P.219Bone mass, nutrition and motor function in children and adolescents with spinal muscular atrophy type II and III

Abstract

Spinal muscle atrophy (SMA) is the second most common neuromuscular disease in children with an incidence of 1:11 800 live births. The disease is inherited and is due to degeneration of the anterior horn cells in the spinal cord, leading to extensive muscle weakness. The disorder is clinically divided into three groups; SMA I, II and III. Children with SMA I never learn to sit without support and children with SMA II never learn to walk independently. Children with SMA III achieve independent walking ability, but often lose this ability over time due to muscle weakness. Spinal deformities and joint contractures are common. Bone mass depends on several factors, where diet, motor function and physical activity are some of which are of great importance In a national study, bone mass, nutrition and motor function were investigated in patients with SMA II and III aged 2-18. Twenty children with SMA type II and III have so far been examined at one occasion. The individuals are between 2 and 17 years of age with a median age of 7 years. The bone mass in the hips has been shown to be greatly reduced in all wheelchair users, but also in several of the individuals who have independent walking ability. Normal bone mass in the hips was measured in three individuals with independent walking ability and in one wheelchair user. The energy intake was assessed as good in all but three individuals. This project is expected to provide valuable knowledge of bone mass, nutritional status and motor function, and how these relate to each other. Knowledge that can form the basis for the development of evidence-based recommendations for the treatment and care of this patient group.