Serum IgG autoantibodies to GQ1b ganglioside are associated with the a
cute phase of the Miller-Fisher syndrome (MFS). We investigated the ef
fects of three anti-GQ1b-positive MFS sera in the mouse phrenic-nerve/
diaphragm preparation. Miniature endplate potential frequencies increa
sed eight-fold within 25 min, declined rapidly, and ceased altogether
after 3 h, when nerve stimulation no longer evoked a response. One MFS
convalescent serum (anti-GQ1b negative) and sera from healthy control
s and from patients with other neurological diseases were without effe
ct. Thus muscle weakness in MFS may be caused by a serum factor, likel
y to be GQ1b antibody, that leads to failure of acetylcholine release
from motor nerve terminals.