A progressive encephalomyelopathy of insidious onset affecting a 16-mo
nth-old dog is described. Clinically, the dog was ataxic, stumbled int
o objects and showed mild behavioral abnormalities. Light microscopic
findings included profound degeneration and astrogliosis of the optic
pathways, loss of Purkinje neurons, focal bilateral and symmetrical br
ain stem spongiosis and diffuse neuroaxial astrogliosis with swollen a
nd abnormally shaped nuclei. Ultrastructurally, there were giant and b
izarre mitochondria within neuronal perikarya and axons as well as dif
fuse loosening of the cerebral and cerebellar neuropil. These neuropat
hological findings resemble the mitochondrial encephalomyopathies of m
an.