Childhood chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic disorder of the peripheral nervous system with sensory and motor involvement, and insidious onset over a period of months. In children and adults, both proximal and distal muscles are affected. Muscle stretch reflexes are absent or depressed. Laboratory findings include elevated cerebrospinal fluid protein with no increase of mononuclear cells. Electrophysiological and pathological studies show evidence of demyelination. No control studies of the efficacy of immunomodulating therapy in childhood CIDP are available. However, several studies have indicated clinical improvement after treatment with prednisolone, plasmapheresis and intravenous immunoglobulin, but disappointing results with other immunosuppressive agents. While some children have a monophasic course, with complete recovery, others have a protracted course, with either a slowly progressive or a relapsing-remitting course, resulting in prolonged morbidity and disability.