What causes amyotrophic lateral sclerosis?The symptoms of amyotrophic lateral sclerosis are caused by the death of motor neurons in the spinal cord and brain. Normally, these neurons convey electrical messages from the brain to the muscles to stimulate movement in the arms, legs, trunk, neck, and head. As motor neurons die, the muscles cannot be moved as effectively, and weakness results. In addition, lack of stimulation leads to muscle wasting, or loss of bulk. Involvement of the upper motor neurons causes
spasms and increased tone in the limbs, and abnormal reflexes. Involvement of the lower motor neurons causes muscle wasting and twitching (fasciculations).
Although many causes of motor neuron degeneration have been suggested for amyotrophic lateral sclerosis, none has yet been proven responsible. Results of recent research have implicated toxic molecular fragments known as free radicals. Some evidence suggests that a cascade of events leads to excess free radical production inside motor neurons, leading to their death. Why free radicals should be produced in excess amounts is unclear, as is whether this excess is the cause or the effect of other degenerative processes. Additional agents within this toxic cascade may include excessive levels of a neurotransmitter known as glutamate, which may overstimulate motor neurons, thereby increasing free-radical production, and a faulty detoxification enzyme known as SOD-1, for superoxide dismutase type 1. The actual pathway of destruction is not known, however, nor is the trigger for the rapid degeneration that marks ALS. Further research may show that other pathways are involved, perhaps ones even more important than this one. Autoimmune factors or premature aging may play some role, as could viral agents or environmental toxins.