Chronic inflammatory demyelinating polyneuropathy of infancy: a corticosteroid-responsive disorder

We present the clinical, electrophysiological, and histopathological findings in 6 children with early-onset chronic inflammatory demyelinating neuropathy. The clinical features initially suggested a genetically determined disorder in each patient. Sural nerve biopsy showed changes of chronic demyelination with multifocal endoneurial edema and mononuclear cellular infiltrates. All children improved with corticosteroid therapy.