Motor conduction block and high titres of anti-GM1 ganglioside antibodies: Pathological evidence of a motor neuropathy in a patient with lower motor neuron syndrome

Abstract

A patient with a progressive lower motor neuron syndrome had neurophysiological evidence of motor axon loss, multifocal proximal motor nerve conduction block, and high titres of anti-ganglioside GM1 antibodies. Neuropathological findings included a predominantly proximal motor radiculoneuropathy with multifocal IgG and IgM deposits on nerve fibres associated with a loss of spinal motor neurons. These findings support an autoimmune origin of this lower motor neuron syndrome with retrograde degeneration of spinal motor neurons and severe neurogenic muscular atrophy.