* Interest of the canine models in hereditary myopathies 

* Characterization of hereditary  myopathies

* Research of therapeutic processes

* Development of tools of functional evaluations





Development of functional evaluating tools


Each evaluation method presented below allows distinguishing significantly healthy dogs and dystrophic dogs. Therefore, these tools are able to highlight the benefits provided by different therapeutic strategies applied to the dystrophic dogs.



Qualitative evaluation of the motor abilities using clinical scores

In order to track the locomotive evolution of the dogs, we established a grid of clinical scores. 12 locomotive criteria (12 items) are examined (gait, weight transfer, jumping, crossing of an obstacle, etc). For each item, a mark between 0 and 2 is assigned; the zero value corresponding to the normal situation, the value 2 corresponding to the most critical situation. Each animal is evaluated once a month.




Quantitative evaluation of the gait quality using accelerometry
Project coordinator: Ines Barthelemy   

The accelerometry measurements consist in recording dog accelerations according to three axes (dorso-ventral, medio-lateral, cranio-caudal).




The accelerometer (Equimetrix device ®) is fixed under the dog sternum using an elastic belt.  Healthy and dystrophic dogs are encouraged to walk as spontaneously as possible along a corridor. Accelerations are recorded without interruptions and data are analyzed by samples of 10 seconds of stabilized walk. This study is conducted with the scientific assistance of Jean Yves Hogrel (Institute of Myology).
 
Examples of recordings showing the irregular gait of the dystrophic dog.  


Muscle characterization by MNR
Project coordinator: Jean Laurent Thibaud

In the aim to appreciate objectively and non-invasively the muscle state, MNR analyses are performed in collaboration with the team of the professor Pierre Carlier (Institute of Myology). Using a SIEMEMS imager equipped with a magnet of 3 Tesla, standard and fat-saturated T1
-weighted images and T2-weighted images are acquired. After gadolinium injection, dynamic acquisitions of fat-saturated T1-weighted images are performed.
 

Comparison of T2-weighted images between a healthy and a GRMD dog shows a heterogeneous signal between and within the muscles of the GRMD dog.

Analysis of the muscular force in vivo
Project coordinator: Ines Barthelemy

This examination consists in the measurement of the force generated by the foot flexion and the finger extension following the stimulation of the fibular nerve (under the knee). Six trains of 100 stimulations of 0,1ms at 50Hz are applied with one-minute interval. The absolute tetanic contraction of a leg is given by calculating the average of five tetanic contractions measurements. This value is standardized according to the animal weight in order to compare results obtained for different animals.