Late motor involvement in cases presenting as “chronic sensory demyelinating polyneuropathy”

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is usually characterized by prominent motor deficits. A pure sensory presentation, labeled chronic sensory demyelinating neuropathy (CSDN), has been reported, but it is unclear if this neuropathy is a distinct clinical and immunologic entity or merely the sensory presentation of the more usual sensorimotor CIDP. We describe 5 patients with what initially appeared to be CSDN; 3 subsequently developed substantial weakness coincident with the electrophysiologic appearance of multifocal motor conduction block. These cases indicate that, in some cases, CSDN may be a transitional clinical stage of CIDP in which the more usual sensorimotor deficits develop later. Immune-based therapy, including intravenous immunoglobulin, was found to be effective in both the pure sensory and sensorimotor types.