively, and patients were divided into the acute inflammatory demyelinating polyneuropathies (AIDP) group and the AMAN group. The clinical features and efficacy of IVIg were compared between the two groups. Logistic regression analysis was used to analyze the predictors of AMAN. Results: There were 25 cases in the AIDP group and 20 cases in the AMAN group. AIDP usually started with distal limb weakness (P=0.001), and AMAN often started with limb weakness (P=0.001) and mostly accompanied by dyspnea (P=0.042). AIDP was often associated with paresthesia (P=0.001) and autonomic dysfunction (P=0.007). The response days of active treatment in the AIDP group and the AMAN group were (1.6±0.5)d and (2.3±0.8)d (P=0.022), the improvement days were (3.6±0.8)d and (5.9±1.0)d (P=0.000), the basic cure days were (7.7±1.3)d and (9.0±0.8)d (P=0.002), the cure days were (12.3±1.1)d and (12.8±0.9)d (P=0.148). Multivariate Logistic regression analysis revealed that preceding diarrhea (OR=13.750; 95% CI 1.386-136.387), limb weakness(OR=12.000;95% CI 2.359-61.048) and limb weakness with dyspnea (OR=10.000; 95% CI 1.048-95.457) were significantly associated with the AMAN-type GBS. Conclusions: AIDP and AMAN are the main types of pregnancy complicating GBS. Most patients present with a single and benign course of disease. IVIg is generally safe and effective. Preceding diarrhea, limb weakness and limb weakness with dyspnea are the predictors of AMAN-type pregnancy complicating GBS.


Assuntos
Síndrome de Guillain-Barré , Complicações na Gravidez , Diarreia , Feminino , Humanos , Imunoglobulinas Intravenosas , Gravidez , Estudos Retrospectivos
14.
Zhonghua Yi Xue Za Zhi ; 99(17): 1332-1335, 2019 May 07.
Artigo em Chinês | MEDLINE | ID: mdl-31091582

RESUMO

Objective: To investigate the factors of first misdiagnosis, treatment and prognosis of acute pregnancy complicating with Guillain-Barré syndrome (GBS) in order to improve the first diagnosed rate. Methods: A total of 45 acute pregnancy complicating with GBS patients were retrospectively analyzed recruited from January 2009 to October 2017 at the Tianjin Fourth Central Hospital.Patients were divided into the first diagnosis group and the first misdiagnosis group, and GBS clinical types were classified into classic and variant types to analyze the misdiagnosis factors of the first diagnosis. All patients received intravenous immunoglobulin (IVIG) treatment, and the therapeutic effect and prognosis were compared and analyzed. Results: There were 20 cases in the first diagnosis group, 25 cases in the first misdiagnosis group, 35 cases in the typical GBS group, and 10 cases in the variant GBS group.There was no statistically significant difference in the baseline data of the patients(P>0.05).The misdiagnosis factors are divided into four categories: physician factors, patient factors, disease itself factors and laboratory factors. Variant GBS is more likely to lead to misdiagnosis in the first diagnosis than typical GBS. The therapeutic effect of the first diagnosis group was better than that of the first misdiagnosis group(P<0.05).Three patients died in the first misdiagnosis group, and the rest of the pregnant patients gave birth normally, and the babies were born without congenital malformation. Conclusions: Pregnancy is one of the inducing factors of GBS. Early diagnosis and correct treatment can improve maternal and infant clinical outcomes.


Assuntos
Síndrome de Guillain-Barré , Erros de Diagnóstico , Feminino , Humanos , Imunoglobulinas Intravenosas , Gravidez , Complicações na Gravidez , Prognóstico , Estudos Retrospectivos
15.
Neurologist ; 24(3): 75-83, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31045716

RESUMO

OBJECTIVES: The objective of this study was to assess risk of neurological toxicities following the use of different immune checkpoint inhibitor (ICI) regimens in solid tumors. METHODS: Pubmed, Embase, and ClinicalTrials.gov databases were searched for publications, and data were analyzed using Review Manager 5.3 software to compare the risk of immune-related and nonspecific neurological complications potentially triggered by ICIs to controls. RESULTS: In total 23 randomized clinical trials comprising 11,687 patients were included in this meta-analysis. Patients with PD-L1 (OR, 0.29; 95% confidence interval [CI], 0.18-0.48; P<0.01) or programmed cell-death protein 1 (PD-1) inhibitor (OR, 0.21; 95% CI, 0.14-0.31; P<0.01) were less likely to develop any-grade peripheral neuropathy than chemotherapy, while the risk of grade 3-5 was also smaller for PD-1 inhibitor (OR, 0.16; 95% CI, 0.05-0.54; P=0.003). Combination therapy with CTLA4 and PD-1 inhibitor did not significantly increase the risk of any-grade (OR, 0.83; 95% CI, 0.21-3.32; P>0.05) or grade 3-5 (OR, 1.4; 95% CI, 0.2-9.61; P>0.05) peripheral neuropathy compared to monotherapy with CTLA4 or PD-1 inhibitor. However, difference in risk of immune-related adverse events (irAEs) involving central nervous system did not reach statistical significance in patients with different ICI regimens compared those under chemotherapy. Additionally, risk of experiencing paresthesia was in line with that of peripheral neuropathy (OR, 0.42; 95% CI, 0.28-0.62; P<0.01). CONCLUSIONS: This meta-analysis shows that PD-L1/PD-1 and CTLA4 inhibitor have decreased risk of peripheral neuropathy compared to chemotherapy, while combination therapy with CTLA4 and PD-1 inhibitor have no difference in neurological toxicities compared to monotherapy with CTLA4 or PD-1 inhibitor.


Assuntos
Antineoplásicos Imunológicos/efeitos adversos , Neoplasias/tratamento farmacológico , Síndromes Neurotóxicas/epidemiologia , Antígeno B7-H1/antagonistas & inibidores , Antígeno CTLA-4/antagonistas & inibidores , Síndrome de Guillain-Barré/induzido quimicamente , Humanos , Miastenia Gravis/induzido quimicamente , Miosite/induzido quimicamente , Doenças Neuromusculares/induzido quimicamente , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Receptor de Morte Celular Programada 1/antagonistas & inibidores , Ensaios Clínicos Controlados Aleatórios como Assunto , Fatores de Risco
16.
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
17.
J Neuroinflammation ; 16(1): 73, 2019 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-30953561

RESUMO

BACKGROUND: Autoantibodies against the paranodal protein contactin-1 have recently been described in patients with severe acute-onset autoimmune neuropathies and mainly belong to the IgG4 subclass that does not activate complement. IgG3 anti-contactin-1 autoantibodies are rare, but have been detected during the acute onset of disease in some cases. There is evidence that anti-contactin-1 prevents adhesive interaction, and chronic exposure to anti-contactin-1 IgG4 leads to structural changes at the nodes accompanied by neuropathic symptoms. However, the pathomechanism of acute onset of disease and the pathogenic role of IgG3 anti-contactin-1 is largely unknown. METHODS: In the present study, we aimed to model acute autoantibody exposure by intraneural injection of IgG of patients with anti-contacin-1 autoantibodies to Lewis rats. Patient IgG obtained during acute onset of disease (IgG3 predominant) and IgG from the chronic phase of disease (IgG4 predominant) were studied in comparison. RESULTS: Conduction blocks were measured in rats injected with the "acute" IgG more often than after injection of "chronic" IgG (83.3% versus 35%) and proved to be reversible within a week after injection. Impaired nerve conduction was accompanied by motor deficits in rats after injection of the "acute" IgG but only minor structural changes of the nodes. Paranodal complement deposition was detected after injection of the "acute IgG". We did not detect any inflammatory infiltrates, arguing against an inflammatory cascade as cause of damage to the nerve. We also did not observe dispersion of paranodal proteins or sodium channels to the juxtaparanodes as seen in patients after chronic exposure to anti-contactin-1. CONCLUSIONS: Our data suggest that anti-contactin-1 IgG3 induces an acute conduction block that is most probably mediated by autoantibody binding and subsequent complement deposition and may account for acute onset of disease in these patients. This supports the notion of anti-contactin-1-associated neuropathy as a paranodopathy with the nodes of Ranvier as the site of pathogenesis.


Assuntos
Contactina 1/imunologia , Síndrome de Guillain-Barré/complicações , Imunização Passiva/métodos , Imunoglobulina G/farmacologia , Transtornos Motores/fisiopatologia , Transtornos Motores/cirurgia , Animais , Moléculas de Adesão Celular Neuronais/metabolismo , Complemento C1q/metabolismo , Citocinas/metabolismo , Modelos Animais de Doenças , Feminino , Síndrome de Guillain-Barré/etiologia , Humanos , Transtornos Motores/induzido quimicamente , Condução Nervosa/efeitos dos fármacos , Neurite Óptica/sangue , Neurite Óptica/imunologia , Nós Neurofibrosos/efeitos dos fármacos , Nós Neurofibrosos/metabolismo , Ratos , Ratos Endogâmicos Lew , Tempo de Reação/efeitos dos fármacos , Recuperação de Função Fisiológica/efeitos dos fármacos , Teste de Desempenho do Rota-Rod , Estatísticas não Paramétricas
18.
BMJ Case Rep ; 12(3)2019 Mar 31.
Artigo em Inglês | MEDLINE | ID: mdl-30936342

RESUMO

Ophthalmoplegia, ataxia and areflexia characterise the clinical triad of Miller-Fisher Syndrome (MFS). When the disease presents acutely, it can mimic posterior circulation stroke. We describe a case of an adult patient presenting with sudden dizziness, diplopia, vomiting, and loss of balance. She was initially managed as a case of a brainstem stroke, but the progression of craniopathies without deterioration in sensorium coupled with areflexia clinched the diagnosis of MFS two days into her admission. On the third day, her MFS progressed rapidly to acute motor and sensory axonal neuropathy (AMSAN) variant of Guillain-Barre Syndrome, a rare occurrence in patients with MFS, with only four reported cases including our own. Among the four cases, ours is the only one still non-ambulatory eight months after the initial onset of symptoms. The case highlights the importance of early recognition of MFS in patients with ophthalmoplegia and ataxia despite initially normal reflexes.


Assuntos
Síndrome de Guillain-Barré/diagnóstico , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Síndrome de Miller Fisher/diagnóstico , Recuperação de Função Fisiológica/fisiologia , Diagnóstico Diferencial , Diplopia/etiologia , Progressão da Doença , Tontura/etiologia , Feminino , Síndrome de Guillain-Barré/tratamento farmacológico , Síndrome de Guillain-Barré/imunologia , Síndrome de Guillain-Barré/fisiopatologia , Humanos , Imagem por Ressonância Magnética , Pessoa de Meia-Idade , Síndrome de Miller Fisher/tratamento farmacológico , Síndrome de Miller Fisher/imunologia , Síndrome de Miller Fisher/fisiopatologia , Exame Neurológico , Equilíbrio Postural , Recuperação de Função Fisiológica/imunologia , Fatores de Tempo , Resultado do Tratamento , Vômito/etiologia
19.
Nihon Shokakibyo Gakkai Zasshi ; 116(4): 324-329, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-30971669

RESUMO

We herein report a case involving a 23-year-old male patient with active Crohn's disease complicated by Guillain-Barrè syndrome during ustekinumab therapy. At age 11, the patient developed an anal fistula and was found to have multiple aphthae on the rectosigmoid colon, for which he was diagnosed with Crohn's disease. At age 12, he underwent gastrojejunal anastomosis for pyrolic stenosis. At age 20, a longitudinal ulcer was found on the ascending colon, and at age 21, aphthae were found on the stomach and efferent jejunum. At age 22, adalimumab was started, but the patient noted abdominal pain and diarrhea 4 months later. Hence, adalimumab was switched to ustekinumab (2017 June). Though ustekinumab was effective, the patient noted anorexia, weakness, and bilateral lower extremity numbness 1 year later (2018 June) and was admitted to the hospital. He was then diagnosed with Guillain-Barrè syndrome after spinal tap, neurological, and hematological examinations. Immunoglobulin therapy was provided but was less effective. The patient has since been receiving physical therapy. This has been the first report regarding Guillain-Barrè syndrome as a complication during ustekinumab therapy.


Assuntos
Doença de Crohn/complicações , Fármacos Dermatológicos/uso terapêutico , Síndrome de Guillain-Barré/tratamento farmacológico , Ustekinumab/uso terapêutico , Dor Abdominal , Adalimumab , Adulto , Síndrome de Guillain-Barré/complicações , Humanos , Masculino , Adulto Jovem
20.
BMJ Case Rep ; 12(4)2019 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-30992282

RESUMO

A 56-year-old woman with a medical history of hypertension presented to our hospital with back pain, abdominal pain, vomiting and elevated blood pressure. The laboratory parameters including evaluation for secondary hypertension were within normal ranges at the time of presentation. During her hospitalisation, fluctuations in her blood pressure and pulse were observed which were attributed to autonomic disturbances, the cause of which was unknown. On the seventh day after presentation to the hospital, the patient developed focal seizures and slurred speech which was believed to be secondary to hyponatraemia detected at that time. Hyponatraemia improved with hypertonic saline and she experienced no further seizures. On the eighth day of her admission, she developed acute flaccid paralysis of all her limbs and respiratory distress. We concluded this to be secondary to Guillain-Barre syndrome (GBS). She responded to plasmapheresis.The presence of dysautonomia and hyponatraemia before the onset of paralysis makes this a rare presentation of GBS.


Assuntos
Síndrome de Guillain-Barré/diagnóstico , Hiponatremia/diagnóstico , Dor Lombar/etiologia , Disautonomias Primárias/diagnóstico , Pressão Sanguínea , Feminino , Síndrome de Guillain-Barré/complicações , Frequência Cardíaca , Humanos , Hiponatremia/sangue , Hiponatremia/complicações , Vértebras Lombares/diagnóstico por imagem , Pessoa de Meia-Idade , Paralisia/etiologia , Disautonomias Primárias/complicações , Convulsões/etiologia
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