Congenital Muscular Dystrophy and Rigid Spine Syndrome

Abstract

In three patients with muscular dystrophy, the unexpected occurrence of spinal stiffness suggested a diagnosis of rigid spine syndrome. One case belongs to the benign form of the congenital muscular dystrophies; in the other cases however, the severity and the distribution of the muscular process, associated with neurological abnormalities, seems to correspond to a unique variant of congenital muscular dystrophy. These observations underline the heterogeneity of the rigid spine syndrome.