Chronic demyelinating polyneuropathy: variability of clinical and electrophysiological course and treatment outcome

Abstract

Chronic demyelinating polyneuropathy (CIDP) manifests with a monophasic, relapsing or progressive course. The pathogenesis of CIDP is still unknown, but involvement of the immune system has been firmly established. Childhood CIDP is characterized by large variability in clinical course, response to treatment, and clinical and electrophysiological outcome. Resistance to the treatment might be induced by the axonal involvement during the long course of the illness. Electrodiagnostic parameters of pronounced demyelination or axonal damage might remain unchanged during long-term follow-up although the clinical outcome is usually favourable. CIDP in children with a variable clinical course and elecrophysiological findings during long-term follow up are presented and discussed.