An ancillary imaging study confirms the potentially deleterious role of intensive sports practice in dysferlinopathies

Dysferlinopathies are hereditary muscular dystrophies resulting in motor deficits of a distal (Miyoshi myopathy) or proximal nature (LGMD R2-type Limb Girdle Muscular Dystrophy), or even a combination (proximo-distal deficit). These myopathies are due to mutations in the DYSF gene encoding dysferlin, a protein involved in membrane repair processes. Since the first disorders usually begin around the age of twenty, the question of playing sports as an compounding factor is regularly asked. 

In an article published in December 2020, researchers involved in the natural history of dysferlinopathies protocol took an interest in this subject in particular by carrying out an ancillary study of the natural history study Jain COS. The degeneration observed on T1 sequence images of the muscles of the pelvis and lower limbs of 160 adults with various forms of dysferlinopathy was analyzed and related to the degree of sports practice of each of the participants (four levels having been defined ranging from absence of sports practice until intensive activity, all between the age of 10 to 18 years). People who have had intensive physical activity seem to have worsened the degenerative process, especially in the psoas muscle. The authors thus objectively confirm that intensive sports practice is not a good thing in this pathology and that subjects diagnosed at a presymptomatic stage of the disease should refrain from it. 


Intensive Teenage Activity Is Associated With Greater Muscle Hyperintensity on T1W Magnetic Resonance Imaging in Adults With Dysferlinopathy.  Moore U, Jacobs M, Fernandez-Torron R, LLauger Rossello J, Smith FE, James M, Mayhew A, Rufibach L, Carlier PG, Blamire AM, Day JW, Jones KJ, Bharucha-Goebel DX, Salort-Campana E, Pestronk A, Walter MC, Paradas C, Stojkovic T, Mori-Yoshimura M, Bravver E, Pegoraro E, Mendell JR, Bushby K, Straub V, Diaz-Manera J.  Front Neurol. 2020 (Dec).