Juvenile progressive scoliosis and congenital horizontal gaze palsy

Abstract

Eleven articles reporting 39 patients with the combination of progressive scoliosis and familial congenital gaze palsy have now been published. This disorder appears to be caused by a malfunction of the normal equilibrial control mechanism related to the brainstem or the central nervous system. The scoliosis progresses when the children begin to walk. Even when they are treated with physiotherapy or brace, they often require early operation. We consider that progressive juvenile or infant scoliosis associated with familiar congenital horizontal gaze palsy constitutes a clinical entity. We report two sisters with congenital gaze palsy, in one of whom scoliosis of at least 70 degrees (Cobb) developed, and review and analyze all cases published until now.