Home Based Personalized Training and Video Consultation in Mitochondrial Myopathies: Study of Efficacy and Tolerance.

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.


Eligibility Criteria:
Inclusion criteria:

* patient over 18 years-old,
* with genetically-confirmed mitochondria myopathy,
* with mitochondrial DNA mutation,
* clinically dominant myopathic-like disorders according investigator,
* able to walk with or without assistance,
* likely to be followed for at least one year without interruption by video consultation (implying internet connexion with computer and camera or smartophone),
* having given the written and informed consent and being social security member.
* For patients in childbearing age, she must have used at least one month if reliable contraception.

Exclusion criteria:

* 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,
* patient expecting pregnancy during the course of the study,
* patient protected by law or under guardianship or curatorship, or not able to participate in a clinical trial under L.1121-16 article of French Public Health Regulations.