Ahmad Razi

Mrs. Litchfield Biology P4

12/7/10

Twenty to thirty thousand people in America suffer from amyotrophic lateral sclerosis (ALS, also known as Lou Gehrigs Disease), a fatal, progressive neuromuscular disease that causes your nerve cells in the brain and spinal cord to deteriorate. Amyotrophic is Greek for no (a-) muscle (-myo-) nourishment (-trophic). Five thousand people are estimated to be diagnosed each year. The age range for patients with ALS is between 40 and 60 years old, but it can also occur in younger and older people. Motor neurons (ALS effects voluntary movements and muscle power, but not involuntary movements) go from the brain to the spinal cord to the muscles throughout the body. Motor neurons in the brain (called upper motor neurons) transmit impulses to motor neurons in the spinal cord (lower motor neurons) that transmit the impulses to specific muscles. ALS causes the upper and lower motor neurons to die. Once the motor neurons die, the brain cannot move the muscles. Since they muscles dont move, they dont get exercise and waste away (atrophy), making arms and legs look thinner, and they twitch (fasciculation). Paralysis eventually occurs. When the motor neurons degenerate, they become unable to send impulses to muscles. One early symptom is muscle weakness, especially the arms and legs (symptoms start in one leg/arm); patients start noticing that they are tripping, having trouble buttoning their shirt, etc. Other problems include: having trouble talking (dysarthria), swallowing (dysphagia), muscle cramps, and exaggerated reflexes (hyperreflexia, like an overactive gag reflex). Breathing can also be affected, even it seems that its involuntary, we can willingly stop our breathing. Its is not like our heart; we cant willingly stop our heart. There is no cure for ALS, though there are drugs like riluzole that slow down the progression of ALS. There are also medical devices and therapy for people with ALS. There even

have been same cases of ALS where is slowed down or even stopped. There is no test to directly point out ALS; instead tests for other neurological diseases must be given to rule them out. Tests include: electromyography (EMG), which records electrical activity in muscles (certain results can support ALS); a nerve conduction velocity (NCV) test, which can help diagnose or rule out peripheral neuropathy (damage to peripheral nerves) or myopathy (muscle disease); and magnetic resonance imaging (MRI), which shows other possible causes like a tumor on the spinal cord, herniated disk in the neck, syringomyelia, or cervical spondylitis. Blood and urine tests can help, along with a muscle biopsy. Human immunodeficiency virus (HIV), human T-cell leukemia virus (HTLV), and Lyme disease are examples of diseases that can cause symptoms similar to ALS. Other neurological disorders that have symptoms like ALS include multiple sclerosis, post-polio syndrome, multifocal motor neuropathy, and spinal muscular atrophy. The cause of ALS is uncertain, but scientists have found in some cases of ALS that the gene that produces the SOD1 enzyme was mutated. The SOD1 enzyme is a strong antioxidant that protects the body from highly reactive molecules produced by cells during normal metabolism (they are called free radicals). If free radicals are not neutralized, they can damage DNA and proteins in cells. Scientists speculate that a build-up of free radicals can cause the gene to go bad. Animal studies have shown motor neuron degeneration and problems with motor function when their gene (that produces SOD1) was mutated. ALS patients also have high glutamate levels (a neurotransmitter); long time exposure to high amounts of glutamate causes neurons to die.
http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_amyotrophiclateralsclerosis.ht m

http://www.alsa.org/als/what.cfm