CHRONIC PROGRESSIVE STEROID-RESPONSIVE AXONAL POLYNEUROPATHY - A CIDPVARIANT OR A PRIMARY AXONAL DISORDER

Five patients presented with chronic, progressive, predominantly motor polyneuropathy. CSF protein content was increased in 4 patients, Moto r conduction velocities and EMG were consistent with axonal involvemen t. Sural nerve conductions were normal in all cases and sural nerve bi opsy performed in 1 patient was normal. Serum antibodies to GM(1), GD( 1a), GD(1b), and GM2 were negative. All patients improved after steroi d treatment and 3 completely recovered, Because of therapeutic implica tions it is important to differentiate these patients from those with chronic idiopathic axonal neuropathies. It is unclear whether this is a primary axonal, probably immune-mediated, polyneuropathy, or whether it represents one extreme of the chronic inflammatory demyelinating p olyradiculoneuropathy spectrum characterized by severe axonal loss, We suggest that the term ''chronic inflammatory polyneuropathy,'' encomp assing cases from pure demyelinating to pure axonal neuropathies respo nsive to steroids, should be reinstated and that, like in Guillain-Bar re syndrome, different subtypes should be individuated. (C) 1996 John Wiley & Sons, Inc.