A congenital myopathy is reported in the only male puppy from a litter
of Brittany Spaniels. The condition was manifested clinically as gene
ralized weakness, mild generalized muscle atrophy and difficulty in ea
ting due to tongue muscle hypertrophy. Creatine kinase values were ext
remely high. Electromyographically, there were complex repetitive disc
harges that persisted (pseudomyotonia). Histopathologic examination re
vealed muscle fiber degeneration, size variation, hyalinization, miner
alization, necrosis, and regeneration. Clinical signs, serum biochemis
try, electrophysiological and pathological findings of this myopathy h
ave much in common with the canine X-linked muscular dystrophies and w
ith Duchenne muscular dystrophy in humans.