Multifocal motor neuropathy, a rare insidious immune-mediated disorder, fea
tures muscular weakness and atrophy, as well as arreflexia, due to nerve co
nduction block and is often associated with the presence of anti-GM1 antibo
dy. We report a patient with a nine-year history of progressive upper limb
weakness, misdiagnosed as amyotrophic lateral sclerosis, who responded with
in hours to intravenous immunoglobulin treatment with full recovery of musc
le strength. This case highlights the need to search for conduction block i
n patients with lower motor neuron involvement.