Motor neuron diseases are almost exclusively due to amyotrophic lateral sclerosis (ALS), a sporadic and rare condition that is easily diagnosed because it is a disease of spinal and cortical motor neurons. This combination causes the classic picture of lower motor neuron signs (atrophy, fascic-ulations), as well as upper motor neuron signs (brisk reflexes and upgoing toes). Sensation is normal, as this is strictly a disease of the motor system. Its cause is unknown.
Although ALS is rare, it is rather easy to diagnose because of its unique combination of signs. The weakness is associated with lower motor neuron signs (atrophy, fasciculations) due to the death of lower motor neurons and brisk rather than depressed reflexes due to the death of upper motor neurons. Recent clinical trials have shown that the clinical course of ALS can be slowed with riluzole.
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