Positive effect of physical activity on health arouses a strong interest at international level and is developped within the scope of national programs. Recommandations exist but must be designed for patients with functional limitations of activities.
Patients with mitochondrial diseases have exercice intolerance with an increase of muscular weakness and fatigue after low exercice volume. Theses patients have functional limitations of activities. In order to establish an appropriate training programme, it will be important to define and consider the physical condition. The Society of Mitochondrial Medecine published recommandations for management of theses patients,However, theses recommandations do not allow them to propose a training program of what can be done. For these vulnerable patients, therapists are responsible fo advising a training programm without guidelines to establish its terms and conditions. In addition, some exercices do not appear to have been the subject of complete assessmeents.
Regarding training programs (aerobic training, muscle reinforcement, miwed training), scientific literature shows a significant genetic and clinical variabilities, as well as a lack of data on clinical severity of included patients. In addition, the lack of informations regarding training effects of heteroplamy level limits our comprehension of mechanisms involved in adaptation of mitochondrial pool during training. Therefore, further reserchs on this subject are essential.
It is necessary to offer these patients a follow-up and personalized training program, which are in adequation with daily life. Some publications call on specifics concepts which are not compatible with day-to-day life. The investigators think it will be useful to investigate training effects in order to have practival conclusions, easily reproducible at home by patients with simple and inexpensive equipment. In this context, video consultation could allow the close follow-up of these patients.
The investigators hypothesize that a mixed training (endurance and muscle reinforcement), personalized, at home and followed by video consultation have positive effects on some physical criteria (such as musclar strength, tolerance to effort, functional abilities) without increasing heteroplasmy and creatine phosphokinase levels.
patient over 18 years-old,
with genetically-confirmed mitochondria myopathy,
with mitochondrial DNA mutation,
clinically dominant myopathic-like disorders according investigator,
patient wih pathology or comorbidity incompatible with the conduct of the study according the investigator during inclusion visit,
patient with physical condition incompatible with training program according investigator during inclusion visit,
patient having a physical tiredness and/or myalgia during last week with an intensity of 6 on visual and analog scale,
pregnant or nursing patient,
Single group
Personalized and mixed training program
Procedure/SurgeryPatients perform a personalized and mixed training program (endurance and muscle reinforcement), at home
Change of overall functional capacity of moving during training period from 6 months (S0) to 9 (S12) and 12 (S24) months
The overall functional capacity of moving will be measured by the time to perform the Timed Up and Go test.
Change of muscular strength of upper and lower limbs during training period from 6 months (S0) to 9 (S12) and 12 (S24) months
Muscular strength of upper (shoulder abduction, elbow and fingers bending) and lower (knee extension) limbs during isometric and maximal muscular contractions will be measured with a quantified muscle testing device. Upper limbs strength will also measured during the developped sitting with dumbbells.
Change of muscular endurance of upper and lower limbs during training period from 6 months (S0) to 9 (S12) and 12 (S24) months
Muscular endurance of upper (shoulder abduction, elbow and fingers bending) and lower (knee extension) limbs during isometric and maximal muscular contractions, keeped during 30 secondes will be measured by the difference of the force between the beggining and the end of the contraction
Change of functional abilities in walking during training period from 6 months (S0) to 9 (S12) and 12 (S24) months
Modification of functional abilities in walking will be measured by the maximal distance of walking (in meter) during a 6-minutes walking test
Change of functional abilities during transferts during training period from 6 months (S0) to 9 (S12) and 12 (S24) months
Modification of functional abilities during transferts will be measured by the maximal number of transferts sitting to standing performed during a 1-minute chair lifts test
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