We reviewed the clinical and electrophysiologic features of 36 patient
s with increased titers of IgM anti-GM, antibodies. Mildly elevated ti
ters of up to 3,200 were not associated with any particular clinical s
yndrome or disease. Clinically, 14 of 16 patients with highly elevated
titers of 6,400 or higher had progressive weakness with lower motor n
euron signs; six had active tendon reflexes and eight had absent refle
xes, but none had definite upper motor neuron signs. Electrophysiologi
c studies showed spontaneous activity in all 14 patients, one or more
motor conduction blocks in nine, slowed motor conductions in one, and
normal conductions in four patients. None had abnormal sensory conduct
ions. These patients presented with a syndrome that has features of, b
ut is distinct from, both motor neuron disease and demyelinating neuro
pathy.