Central Core Myopathy

Myopathy is a disease or disorder of the muscle tissue, including fibromyalgia, muscular dystrophy, and myositis.

Central core myopathy is an inherited congenital condition of the heart muscle characterized by weakness and hypotonia in infancy and delayed motor development. Muscle biopsy reveals a condensation of myofibrils and myofibrillar material in the central portion of each muscle fiber (core).

Central core myopathy is named for the appearance of the presence of cores on muscle biopsies. If the cores appear large, the name central core myopathy is used; if there are multiple small cores, the term multiminicore myopathy is used. These cores are areas within the muscle that contain no mitochondria and contain a collection of proteins that have been identified in other muscle diseases

In young Great Danes onset of clinical signs is usually before one year of age and both sexes are affected. Clinical signs are characterised by exercise intolerance, muscle wasting, and an exercise-induced tremor. Although most affected dogs have a severe form of the disease, occasional dogs may have a less pronounced form and survive into adulthood with an acceptable quality of life. The structures involved differ from those of the well-characterised central core myopathy in human beings. In fact, these structures differ from any known myopathy in human beings and likely represent a unique non-inflammatory myopathy affecting dogs.

References

1. Lujan Feliu-Pascual A, Shelton GD, Targett MP, Long SN, Comerford EJ, McMillan C, Davies D, Rusbridge C, Mellor D, Chang KC, Anderson TJ. Inherited myopathy of great Danes
2. Jan Stephen Tecklin. Pediatric Physical Therapy.