Cytotoxic T lymphocyte-mediated cell death in paraneoplastic sensory neuronopathy with anti-Hu antibody

J Neurol Sci. 1999 Mar 1;163(2):159-62. doi: 10.1016/s0022-510x(99)00029-5.


Paraneoplastic sensory neuronopathy (PSN) has been shown to harbor characteristic anti-neuronal autoantibody 'anti-Hu' in their sera and cerebrospinal fluid. Creation of animal models exhibiting clinical or pathological features seen in PSN by means of passive transfer of anti-Hu positive IgG has not been achieved. Although, anti-Hu antibody was shown to induce neuronal cell lysis in vitro, this result has not been reproduced so far. Since prominent T cell infiltration are seen in the central nervous system and posterior spinal ganglion of the patients with anti-Hu syndrome, we studied cytotoxic T cell (CTL) activity in peripheral mononuclear cells from a patient with PSN harboring anti-Hu antibody. The activated CD8+ T cells from the patient's venous blood were shown to lyse her own fibroblasts which were incubated with interferon-gamma to induce HLA class I molecules on their surface and the recombinant HuD protein was injected into the cells by microinjector. This is the first report showing the existence of CTL in a patient with PSN.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autoantibodies / blood
  • Autoantibodies / cerebrospinal fluid*
  • Cytotoxicity, Immunologic*
  • ELAV Proteins
  • ELAV-Like Protein 4
  • Humans
  • Nerve Tissue Proteins*
  • Paraneoplastic Syndromes / blood
  • Paraneoplastic Syndromes / cerebrospinal fluid
  • Paraneoplastic Syndromes / immunology*
  • Paraneoplastic Syndromes / physiopathology
  • RNA-Binding Proteins / immunology*
  • Recombinant Proteins / immunology
  • Sensation Disorders / etiology
  • Sensation Disorders / immunology*
  • T-Lymphocytes, Cytotoxic / immunology*


  • Autoantibodies
  • ELAV Proteins
  • ELAV-Like Protein 4
  • ELAVL4 protein, human
  • Nerve Tissue Proteins
  • RNA-Binding Proteins
  • Recombinant Proteins