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Ehlers-Danlos syndrome first described by Tschernogobow (1896) in Moscow and Ehlers (1900) in Copenhagen is a mostly autosomal inherited genetic disease of collagen synthesis that sensitizes the ensemble of the connective tissue which becomes less resistant and less elastic. These two characteristics explain the symptomatology: fragility of the skin, of the vessels (haemorrhages) and the presence of a diffuse proprioceptive syndrome due to dysfunction of the receptors which are implanted into little or nonreactive connective tissue. Diagnosis of the hypermobile type of EDS is solely clinical as there is to date no genetic maker for the most frequent form of EDS. The rarity of the disease needs to be put into question before the crowd of patients at consultations. Our experience is based on an active database of 2212 patients which all fall under the Villefranche criteria. A great number of signs and symptoms have yet to be attributed to this syndrome. They are, combined with the unawareness of physicians about the syndrome, at the origin of therapeutic errors accompanied by the iatrogenic effects of prejudice towards these patients. This is the case of dystonia which is present in 75% of our cases. Dystonia plays an important part in the functional discomfort which is at the origin of a number of handicap situations. It seems to be related to dysautonomia common amongst the patients, proprioceptive problems and the multiple pains caused by the syndrome. Dystonia treatment with Amantadine and L-Dopa permits to obtain results which go further than the normally associated extrapyramidal treatment and opens new perspectives on the management of a syndrome that has been particularly difficult to treat.

Dystonia in the joint hypermobility syndrome (a.k.a. Ehlers- Danlos syndrome, hypermobility type)