Treatable gait disorder and polyneuropathy associated with high titer serum IgM binding to antigens that copurify with myelin-associated glycoprotein.
We studied clinical and electrodiagnostic features of 9 patients with very high titers (> 1:10,000) of serum IgM binding to a CNS myelin antigen (CMA) preparation that copurified with myelin-associated glycoprotein (MAG). We found that 8 of the 9 patients had a combined syndrome of gait ataxia and polyneuropathy (GAPN) with late-age onset (mean = 70 years of age). In the 8 GAPN patients progressive difficulty with ambulation led to significant functional disability and frequent falling. Examination showed a wide-based unsteady gait, especially when standing still or turning. There was mild-to-moderate distal sensory loss with involvement of joint position sense only in the toes. Motor changes, when present, were mild and mainly involved distal leg musculature. Treatment of 5 GAPN patients resulted in clear improvement of 2 after intravenous human immunoglobulin and of 3 others after other immunodulating agents. Immune-mediated GAPN syndromes with high titers of serum IgM binding to CMA appear to be treatable causes of gait disorders in older patients.
Pestronk
A
A
Department of Neurology, Washington University School of Medicine, St. Louis, Missouri 63110.
Choksi
R
R
Bieser
K
K
Goldstein
J M
JM
Adler
C H
CH
Caselli
R J
RJ
George
E B
EB
D000368:Aged; D000906:Antibodies / Q000097:blood*; D000941:Antigens / Q000032:analysis; D001259:Ataxia / Q000150:complications / Q000276:immunology; D005260:Female; D005684:Gait; D006801:Humans; D007075:Immunoglobulin M / Q000032:analysis*; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008875:Middle Aged; D009069:Movement Disorders / Q000150:complications* / Q000276:immunology* / Q000628:therapy; D009185:Myelin Proteins / Q000276:immunology*; D018992:Myelin-Associated Glycoprotein; D009419:Nerve Tissue Proteins / Q000032:analysis; D010523:Peripheral Nervous System Diseases / Q000150:complications* / Q000276:immunology* / Q000628:therapy
D000906:Antibodies; D000941:Antigens; D007075:Immunoglobulin M; D016756:Immunoglobulins, Intravenous; D009185:Myelin Proteins; D018992:Myelin-Associated Glycoprotein; D009419:Nerve Tissue Proteins