Appareil locomoteur SOFMER/ SOFCOT

Le 02/12/2022 de 08:00 à 09:30

Retour Session


Apport de l’IRM musculaire dans l’évaluation fonctionnelle d’adultes présentant une arthrogrypose multiple congénitale

marion finzi (Grenoble, France), Mariya Raikova (Grenoble, France), Thu Nguyen (Grenoble, France), Shendao Dai (Grenoble, France), Chantal Durand (Grenoble, France), Dominic Perennou (Grenoble, France), Klaus Dieterich (Grenoble, France)

Objective : Arthrogryposis multiplex congenita (AMC) defines multiple congenital contractures involving two or more body areas, leading to various disabilities. Muscle function is impaired. The purpose of this study was to evaluate the correlation between muscle fat infiltration and deficiencies and activity limitations in adults with AMC.


Material / Patients and Methods : This analysis relies on data of unselected adults referred to the French AMC reference center from 2010 to 2020. All underwent a pluriprofessionnal disability evaluation and had an all-body muscular MRI. We analyse each muscles with Mercuri score (muscle fat infiltration quantification). Participants were divided into Amyoplasia and Distal Arthrogryposis (DA), the two most common forms of AMC.

Results : Mean age of the 53 participants (34 females) was 34 years. We demonstrate a significant correlation between mean Mercuri score and manual testing of muscular weakness, for both upper and lower limbs, in both groups of individuals with Amyoplasia (n=35, p<0.001) and DA (n=18, p=0.003 and p=0.002 respectively). Furthermore, in Amyoplasia, they were correlated with joint stiffness (p<0.001), Mercuri score of lower limbs muscles was correlated with the six-minute-walking test (p<0.001), and Mercuri score of upper limbs and trunk muscles were correlated with the upper limbs reaching capacity (p<0.001). In DA, no further correlation was found.

Discussion - Conclusion : Our study is the first to focus on the link between functional and imaging aspects in AMC. We show that muscular MRI, a powerful diagnostic tool, is also highly useful for disability evaluation. Higher Mercuri scores were correlated with higher deficiencies and lower mobility.

Keywords : AMC, arthrogryposis, MRI, muscular fat infiltration, disability.

Disclosure of interest : aucun