Steroid-responsive polyneuropathy with subacute onset in childhood.

Five pediatric patients with subacute onset polyneuropathy are presented, with electrophysiologic and pathologic data. All patients improved, the majority to resolution, with administration of prednisone. Distinguishing factors included (1) subacute onset polyneuropathy progressing gradually over weeks to months, (2) primarily motor neuropathy with little cranial nerve involvement, (3) elevated CSF protein concentration, (4) markedly delayed nerve conduction velocities, and (5) tendency toward relapse and recurrence. Although this disorder may share characteristics with the Guillain-Barré syndrome, its steroid responsiveness sets it apart clinically from the acute form of the disease. Because of the steroid responsiveness, it is important to recognize this entity.