Chronic inflammatory demyelinating polyneuropathy in childhood can present acutely: A report of three cases

Three patients with chronic inflammatory demyelinating polyneuropathy (CIDP) with acute onset initially diagnosed as Guillain-Barre syndrome were presented. Case 1 had profound weakness over 8 weeks but followed a monophasic recovery course and was almost full recovery at 6 months and remained well one year later, whereas Case 2 recovered with two relapses at 4 and 5 months followed by full remission at 6 months. Case 3 had almost monthly relapses over 2 years, requiring monthly intravenous immunoglobulin and 4 courses of intravenous methylprednisolone. Despite frequent relapses, clinical evidence of areflexia and neurophysiologic evidence of chronic neuropathy, Case 3 remained strong during remission. No causes were found except Case 2 may be due to reactivated latent Epstein-Barr virus. Unlike those with subacute or indolent onset, CIDP with acute onset may represent a very distinct variant with good outcome. We believe that acute onset CIDP variant and Guillain-Barre syndrome most likely represent parts of a continuum, arbitrarily separated by their time course. The supporting arguments are presented; diagnosis and management difficulties are briefly discussed.