Lambert-Eaton myasthenic syndrome: I. Early morphological effects of IgG on the presynaptic membrane active zones

Ann Neurol. 1987 Aug;22(2):193-9. doi: 10.1002/ana.410220203.

Abstract

In the freeze-fractured presynaptic membrane of the motor end-plate, the active zones consist of two parallel arrays and each array contains 10- to 12-nm particles arranged in two rows. In the Lambert-Eaton myasthenic syndrome (LEMS) and in mice treated with 10 mg/day of LEMS IgG, administered intraperitoneally for several weeks, there was a paucity and disorganization of the active zones, and clusters of 10- to 12-nm particles appeared. To further define the changes in the active zones, mice were studied that had been treated over 2 days with 104 to 180 mg of IgG. Treatment transferred the physiological defect of LEMS. Control mice received normal human IgG or no IgG. The spacing and density (number/unit area) of active-zone particles were evaluated in presynaptic membrane P-faces using computer-assisted stereometry. In the normal active zone, the distance between particles in a given row and between adjacent rows of an array was less than, but the distance between the two arrays was greater than, the distance between the two antigen-binding sites on human IgG. In mice treated with LEMS IgG, the initial alteration in the active zone was a decrease in the distance between particles in a given row and between adjacent rows of an array; the distance between the two arrays remained unaltered. In more affected active zones, the parallel orientation of the rows was disturbed and the arrays became clusters. There was a significantly reduced density of active zones and of large-membrane particles associated with all active zones and clusters.(ABSTRACT TRUNCATED AT 250 WORDS)

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Freeze Fracturing
  • Humans
  • Immunoglobulin G / pharmacology*
  • Mice
  • Mice, Inbred Strains
  • Motor Endplate / drug effects
  • Motor Endplate / ultrastructure
  • Muscular Diseases / blood
  • Muscular Diseases / pathology*
  • Synaptic Membranes / drug effects*
  • Synaptic Membranes / ultrastructure
  • Syndrome

Substances

  • Immunoglobulin G