From: The golden retriever model of Duchenne muscular dystrophy
Age at clinical onset | Gender | Clinical signs | CK | EMG | Pathologic lesions | Outcome | Reference |
---|---|---|---|---|---|---|---|
10 days | M | Body stunting; glossal, shoulder, and neck muscle hypertrophy; bradycardia | NE | NE | Gross body stunting and muscle hypertrophy. Skeletal muscle histologic evidence of variation in fiber size, hyaline fibers with calcification, and basophilia consistent with regeneration; inflammation including giant cells; milder lesions in the heart. | Euthanasia at 10 days | [88] |
6–8 weeks (two littermates were described) | M | Stiff gait with progression to a stilted, shuffling gait by 3 months. Fatigue with exercise, occasional respiratory distress, neck stiffness, resistance to jaw opening, and glossal hypertrophy. | Elevated | “Myotonia” | Severe degenerative muscle disease: myofiber necrosis, mononuclear phagocytosis, giant cells, and calcification | Euthanasia at 6–9 months when the dogs were still ambulatory | [91] |
4–5 months | M | Generalized muscle atrophy, stiff gait, dysphagia, stenotic breathing, exercise intolerance. | NE | CRDs | Gross muscle atrophy; scattered necrotic fibers undergoing phagocytosis, increased endomysial connective tissue, calcification of myofibers, targetoid fibers, and fiber type grouping; myocardial degeneration and calcification | 8 months at biopsy; outcome not given. | [82] |
8 weeks | M | Stiff, shuffling gait; simultaneous pelvic limb advancement (bunny hopping); overextension at the carpus, overflexion at the tarsus, and abduction of the paws; stifle adduction; hypertrophy of proximal limb muscles; mild vertebral kyphosis; trismus; drooling. | 13,435 IU/l | CRDs and positive sharp waves | 4 months: fiber size variation, hyaline and necrotic fibers with myophagocytosis; centrally nucleated fibers; basophilic fibers consistent with regeneration. 8 months: progressive changes with mineralization and endoymysial and perimysial fibrosis; fibers with chains of central nuclei; fiber grouping. | Euthanasia at 8 months | [93] |