Clinical electromyographic studies of canine X-linked muscular dystrophy

Clinical electromyographic studies were performed in dogs (6 weeks to 5.5 years old) with a degenerative myopathy analogous to Duchenne muscular dystrophy. Spontaneous activity, consisting primarily of complex repetitive discharges (pseudomyotonic discharges), was found in all dogs tested, but was most prominent in dogs greater than or equal to 10 weeks old. Myotonic discharges also were found, but were less frequent. Motor unit potentials were generally abnormally brief and frequently polyphasic. Ulnar nerve conduction velocities determined in two 4-month-old dogs were similar to those of unaffected littermates. It was concluded that canine X-linked muscular dystrophy is a primary myopathic process in which complex repetitive discharges and myotonic discharges are a prominent feature. The basis for this spontaneous activity is not known.