Late-onset Becker-type muscular dystrophy in a Border terrier dog

Area of Science:

• Veterinary Neurology
• Canine Myology
• Genetic Disorders

Background:

• Muscular dystrophies are debilitating genetic muscle diseases.
• Dystrophinopathies, a subset of muscular dystrophies, result from dystrophin protein defects.
• Canine models of muscular dystrophy are crucial for understanding human disease.

Observation:

• A 9-year-old Border terrier presented with a year of progressive stiffness and exercise intolerance.
• Neurological and electrophysiological examinations suggested a neuromuscular disorder.
• Muscle biopsy revealed a severe, non-inflammatory, dystrophic myopathy.

Findings:

• Immunofluorescence and western blotting confirmed a dystrophinopathy.
• An 80-kDa truncated dystrophin fragment was identified, analogous to Becker muscular dystrophy in humans.
• This is the first reported case of late-onset Becker-type muscular dystrophy in a dog and the first dystrophinopathy in a Border terrier.

Implications:

• Canine muscular dystrophy diagnosis should consider late-onset signs and mild creatine kinase elevations.
• This case expands the known spectrum of canine dystrophinopathies.
• Further research into canine muscular dystrophies can inform human therapeutic strategies.