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Up to: Congenital muscular dystrophy · Neuromuscular disease caused by qualitative or quantitative defects of selenoprotein N1 · Qualitative or quantitative defects of desmin
Rigid spine syndrome
Rigid spine syndrome (RSS) is a slowly progressive childhood-onset congenital muscular dystrophy characterized by contractures of the spinal extensor muscles associated with abnormal posture (limitation of neck and trunk flexure), progressive scoliosis of the spine, early marked cervico-axial muscle weakness with relatively preserved strength and function of the extremities and progressive respiratory insufficiency.