Differential susceptibility of the ocular motor system to disease

This review summarizes an alternative approach to the understanding of neuromuscular disease. By contrasting disease susceptibility of extraocular muscle and ocular motor neurons, it is hoped that unique insights into disease mechanisms may be identified. Disorder of eye movements leads to dramatic symptoms for patients and the ocular motor system is relatively limited in its ability to compensate rapidly for such disruptions. However, more profound reasons exist as to why myasthenia gravis compromises neuromuscular transmission at ocular muscle synapses as well as why Graves' ophthalmopathy exists. In contrast, muscular dystrophies spare the eye muscles while devastating all other skeletal muscles; the same is true for motor neuron diseases. It is hoped that this review will encourage others to view the world of neuromuscular diseases as delineated into those that spare the ocular motor system and those that do not.