Selected IgG rapidly induces Lambert-Eaton myasthenic syndrome in mice: Complement independence and EMG abnormalities

E. H. Lambert, Vanda A Lennon

Research output: Contribution to journalArticle

48 Citations (Scopus)

Abstract

Antibodies in individual patients with Lambert-Eaton myasthenic syndrome (LES) differ in their reactivity with mouse motor nerve terminals. Of 26 LES patients' sera injected a single time into mice, 3 caused a highly significant reduction in stimulus-dependent quantal release (m) of acetylcholine (ACh) (to 6, 33, and 42 quanta per impulse at 1 Hz, respectively; mean for 10 control sera, 100 quanta at 1 Hz). The most potent serum (LES-A) was fully effective in mice deficient in complement component C5 and in mice depleted of complement components C3→C9 by cobra venom factor. A single i.v. injection of serum reduced m in direct proportion to log dose. Responses to K+ depolarization and increasing concentrations of Ca2+ were like those observed in human LES. With LES-A serum, or its IgG, m was reduced near maximally in 1 day and plateaued in 3-4 days. Recovery began after day 8; m was in the normal range by day 20-30. Electromyographic (EMG) abnormalities were not seen until m fell below 40 quanta per impulse at 1 Hz. Below 10 quanta, clinical signs of weakness appeared, and the EMG abnormalities were those classically associated with LES: a marked reduction of compound muscle action potential to a single nerve stimulus in rested muscle, a further decrement during stimulation at slow rates, but marked facilitation during rapid repetitive stimulation.

Original languageEnglish (US)
Pages (from-to)1133-1145
Number of pages13
JournalMuscle and Nerve
Volume11
Issue number11
StatePublished - 1988

Fingerprint

Lambert-Eaton Myasthenic Syndrome
Immunoglobulin G
Serum
Complement C5
Muscles
Action Potentials
Acetylcholine
Reference Values
Injections
Antibodies

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Selected IgG rapidly induces Lambert-Eaton myasthenic syndrome in mice : Complement independence and EMG abnormalities. / Lambert, E. H.; Lennon, Vanda A.

In: Muscle and Nerve, Vol. 11, No. 11, 1988, p. 1133-1145.

Research output: Contribution to journalArticle

@article{20eae5b0daa240859f9df43c61286742,
title = "Selected IgG rapidly induces Lambert-Eaton myasthenic syndrome in mice: Complement independence and EMG abnormalities",
abstract = "Antibodies in individual patients with Lambert-Eaton myasthenic syndrome (LES) differ in their reactivity with mouse motor nerve terminals. Of 26 LES patients' sera injected a single time into mice, 3 caused a highly significant reduction in stimulus-dependent quantal release (m) of acetylcholine (ACh) (to 6, 33, and 42 quanta per impulse at 1 Hz, respectively; mean for 10 control sera, 100 quanta at 1 Hz). The most potent serum (LES-A) was fully effective in mice deficient in complement component C5 and in mice depleted of complement components C3→C9 by cobra venom factor. A single i.v. injection of serum reduced m in direct proportion to log dose. Responses to K+ depolarization and increasing concentrations of Ca2+ were like those observed in human LES. With LES-A serum, or its IgG, m was reduced near maximally in 1 day and plateaued in 3-4 days. Recovery began after day 8; m was in the normal range by day 20-30. Electromyographic (EMG) abnormalities were not seen until m fell below 40 quanta per impulse at 1 Hz. Below 10 quanta, clinical signs of weakness appeared, and the EMG abnormalities were those classically associated with LES: a marked reduction of compound muscle action potential to a single nerve stimulus in rested muscle, a further decrement during stimulation at slow rates, but marked facilitation during rapid repetitive stimulation.",
author = "Lambert, {E. H.} and Lennon, {Vanda A}",
year = "1988",
language = "English (US)",
volume = "11",
pages = "1133--1145",
journal = "Muscle and Nerve",
issn = "0148-639X",
publisher = "John Wiley and Sons Inc.",
number = "11",

}

TY - JOUR

T1 - Selected IgG rapidly induces Lambert-Eaton myasthenic syndrome in mice

T2 - Complement independence and EMG abnormalities

AU - Lambert, E. H.

AU - Lennon, Vanda A

PY - 1988

Y1 - 1988

N2 - Antibodies in individual patients with Lambert-Eaton myasthenic syndrome (LES) differ in their reactivity with mouse motor nerve terminals. Of 26 LES patients' sera injected a single time into mice, 3 caused a highly significant reduction in stimulus-dependent quantal release (m) of acetylcholine (ACh) (to 6, 33, and 42 quanta per impulse at 1 Hz, respectively; mean for 10 control sera, 100 quanta at 1 Hz). The most potent serum (LES-A) was fully effective in mice deficient in complement component C5 and in mice depleted of complement components C3→C9 by cobra venom factor. A single i.v. injection of serum reduced m in direct proportion to log dose. Responses to K+ depolarization and increasing concentrations of Ca2+ were like those observed in human LES. With LES-A serum, or its IgG, m was reduced near maximally in 1 day and plateaued in 3-4 days. Recovery began after day 8; m was in the normal range by day 20-30. Electromyographic (EMG) abnormalities were not seen until m fell below 40 quanta per impulse at 1 Hz. Below 10 quanta, clinical signs of weakness appeared, and the EMG abnormalities were those classically associated with LES: a marked reduction of compound muscle action potential to a single nerve stimulus in rested muscle, a further decrement during stimulation at slow rates, but marked facilitation during rapid repetitive stimulation.

AB - Antibodies in individual patients with Lambert-Eaton myasthenic syndrome (LES) differ in their reactivity with mouse motor nerve terminals. Of 26 LES patients' sera injected a single time into mice, 3 caused a highly significant reduction in stimulus-dependent quantal release (m) of acetylcholine (ACh) (to 6, 33, and 42 quanta per impulse at 1 Hz, respectively; mean for 10 control sera, 100 quanta at 1 Hz). The most potent serum (LES-A) was fully effective in mice deficient in complement component C5 and in mice depleted of complement components C3→C9 by cobra venom factor. A single i.v. injection of serum reduced m in direct proportion to log dose. Responses to K+ depolarization and increasing concentrations of Ca2+ were like those observed in human LES. With LES-A serum, or its IgG, m was reduced near maximally in 1 day and plateaued in 3-4 days. Recovery began after day 8; m was in the normal range by day 20-30. Electromyographic (EMG) abnormalities were not seen until m fell below 40 quanta per impulse at 1 Hz. Below 10 quanta, clinical signs of weakness appeared, and the EMG abnormalities were those classically associated with LES: a marked reduction of compound muscle action potential to a single nerve stimulus in rested muscle, a further decrement during stimulation at slow rates, but marked facilitation during rapid repetitive stimulation.

UR - http://www.scopus.com/inward/record.url?scp=0023812264&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0023812264&partnerID=8YFLogxK

M3 - Article

C2 - 3226429

AN - SCOPUS:0023812264

VL - 11

SP - 1133

EP - 1145

JO - Muscle and Nerve

JF - Muscle and Nerve

SN - 0148-639X

IS - 11

ER -