Familial reflex myoclonus in Labrador Retrievers

Three 6-week-old male purebred yellow Labrador Retrievers were presented with intermittent stimulus-sensitive contractions of the appendicular and axial muscles. Five littermates were normal, although the grandsire of the affected litter had sired 2 previous litters containing similarly affected pup. Although alert and responsive, the affected dogs appeared decerebrate with extensor rigidity and opisthotonus during handling. During severe episodes, respiratory distress was observed. Generalized contractions were initiated by voluntary movements, but at rest the muscles relaxed. Neurologic deficits were not detected, although efforts to elicit segmental reflexes, assess muscle tone, or assist walking resulted in generalized stiffness. Electromyograms from the semitendinous muscles (musculi semitendinosus) had increased motor unit amplitude (up to 5,000 microV) with polyphasic action potentials. There were no myotonic discharges. A reduced interference pattern was seen. A single tactile stimuli of the distal limb resulted in 3 responses, each lasting less than or equal to 10 ms at latencies of 0 to 12 ms, 20 to 30 ms, and 35 to 40 ms, characteristic for reflex myoclonus. The motor nerve conduction velocities were normal for age (32 to 35 ms). Age-matched control dogs had motor unit action potentials of 100 to 200 microV and single compound motor unit discharges to single tactile stimuli. Therapeutic trials with diazepam and clonazepam produced minimal effects on the muscle contractions. Values of urinalyses, complete blood cell counts, and serum chemistries were within normal limits. Frozen muscle section biopsy findings, including enzyme histochemical assessment of muscle fiber types, were normal.(ABSTRACT TRUNCATED AT 250 WORDS)