Titre : | Multicentric study on limb girdle muscular dystrophy type 2A (calpainopathy): a 4-year follow-up : Communication lors du 4th International Congress of Myology (4ème colloque international de Myologie) 9-13 mai 2011 Lille, France |
contenu dans : | |
Auteurs : | Hogrel JY, Auteur ; Canal A ; Ollivier G ; Decostre V ; Payan C ; Fardeau M |
Type de document : | Article |
Editeur : | Institut de Myologie AIM, 2011 |
Pages : | p 117 |
Langues: | Anglais |
Résumé : |
Poster :
A natural history study on calpainopathy was performed in 3 centres: two centres representing ethnical and geographical isolates at the source of the original mutation (Northern Basque Country and Rion Island) and the Institute of Myology, with a more heterogeneous population. The study lasted for four years with a total of 86 patients included (21 at the Institute of Myology, 35 in the Northern Basque Country and 30 in the Rion Island). Patients were between 16 and 65 years old at inclusion.Patient assessments were based on muscular and functional evaluations: manual muscle testing (MMT) on 23 muscles or muscles group; quantified muscle testing (QMT) on elbow and knee flexors and extensors and grip muscles; isometric muscle testing with the Biodex system on elbow and knee flexors and extensors; and the Motor Function Measure (MFM). Evaluations were performed every 6 months for 2 years and then once a year.Results show a very slow deterioration rate for most patients. This study also highlighted the selectivity of MMT to discriminate affected muscles. Some muscles of muscles groups were shown as significantly affected (latissimus dorsi, pectoralis major, trapezius, psoas major, gluteus medius, adductors, hamstring muscles, abdominal and spinal muscles). Others, however, appeared to be notably preserved (triceps brachii, radialis, palmaris, sternocleidomastoideus, triceps surae, tibialis anterior, tibialis posterior and peroneus). The slow evolution rate of strength parameters over 4 years underlines the difficulties to recommend evaluation criteria of therapeutical effects that are sensitive to changes occurring over durations which are usually much shorter than 4 years. |