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Late motor involvement in cases presenting as "chronic sensory demyelinating polyneuropathy"
A R Berger1, S Herskovitz, J Kaplan
1Department of Neurology, Montefiore Medical Center, Bronx, NY 10467, USA.
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.