A 4-month-old, female collie-cross dog was presented for evaluation of slowly progressive weakness, exercise intolerance and muscle atrophy. Neurological examination and electrodiagnostic testing were consistent with a generalized myopathy or, less likely, an axonal polyneuropathy. Muscle biopsy samples revealed marked variability in myofibre size with scattered or clustered atrophic or hypotrophic type 1 fibres. Type 1 fibres were 65% smaller than type 2A fibres and the percentage of type 1 fibres exceeded reference values for both limb muscles examined. On the basis of the clinical evaluation, pathological changes and the absence of another defined congenital or acquired myopathy, a diagnosis of a myopathy associated with congenital fibre type disproportion was made. Three months later the animal was humanely euthanized because of worsening clinical signs.
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