Hypermobilité du membre supérieur (épaule exceptée)

Abstract

The benign joint hypermobility syndrome (BJHS) where the joint hypermobility is the major sign will be essentially treated. The other main diseases where the joint hypermobility could be present are Marfan syndrome, Stickler syndrome and osteogenesis imperfecta. The localized hypermobility following a trauma will be excluded. BJMS is related to Ehlers-Danlos hypermobility type, previously named type III. The diagnosis is based on an association of criterion according to Brighton. Determining the Beighton'score is the first step for making the diagnosis. A score higher than 4 is required (the highest possible score is 9). The fingers and elbows localization is important reaching 6/9 of the total score. It is a connective tissue disorder, genetically determined with an autosomal dominant transmission. The abnormalities concern the synthesis of fibrillar collagens or tenascin-X (non collagenic molecule of extracellular matrix). Pain are frequent and severe with functional impairment and repercussions on day-life, sleep and quality of life.