Motor nerve conduction in motor neuron disease is considered normal until the terminal stages of the disease, a notable exception being lower motor neuron syndrome associated with anti-glycolipid antibodies. We reviewed the electrophysiological findings in all our patients who were diagnosed as having amyotrophic lateral sclerosis (ALS) during the last 6 years. Six patients, clinically indistinguishable from "classical" ALS patients, out of 31 (19%) displayed motor nerve conduction abnormalities. The most consistent finding, occurring in all 6, was prolonged distal latency or reduced conduction velocity in the distal segment of the median nerve, with normal sensory conduction, suggesting possible pressure proneness of motor nerve fibers in ALS. Additional abnormalities included multifocal motor conduction slowing (3 patients), and conduction blocks (4 patients). None of the patients had paraproteinemia and anti-GM1 and anti-GD1a antibodies were not detected. Thus, a subgroup of clinically indistinguishable ALS patients may have multifocal motor nerve conduction abnormalities, indicating motor nerve fiber involvement. The etiology and pathogenesis of the peripheral nerve involvement are presently unknown.