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Skeletal muscle and peripheral nerve were obtained over several years from 12 Labrador retrievers with signs of hereditary myopathy. Biopsy and necropsy samples were examined by histology and histochemistry. Despite normal findings in peripheral nerves, a wide range of morphologic changes were observed in muscle including features generally considered characteristic of neurogenic disease. These included small and large muscle fiber group atrophy, presence of small angular fibers, and occasional fiber type grouping. Other prominent changes in muscle were increased numbers of internal nuclei, architectural disturbances, type II fiber deficiency, necrosis, regeneration, and fibrosis--all of which are more usually associated with destructive myopathies or muscular dystrophies. The pathology of this condition, therefore, includes features of both neurogenic and myopathic disease, while the underlying pathophysiology remains unclear.