Case Report
Kataoka H, Kobayashi Y, Terash
Abstract
The subtypes of chronic inflammatory demyelinating polyneuropathy (CIDP) differ in clinical presentation, electrophysiological and laboratory features, and response to treatment. Steroid therapy is now accepted as a firstor second-line treatment for CIDP. Patients with steroid-resistant CIDP have been reported, especially among those with pure-motor or motor-dominant CIDP. The clinical and prognostic features of patients with motor dominant CIDP are poorly understood. We describe a patient with steroid-unresponsive motor dominant CIDP who subsequently responded to steroid therapy and review previously reported patients with motor dominant CIDP. Most patients with motor-dominant CIDP did not respond to steroids, but remarkably improved after treatment with intravenous immunoglobulins. Our findings suggest that some patients with steroid-unresponsive motor-dominant CIDP can subsequently become responsive to steroid therapy.