ARTHROGRYPOSIS MULTIPLEX CONGENITA - PERINATAL AND ELECTROMYOGRAPHIC FINDINGS, DISABILITY, AND PSYCHOSOCIAL OUTCOME

Fifty-two patients with arthrogryposis multiplex congenita were follow ed up for 1 to 36 years. There were six twin pregnancies, and delivery was complicated by breech position in 16 cases. In 19 cases the cause was atrophy of the alpha motoneurons of the spinal cord, detected by electromyography. Six patients did not achieve walking ability. Severe disability for other reasons was noted in two patients who had bilate ral rigid extension contractures of the elbows and in six patients who se spinal deformities interfered with the balance of the trunk. Treatm ent of flexion contractures of the hips and knees seemed to be importa nt in case it promotes the walking ability. Spinal deformities interfe ring with the balance of the trunk should be treated operatively. Rest oration of elbow flexion was the main goal in operative treatment of t he upper extremities. The intelligence of the patients was slightly ab ove normal. The psychological analysis revealed significant diverging features compared with average population in testing situation. Social ly the patients seemed to cope well.