Lower motor neuron syndromes defined by patterns of weakness, nerve conduction abnormalities, and high titers of antiglycolipid antibodies

A. Pestronk, Vinay Chaudhry, E. L. Feldman, J. W. Griffin, David Cornblath, E. H. Denys, M. Glasberg, R. W. Kuncl, R. K. Olney, W. C. Yee

Research output: Contribution to journalArticle

Abstract

We studied 74 patients with progressive, asymmetrical lower motor neuron syndromes. Clinical features of these patients, including age, sex, disease duration, patterns of weakness, and reflex changes, were evaluated by review of records. In each patient the clinical features were compared to the type of nerve conduction abnormalities and to the specificities of high-titer serum antiglycolipid antibodies. Antibody specificities were determined by an enzyme-linked immunosorbent assay using purified glycolipids and carbohydrates as substrates. Our results show that high titers of antibodies to glycolipids are common in sera of patients with lower motor neuron syndromes. Selective patterns of reactivity indicate that specific carbohydrate epitopes on the glycolipids are the targets of the high-titer antibodies in individual patients with lower motor neuron syndromes. Several distinct lower motor neuron syndromes can be identified based on clinical, physiological, and antiglycolipid antibody characteristics. These syndromes include multifocal motor neuropathy with evidence of multifocal conduction block on motor, but not sensory, axons and frequent (84%) high titers of anti-GM1 ganglioside antibodies; a lower motor neuron syndrome with predominantly distal weakness early in the disease course, no conduction block, and a high incidence (64%) of anti-GM1 antibodies; and a lower motor neuron syndrome with predominant early weakness in proximal muscles and serum antibodies to asialo-GM1 that do not cross-react with GM1 ganglioside.

Original languageEnglish (US)
Pages (from-to)316-326
Number of pages11
JournalAnnals of Neurology
Volume27
Issue number3
StatePublished - Mar 1990

Fingerprint

Neural Conduction
Motor Neurons
Antibodies
Glycolipids
G(M1) Ganglioside
Serum
Carbohydrates
Antibody Specificity
Axons
Reflex
Epitopes
Anti-Idiotypic Antibodies
Enzyme-Linked Immunosorbent Assay
Muscles
Incidence

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Lower motor neuron syndromes defined by patterns of weakness, nerve conduction abnormalities, and high titers of antiglycolipid antibodies. / Pestronk, A.; Chaudhry, Vinay; Feldman, E. L.; Griffin, J. W.; Cornblath, David; Denys, E. H.; Glasberg, M.; Kuncl, R. W.; Olney, R. K.; Yee, W. C.

In: Annals of Neurology, Vol. 27, No. 3, 03.1990, p. 316-326.

Research output: Contribution to journalArticle

Pestronk, A, Chaudhry, V, Feldman, EL, Griffin, JW, Cornblath, D, Denys, EH, Glasberg, M, Kuncl, RW, Olney, RK & Yee, WC 1990, 'Lower motor neuron syndromes defined by patterns of weakness, nerve conduction abnormalities, and high titers of antiglycolipid antibodies', Annals of Neurology, vol. 27, no. 3, pp. 316-326.
Pestronk, A. ; Chaudhry, Vinay ; Feldman, E. L. ; Griffin, J. W. ; Cornblath, David ; Denys, E. H. ; Glasberg, M. ; Kuncl, R. W. ; Olney, R. K. ; Yee, W. C. / Lower motor neuron syndromes defined by patterns of weakness, nerve conduction abnormalities, and high titers of antiglycolipid antibodies. In: Annals of Neurology. 1990 ; Vol. 27, No. 3. pp. 316-326.
@article{62078c3860bd4f52b351167649fb2015,
title = "Lower motor neuron syndromes defined by patterns of weakness, nerve conduction abnormalities, and high titers of antiglycolipid antibodies",
abstract = "We studied 74 patients with progressive, asymmetrical lower motor neuron syndromes. Clinical features of these patients, including age, sex, disease duration, patterns of weakness, and reflex changes, were evaluated by review of records. In each patient the clinical features were compared to the type of nerve conduction abnormalities and to the specificities of high-titer serum antiglycolipid antibodies. Antibody specificities were determined by an enzyme-linked immunosorbent assay using purified glycolipids and carbohydrates as substrates. Our results show that high titers of antibodies to glycolipids are common in sera of patients with lower motor neuron syndromes. Selective patterns of reactivity indicate that specific carbohydrate epitopes on the glycolipids are the targets of the high-titer antibodies in individual patients with lower motor neuron syndromes. Several distinct lower motor neuron syndromes can be identified based on clinical, physiological, and antiglycolipid antibody characteristics. These syndromes include multifocal motor neuropathy with evidence of multifocal conduction block on motor, but not sensory, axons and frequent (84{\%}) high titers of anti-GM1 ganglioside antibodies; a lower motor neuron syndrome with predominantly distal weakness early in the disease course, no conduction block, and a high incidence (64{\%}) of anti-GM1 antibodies; and a lower motor neuron syndrome with predominant early weakness in proximal muscles and serum antibodies to asialo-GM1 that do not cross-react with GM1 ganglioside.",
author = "A. Pestronk and Vinay Chaudhry and Feldman, {E. L.} and Griffin, {J. W.} and David Cornblath and Denys, {E. H.} and M. Glasberg and Kuncl, {R. W.} and Olney, {R. K.} and Yee, {W. C.}",
year = "1990",
month = "3",
language = "English (US)",
volume = "27",
pages = "316--326",
journal = "Annals of Neurology",
issn = "0364-5134",
publisher = "John Wiley and Sons Inc.",
number = "3",

}

TY - JOUR

T1 - Lower motor neuron syndromes defined by patterns of weakness, nerve conduction abnormalities, and high titers of antiglycolipid antibodies

AU - Pestronk, A.

AU - Chaudhry, Vinay

AU - Feldman, E. L.

AU - Griffin, J. W.

AU - Cornblath, David

AU - Denys, E. H.

AU - Glasberg, M.

AU - Kuncl, R. W.

AU - Olney, R. K.

AU - Yee, W. C.

PY - 1990/3

Y1 - 1990/3

N2 - We studied 74 patients with progressive, asymmetrical lower motor neuron syndromes. Clinical features of these patients, including age, sex, disease duration, patterns of weakness, and reflex changes, were evaluated by review of records. In each patient the clinical features were compared to the type of nerve conduction abnormalities and to the specificities of high-titer serum antiglycolipid antibodies. Antibody specificities were determined by an enzyme-linked immunosorbent assay using purified glycolipids and carbohydrates as substrates. Our results show that high titers of antibodies to glycolipids are common in sera of patients with lower motor neuron syndromes. Selective patterns of reactivity indicate that specific carbohydrate epitopes on the glycolipids are the targets of the high-titer antibodies in individual patients with lower motor neuron syndromes. Several distinct lower motor neuron syndromes can be identified based on clinical, physiological, and antiglycolipid antibody characteristics. These syndromes include multifocal motor neuropathy with evidence of multifocal conduction block on motor, but not sensory, axons and frequent (84%) high titers of anti-GM1 ganglioside antibodies; a lower motor neuron syndrome with predominantly distal weakness early in the disease course, no conduction block, and a high incidence (64%) of anti-GM1 antibodies; and a lower motor neuron syndrome with predominant early weakness in proximal muscles and serum antibodies to asialo-GM1 that do not cross-react with GM1 ganglioside.

AB - We studied 74 patients with progressive, asymmetrical lower motor neuron syndromes. Clinical features of these patients, including age, sex, disease duration, patterns of weakness, and reflex changes, were evaluated by review of records. In each patient the clinical features were compared to the type of nerve conduction abnormalities and to the specificities of high-titer serum antiglycolipid antibodies. Antibody specificities were determined by an enzyme-linked immunosorbent assay using purified glycolipids and carbohydrates as substrates. Our results show that high titers of antibodies to glycolipids are common in sera of patients with lower motor neuron syndromes. Selective patterns of reactivity indicate that specific carbohydrate epitopes on the glycolipids are the targets of the high-titer antibodies in individual patients with lower motor neuron syndromes. Several distinct lower motor neuron syndromes can be identified based on clinical, physiological, and antiglycolipid antibody characteristics. These syndromes include multifocal motor neuropathy with evidence of multifocal conduction block on motor, but not sensory, axons and frequent (84%) high titers of anti-GM1 ganglioside antibodies; a lower motor neuron syndrome with predominantly distal weakness early in the disease course, no conduction block, and a high incidence (64%) of anti-GM1 antibodies; and a lower motor neuron syndrome with predominant early weakness in proximal muscles and serum antibodies to asialo-GM1 that do not cross-react with GM1 ganglioside.

UR - http://www.scopus.com/inward/record.url?scp=0025347028&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0025347028&partnerID=8YFLogxK

M3 - Article

VL - 27

SP - 316

EP - 326

JO - Annals of Neurology

JF - Annals of Neurology

SN - 0364-5134

IS - 3

ER -