Front cover image for Innovation physiothérapeutique dans l'amyotrophie spinale infantile : du modèle animal au patient

Innovation physiothérapeutique dans l'amyotrophie spinale infantile : du modèle animal au patient

Objective: Spinal Muscular Atrophy (SMA) is a group of autosomal recessive neurodegenerative diseases differing in their clinical outcome, characterized by the specific loss of spinal motor-neurons, caused by insufficient levels of SMN protein expression. No cure is presently available for SMA. While physical exercise might represent a promising approach for alleviating SMA symptoms, the lack of data dealing with the effects of different exercise types on diseased motor-units still precludes the use of exercise in SMA patients. Methods: We have evaluated the efficiency of two long-term physical exercise paradigms, either based on high intensity swimming or on low intensity running, in alleviating SMA symptoms in a mild type 3 SMA-like mouse model. Results: We found that a 10-month physical training induced significant benefits in terms of resistance to muscle damages, energetic metabolism, muscle fatigue and motor behavior. Both exercise types significantly enhanced motor-neuron survival, independently of SMN expression, leading to the maintenance of neuromuscular junctions and skeletal muscle phenotypes, particularly in the soleus, plantaris and tibialis of trained mice. Most importantly, both exercises significantly improved neuromuscular excitability properties. Besides, all these training-induced benefits are quantitatively and qualitatively related to the specific characteristics of each exercise, suggesting that the related neuroprotection is strongly dependent on the specific activation of some motor-neuron subpopulations. Interpretation: Taken together, the present data show significant long-term exercise benefits in a mild type 3 SMA context and provide important clues for designing rehabilitation programs in patients
Computer File, French, 2014