A. Pestronk et al., TREATABLE GAIT DISORDER AND POLYNEUROPATHY ASSOCIATED WITH HIGH-TITERSERUM IGM BINDING TO ANTIGENS THAT COPURIFY WITH MYELIN-ASSOCIATED GLYCOPROTEIN, Muscle & nerve, 17(11), 1994, pp. 1293-1300
We studied clinical and electrodiagnostic features of 9 patients with
very high titers (>1:10,000) of serum IgM binding to a CNS myelin anti
gen (CMA) preparation that copurified with myelin-associated glycoprot
ein (MAG). We found that 8 of the 9 patients had a combined syndrome o
f gait ataxia and polyneuropathy (GAPN) with late-age onset (mean = 70
years of age). in the 8 GAPN patients progressive difficulty with amb
ulation led to significant functional disability and frequent falling.
Examination showed a wide-based unsteady gait, especially when standi
ng stilt or turning. There was mild-to-moderate distal sensory loss wi
th 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 immunodul
ating 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. (C) 1994 John Wiley & Sons, Inc.