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The sensitivity and specificity of anti-GM sub 1 antibody testing

From the Program in Molecular Neuroscience and Department of Neurology, Mayo Clinic and Mayo Foundation, Rochester, MN.
Supported by NIH NS 14304 and Mayo Foundation.
Received December 5, 1995. Accepted in final form January 24, 1996.
Address correspondence and reprint requests to Dr. Anthony J. Windebank, Mayo Clinic and Mayo Foundation, Program in Molecular Neuroscience, 1501 Guggenheim Building, 200 First Street SW, Rochester, MN 55905.

Elevated titers of antibodies directed at ganglioside epitopes have been associated with multifocal motor neuropathy (MMN), motor variant of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), amyotrophic lateral sclerosis (ALS), and other motor neuropathies.Anti-GM₁ antibodies were measured in 675 patients: 180 age- and sex-stratified healthy blood bank controls, 182 normal controls who had full neurologic assessment including electromyography, 121 patients with definite ALS, 19 patients with pure sensory neuropathy, and 173 consecutive patient serum samples submitted for GM₁ antibody testing. Antibodies to three ganglioside epitopes were determined by ELISA: IgM and IgG anti-monosialo GM₁, asialo GM₁, and disialo GD_1b. Antibody titers for normal subjects and patients with ALS were used to determine normal values and borderline levels below which 99% of normal and 99% of ALS patient titers were found. Clinical evaluation of the next 173 consecutive patients referred for anti-GM₁ antibody testing revealed 36 patients with motor neuropathies. Sera from 18 of these patients had titers above the 99% normal threshold and 14 had titers above the ALS and normal borderline threshold. All 14 with elevated sera titers were from patients with motor neuropathy or neuronopathy. Sixteen patients met the clinical and electrophysiologic criteria for MMN; 10 had elevated titers. Ten patients had the motor variant of CIDP without conduction block and three had elevated titers. Anti-IgM asialo GM₁ antibodies had the highest sensitivity and specificity. High-titer IgM antibodies against monosialo GM₁ occurred only in patients with various forms of pure motor neuropathy (100% specificity). The sensitivity was 50% for this referral-based population.

NEUROLOGY 1996;47: 951-955

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