Multifocal demyelinating motor neuropathy: pathologic evidence of 'inflammatory demyelinating polyradiculoneuropathy'

We report a case of multifocal demyelinating motor neuropathy in a patient with a 5-year history of progressive, asymmetric, predominantly motor weakness characterized by multifocal progression, multifocal conduction block, and lack of response to steroid therapy. Neuropathologic findings at autopsy showed an "inflammatory demyelinating polyradiculoneuropathy" in the motor cranial nerves and motor roots of peripheral nerves, an extensive deposition of IgG and focal accumulations of IgM in the peripheral nerve motor roots, and loss of motor neurons. These findings clearly document an inflammatory demyelinating polyradiculoneuropathy in multifocal demyelinating motor neuropathy, suggesting a close relation with chronic inflammatory demyelinating polyneuropathy.