Amyotrophic Lateral Sclerosis

Overview

Found in a mere 1-3 peopleper 100,000, amyotrophic lateral sclerosis (ALS) is a rare but deadly neurological disease that causes muscle weakness, disability and eventually death. Known also as the Lou Gehrig’s disease, after the baseball player who was diagnosed with it in 1939, the cause of this debilitating condition remains unknown in about 90 per cent of the cases. The remaining five to ten per cent are hereditary. Starting innocuously enough with twitching of muscles, weakness in an arm or leg or slurred speech, ALS is known to progress rapidly and eventually disable the nerve cells that control the muscles needed to move, speak, eat and breathe. Researchers are studying several possible causes of ALS, including: Gene mutation: Various genetic mutations can lead to inherited forms of ALS, which appear nearly identical to the non-inherited forms.Chemical imbalance: People with ALS typically have higher than normal levels of glutamate in their spinal fluid. Too much glutamate,a chemical messenger in the brain, is known to be toxic to some nerve cells.Disorganized immune response: Sometimes an immune system attacks the very body it is designed to defend, triggering a process that maylead to ALS.Protein mishandling: There’s evidence that when mishandled, proteins become abnormal and an accumulation of such abnormal proteins can kill the nerve cells. The disease does not affect a person’s ability to see, smell, taste, hear or recognize touch. However, several recent studies suggest that some people with ALS may develop cognitive problems involving word fluency, decision-making, and memory.

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