Motor neuron diseases of domestic animals have rarely been described.
Three cats with adult-onset, chronic, progressive generalized muscle w
eakness characterized initially by trembling on exertion and later by
extreme difficulty in walking, cervical ventroflexion, dysphagia, and
marked muscle atrophy were elevated. Spinal reflexes were evident earl
y but were nondetectable as the disease progressed. Electromyography r
evealed fibrillation potentials, with nerve conduction velocities with
in the reference range. Histologic examination of muscle specimens rev
ealed denervation. Marked neuron loss and gliosis were detected in the
ventral horns of the spinal cord, with atrophy of ventral nerve rootl
ets. Less dramatic neuron loss was seen in brain stem motor nuclei. El
ectron microscopic examination of the ventral horns disclosed hypertro
phied astrocytes, with densely arrayed intermediate filaments, swollen
axons with large filamentous accumulations, and many macrophages with
lipofuscin-like inclusions. Clinical and pathologic findings were con
sistent with a progressive neurodegenerative disease affecting spinal
and some bulbar motor nuclei.