Precise correlation between structural and electrophysiological disturbances in MADSAM neuropathy

•Multifocal nerve enlargement was characteristic in MADSAM neuropathy.•Nerve enlargement precisely correlated with the location of conduction block.•Factors leading to focal histological change are likely to produce conduction block.

Multifocal acquired demyelinating sensory and motor neuropathy is characterised by multifocal clinical deficits. Imaging studies have identified multifocal enlargements of nerve trunks, but a precise correlation between structural abnormalities and electrophysiological dysfunction has not been elucidated. Two patients diagnosed with multifocal acquired demyelinating sensory and motor neuropathy were evaluated with nerve conduction studies, including short segment nerve conduction studies to precisely localise motor conduction block, and ultrasound studies of corresponding nerve trunks. Motor conduction block was identified in each patient (upper limb nerves in two patients), superimposed on additional demyelinating neurophysiological features. Upper limb ultrasound studies demonstrated focal nerve enlargement that precisely correlated with neurophysiological conduction block. The results of this study suggest that factors contributing to focal structural abnormalities in multifocal acquired demyelinating sensory and motor neuropathy are also those that produce conduction block.