RESUMO
Anti-SOX1 antibodies have been described to be positive in patients with paraneoplastic Lambert-Eaton myasthenic syndrome and, in a lower amount, in patients with anti-Hu positive paraneoplastic neurological syndromes, and with SCLC alone, respectively. We found 5/32 patients with paraneoplastic neuropathy and, surprisingly, 4/22 patients with neuropathy of unknown origin positive for anti-SOX1 antibodies, whereas no patient with inflammatory neuropathy and no healthy controls showed any reactivity (p=0.007). All patients with neuropathy of unknown origin where followed up for four years without diagnosis of a tumour so far. Anti-SOX1 antibodies are associated with paraneoplastic neuropathies and may define another group of non-paraneoplastic, immune-mediated neuropathies.
Assuntos
Autoanticorpos/metabolismo , Síndrome Miastênica de Lambert-Eaton/imunologia , Polineuropatia Paraneoplásica/imunologia , Fatores de Transcrição SOXB1/imunologia , Idoso , Idoso de 80 Anos ou mais , Animais , Linhagem Celular Transformada , Proteínas ELAV/imunologia , Feminino , Humanos , Síndrome Miastênica de Lambert-Eaton/metabolismo , Masculino , Camundongos , Pessoa de Meia-Idade , Polineuropatia Paraneoplásica/metabolismo , Síndromes Paraneoplásicas do Sistema Nervoso/classificação , Síndromes Paraneoplásicas do Sistema Nervoso/imunologia , Síndromes Paraneoplásicas do Sistema Nervoso/metabolismo , Transfecção/métodosRESUMO
Paraneoplastic neurological syndromes are clinically heterogeneous manifestations of cancer, but are not caused by the tumor or its metastases. Because autoantibodies reacting with tumor and nervous system tissue have been described, an autoimmune pathogenesis is suspected. Most autoantibodies are directed against neuronal proteins. Here, we describe the impact of antiglial autoantibodies in paraneoplastic neurological syndromes. Anti-CRMP5 and antiglial nuclear antibody both can be associated with different paraneoplastic neurological syndromes and tumors.
Assuntos
Antígenos/imunologia , Autoanticorpos/imunologia , Neuroglia/imunologia , Síndromes Paraneoplásicas do Sistema Nervoso/imunologia , Animais , Autoimunidade/imunologia , Núcleo Celular/imunologia , Humanos , Síndromes Paraneoplásicas do Sistema Nervoso/patologiaRESUMO
Opsoclonus-myoclonus syndrome (OMS) in children is a rare disorder including a severe eye movement disturbance, myoclonia, ataxia and often developmental retardation. Both OMS forms, idiopathic or neuroblastoma-associated (paraneoplastic), have been suspected to be autoimmune. Recently, autoantibodies have been found in OMS sera. We here show that autoantibodies in OMS, both intracellular and surface binding, belong mainly to the IgG3 subclass, although the total serum IgG3 level is normal. These results support the autoimmune hypothesis and point to a protein autoantigen as antigenic target.
