Immunoglobulin G from a patient with Miller-Fisher syndrome rapidly and reversibly depresses evoked quantal release at the neuromuscular junction of mice

Neurosci Lett. 1995 Dec 8;201(2):163-6. doi: 10.1016/0304-3940(95)12155-2.

Abstract

A neuromuscular blocking factor has been described in the serum of patients with Miller-Fisher syndrome (MFS). We here examined the effect of immunoglobulins (Ig) on neuromuscular transmission in mice recording quantal endplate currents by means of a perfused macro-patch-clamp electrode. Ig and IgM- and IgG-fractions from an anti-GQ1b-positive patient with typical MFS were highly purified. After application of MFS-IgG, quantal release decreased 1000-fold within 2 min. Returning to control solution the average release came back to the baseline level within 4 min. In contrast, control-IgG and MFS-IgM did not cause any blocking effect. The very fast and fully reversible presynaptic blockade of release caused by the highly purified IgG-fraction may be one factor producing muscle weakness in MFS.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Demyelinating Diseases / immunology*
  • Diaphragm / drug effects
  • Diaphragm / physiology
  • Humans
  • Immunoglobulin G / immunology*
  • Immunoglobulin M / immunology
  • In Vitro Techniques
  • Mice
  • Mice, Inbred BALB C
  • Motor Endplate / drug effects
  • Neuromuscular Junction / physiology*
  • Peripheral Nervous System Diseases / immunology*
  • Syndrome
  • Tetrodotoxin / pharmacology

Substances

  • Immunoglobulin G
  • Immunoglobulin M
  • Tetrodotoxin