Lower motor neuron syndromes defined by patterns of weakness, nerve conduction abnormalities, and high titers of antiglycolipid antibodies.
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.
Pestronk
A
A
Department of Neurology, Washington University School of Medicine, St. Louis, MO 63110.
Chaudhry
V
V
Feldman
E L
EL
Griffin
J W
JW
Cornblath
D R
DR
Denys
E H
EH
Glasberg
M
M
Kuncl
R W
RW
Olney
R K
RK
Yee
W C
WC
D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000378:metabolism*; D005260:Female; D005677:G(M1) Ganglioside / Q000276:immunology*; D006017:Glycolipids / Q000276:immunology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology*; D009431:Neural Conduction; D009468:Neuromuscular Diseases / Q000276:immunology / Q000503:physiopathology*; D013577:Syndrome
D001323:Autoantibodies; D006017:Glycolipids; D005677:G(M1) Ganglioside