Insights in Neurosurgery

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Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) may be a group of rare neurological diseases that mainly involve the nerve cells (neurons) to blame for controlling skeletal muscle movement. Voluntary muscles produce movements like chewing, walking, and talking. The disease is progressive, meaning the symptoms decline over time. Currently, there's no cure for ALS and no effective treatment to halt, or reverse, the progression of the disease. ALS belongs to a wider group of disorders referred to as neuron diseases, which are caused by gradual deterioration (degeneration) and death of motor neurons. Motor neurons are nerve cells that reach from the brain to the funiculus and to muscles throughout the body. These motor neurons initiate and supply vital communication links between the brain and also the voluntary muscles. Messages from motor neurons within the brain (called upper motor neurons) are transmitted to motor neurons within the neural structure and to motor nuclei of brain (called lower motor neurons) and from the medulla spinalis and motor nuclei of brain to a selected muscle or muscles. In ALS, both the upper motor neurons and therefore the lower motor neurons degenerate or die, and stop sending messages to the muscles. Unable to function, the muscles gradually weaken, start to twitch (called fasciculations), and waste away (atrophy). Eventually, the brain loses its ability to initiate and control voluntary movements. Early symptoms of ALS usually include muscle weakness or stiffness. Gradually all muscles under voluntary control are affected, and individuals lose their strength and therefore the ability to talk, eat, move, and even breathe. Most people with ALS die from respiratory failure, usually within 3 to five years from when the symptoms first appear. However, about 10 percent of individuals with ALS survive for 10 or more years.

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