Intrathecal injection of P/Q type voltage-gated calcium channel antibodies from paraneoplastic cerebellar degeneration cause ataxia in mice

J Neuroimmunol. 2013 Aug 15;261(1-2):53-9. doi: 10.1016/j.jneuroim.2013.05.003. Epub 2013 May 31.

Abstract

The role of antibodies against the P/Q type voltage-gated calcium channels (VGCC-ab) in the pathogenesis of paraneoplastic cerebellar degeneration (PCD) and lung cancer is unclear. We evaluated in mice the effect of intrathecal injection of IgG purified from serum of a patient with both PCD and Lambert-Eaton myasthenic syndrome (LEMS), and from another patient with isolated LEMS. Mice injected with PCD/LEMS IgG developed marked, reversible ataxia compared with those injected with LEMS or control IgG. These findings suggest that P/Q-type VGCC-ab may play a role in the pathogenesis of ataxia in patients with PCD and SCLC.

Keywords: Intrathecal injection; Lambert–Eaton Myasthenic syndrome; Mouse model; P/Q-type voltage-gated calcium channel antibodies; Paraneoplastic cerebellar degeneration; Small-cell lung cancer.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Autoantibodies / toxicity*
  • Calcium Channels, P-Type / blood
  • Calcium Channels, P-Type / immunology*
  • Calcium Channels, Q-Type / immunology*
  • Cerebellar Ataxia / chemically induced
  • Cerebellar Ataxia / immunology*
  • HEK293 Cells
  • Humans
  • Injections, Spinal
  • Mice
  • Paraneoplastic Cerebellar Degeneration / immunology*
  • Rats

Substances

  • Autoantibodies
  • Calcium Channels, P-Type
  • Calcium Channels, Q-Type