title: Peripheral neuropathies associated with antibodies directed to intracellular neural antigens.
abstract: Antibodies directed to intracellular neural antigens have been mainly described in paraneoplastic peripheral neuropathies and mostly includes anti-Hu and anti-CV2/CRMP5 antibodies. These antibodies occur with different patterns of neuropathy. With anti-Hu antibody, the most frequent manifestation is sensory neuronopathy with frequent autonomic involvement. With anti-CV2/CRMP5 the neuropathy is more frequently sensory and motor with an axonal or mixed demyelinating and axonal electrophysiological pattern. The clinical pattern of these neuropathies is in keeping with the cellular distribution of HuD and CRMP5 in the peripheral nervous system. Although present in high titer, these antibodies are probably not directly responsible for the neuropathy. Pathological and experimental studies indicate that cytotoxic T-cells are probably the main effectors of the immune response. These disorders contrast with those in which antibodies recognize a cell surface antigen and are probably responsible for the disease. The neuronal cell death and axonal degeneration which result from T-cell mediated immunity explains why treating these disorders remains challenging.
authors.affiliation: Service de neurologie, CHU de Saint-Étienne, 42055 Saint-Étienne cedex, France. Electronic address: j.christophe.antoine@chu-st-etienne.fr.
authors.forename: J-C
authors.initials: JC
authors.lastname: Antoine
mesh_terms: D001323:Autoantibodies / Q000276:immunology*; D051959:ELAV Proteins / Q000276:immunology; D006801:Humans; D007111:Immunity, Cellular / Q000276:immunology; D007425:Intracellular Membranes / Q000276:immunology; D009474:Neurons / Q000276:immunology; D010523:Peripheral Nervous System Diseases / Q000188:drug therapy / Q000276:immunology*
chemical_list: D001323:Autoantibodies; D051959:ELAV Proteins
keywords: Anti-CRMP5 antibodies; Anti-CV2 antibodies; Anti-Hu antibodies; Anticorps anti-CRMP5; Anticorps anti-CV2; Anticorps anti-Hu; Cellules T; Immunologie; Immunology; Neuronopathie sensitive; Neuropathie periphérique; Paraneoplastic neurological syndromes; Peripheral neuropathy; Sensory neuronopathy; Syndromes neurologiques paranéoplasiques; T-cells