CHRONIC INFLAMMATORY DEMYELINATING POLYRADICULONEUROPATHY IN CHILDREN.2. LONG-TERM FOLLOW-UP, WITH COMPARISON TO ADULTS

We previously reviewed the presentation, initial clinical course, and electrodiagnostic features of children with chronic inflammatory demye linating polyradiculoneuropathy (CIDP), We now report the long-term fo llow-up of 12 children with idiopathic CIDP, and compare these to 62 a dults with idiopathic CIDP. Children often had more rapidly fluctuatin g courses than adults. A relapsing course was significantly more commo n in children than in adults. The recovery of children from each episo de of deterioration was usually excellent, and better, on average, tha n in adults. Ventilatory support was never required for children with slowly evolving illness; only 2 children with a precipitous onset clin ically resembling Guillain-Barre syndrome required ventilatory support . Prednisone, plasma exchange, and intravenous immunoglobulin (IVIg) u sually were effective in children. Multiple courses of IVlg could be g iven with continued efficacy. Treatment often could be discontinued in children with relapsing courses. The prognosis for children was excel lent. Adults demonstrated a good, but more variable, outcome. (C) 1997 John Wiley & Sons, Inc.