Jornal de Síndromes Genéticas e Terapia Gênica
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Conversion to Steroid-Responsiveness in a Patient with Motor-Dominant Chronic Inflammatory Demyelinating Polyneuropathy

Kataoka H, Kobayashi Y, Terashima M, Sugie K and Ueno S

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.