Progressive axonopathy: an inherited neuropathy of Boxer dogs. 1. Further studies of the clinical and electrophysiological features

The clinical and electrophysiological findings in 14 Boxer dogs with progressive axonopathy (PA) are described. The salient clinical features are hind-limb ataxia which may later involve forelegs, proprioceptive defects, hypotonia, patellar areflexia, absence of muscle atrophy and, in a few instances, ocular tremor and head bobbing. The outward signs are often observed by 2–3 months of age but clinical testing can reveal patellar areflexia at 1 month of age. After an initial progression, the signs may stabilize and dogs are alive at 4 years of age having shown no deterioration over the previous 2 years. The electrophysiology shows reduced motor nerve conduction velocities and evoked potential amplitudes after about 4 months of age. The F-wave latency is considerably increased. Sensory nerves also show a marked reduction in the amplitude of their evoked potentials and eventually cease to conduct. Abnormal spontaneous activity is not a feature on electromyography. The conduction defects probably reflect to some degree the reduced nerve fibre diameters and myelin sheath changes found in PA although other factors may also be operating. The study shows that PA can be diagnosed with reasonable confidence by routine clinical testing at an early age.