ABSTRACT
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.
Subject(s)
Autoantibodies/metabolism , Lambert-Eaton Myasthenic Syndrome/immunology , Paraneoplastic Polyneuropathy/immunology , SOXB1 Transcription Factors/immunology , Aged , Aged, 80 and over , Animals , Cell Line, Transformed , ELAV Proteins/immunology , Female , Humans , Lambert-Eaton Myasthenic Syndrome/metabolism , Male , Mice , Middle Aged , Paraneoplastic Polyneuropathy/metabolism , Paraneoplastic Syndromes, Nervous System/classification , Paraneoplastic Syndromes, Nervous System/immunology , Paraneoplastic Syndromes, Nervous System/metabolism , Transfection/methodsABSTRACT
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.
Subject(s)
Antigens/immunology , Autoantibodies/immunology , Neuroglia/immunology , Paraneoplastic Syndromes, Nervous System/immunology , Animals , Autoimmunity/immunology , Cell Nucleus/immunology , Humans , Paraneoplastic Syndromes, Nervous System/pathologyABSTRACT
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.
