RESUMO
BACKGROUND: Guillain-Barré syndrome (GBS) is an autoimmune-mediated peripheral neuropathy characterized by symmetric weakness. Asymmetric weakness in GBS is uncommon and may be easily confused with other differential diagnoses. We herein present three cases of asymmetric GBS and review the literature on this atypical subtype of GBS in order to describe the characteristics of asymmetric GBS and to provide experience for clinicians. CASE SUMMARY: Different from patients in the previous reports, our patients showed persistent asymmetric limb weakness from the onset to recovery phase. All three patients were serologically positive for antecedent infections. Two of the three cases had IgG antibodies against ganglioside GM1. Two patients received immunotherapy including intravenous immunoglobulin and plasma exchange, while one patient received only supportive treatment. Autoantibodies against gangliosides, asymmetry of congenital development of blood-nerve barrier and limb use may contribute to the development of asymmetric limb weakness in GBS. CONCLUSION: Asymmetric GBS may be a rare clinical variant and should be considered when a patient develops acute and progressive asymmetric limb weakness. The differences in clinical features and prognosis between asymmetric GBS and classic GBS deserve further investigation in a large study.
RESUMO
Guillain-Barré syndrome (GBS) is a post-infectious autoimmune disease. Dendritic cells (DCs) can recognize the pathogen and modulate the host immune response. Exploring the role of DCs in GBS will help our understanding of the disease development. In this study, we aimed to analyze plasmacytoid and conventional DCs in peripheral blood of patients with GBS at different stages of the disease: acute phase as well as early and late recovery phases. There was a significant increase of plasmacytoid DCs in the acute phase (p=0.03 vs healthy donors). There was a positive correlation between percentage of plasmacytoid DCs and the clinical severity of patients with GBS (r=0.61, p<0.001). Quantitative polymerase chain reaction and flow cytometry confirmed the aberrant plasmacytoid DCs in GBS. Thus, plasmacytoid DCs may participate in the development of GBS.
Assuntos
Células Dendríticas/imunologia , Síndrome de Guillain-Barré/imunologia , Doença Aguda , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Antígenos CD/biossíntese , Antígenos CD/genética , Autoanticorpos/sangue , Autoanticorpos/imunologia , Autoantígenos/imunologia , Convalescença , Feminino , Citometria de Fluxo , Gangliosídeos/imunologia , Síndrome de Guillain-Barré/sangue , Síndrome de Guillain-Barré/genética , Síndrome de Guillain-Barré/fisiopatologia , Síndrome de Guillain-Barré/terapia , Humanos , Imunoglobulina G/sangue , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Pessoa de Meia-Idade , Condução Nervosa , Troca Plasmática , Índice de Gravidade de Doença , Receptor 7 Toll-Like/biossíntese , Receptor 7 Toll-Like/genética , Receptor Toll-Like 9/biossíntese , Receptor Toll-Like 9/genética , Regulação para Cima , Adulto JovemRESUMO
Toll-like receptor (TLR) 9, recognizing different ligands, confers distinct features of plasmacytoid dendritic cells (pDCs). Our previous study demonstrated a role for TLR9 in the mechanism of experimental autoimmune neuritis (EAN). In this study, we explored whether suppressive oligodeoxynucleotides (sODN) could induce tolerogenic pDCs via TLR9 and thus promote the recovery of EAN. Effects of different TLR9 ligands, CpG ODN and sODN on P0 180-199 peptide-stimulated pDCs were measured by detecting the expression of co-stimulatory molecules, indoleamine 2,3-dioxygenase (IDO), secretion of Th1- and Th2-type cytokines and the TLR9 signaling pathway. CpG ODN- or sODN-treated pDCs were intravenously injected into the EAN mice and their effects were compared. Our data showed that P0180-199 peptides significantly promoted mRNA expression of co-stimulatory molecules (CD40, CD80 and CD86) in pDCs and induced secretion of Th1-type cytokines. Treatment of CpG ODN aggravated the effects of P0 180-199 peptides on pDCs; however, sODN had the opposite effects and significantly upregulated the IDO expression in pDCs. Further analysis showed that MYD88 is necessary for sODN to modulate the TLR9/NF-κB signaling in pDCs. Finally, the sODN-treated pDCs significantly promoted recovery of the EAN mice. Taken together, sODN could induce tolerogenic pDCs and thus ameliorate the EAN.
Assuntos
Células Dendríticas/imunologia , Tolerância Imunológica/imunologia , Neurite Autoimune Experimental/imunologia , Oligodesoxirribonucleotídeos/farmacologia , Peptídeos/farmacologia , Animais , Antígeno B7-1/genética , Antígeno B7-2/genética , Antígenos CD40/genética , Células Cultivadas , Ilhas de CpG/genética , Citocinas/biossíntese , Citocinas/metabolismo , Indolamina-Pirrol 2,3,-Dioxigenase/biossíntese , Indolamina-Pirrol 2,3,-Dioxigenase/genética , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Fator 88 de Diferenciação Mieloide/metabolismo , Neurite Autoimune Experimental/terapia , RNA Mensageiro/genética , Células Th1/imunologia , Células Th2/imunologia , Receptor Toll-Like 9/imunologia , Fator de Transcrição RelA/metabolismoRESUMO
MicroRNA-155 (miR155) has been demonstrated as a central regulator of immune responses induced by inflammatory mediators. Previous studies suggest that miR155 may play adverse effects in various diseases. We hereby explored the roles of miR155 in the pathogenesis of Guillain-Barré syndrome (GBS). Peripheral blood mononuclear cells (PBMCs) were separated from GBS patients and healthy controls. Expression of miR155 in PBMCs was detected by quantitative PCR. An inhibitor of miR155 was transfected into the cultured PBMCs and the GBS-related cytokines were detected. Significantly, our study demonstrated that miR155 was downregulated in PBMCs from GBS patients and silencing of miR155 profoundly promoted the production of Th1-type cytokines in vitro. Our data effectively demonstrate a protective role of miR155 in GBS, which suggests that miR155 may be a promising target for the therapy of the disease.
