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Dementia in Amyotrophic Lateral Sclerosis (ALS)

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Dementia in ALS Overview

Amyotrophic lateral sclerosis (ALS) is a devastating disease that affects the part of the nervous system that controls voluntary movements. ALS is sometimes called Lou Gehrig's disease, after the famous baseball player who died of the disease. The muscles become progressively weaker, eventually leading to paralysis and death.
 

ALS is one of a group of diseases known as motor neuron diseases. Neurons are nerve cells, and motor neurons control movement. Persons with motor neuron disease gradually lose muscle control and become paralyzed. No cure is available for ALS or any other motor neuron disease. The effects of these diseases are not reversible. Most people with ALS die within five years of the onset of symptoms.
 

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Most experts believe that ALS does not affect a person’s mental processes. In most people, neither cognitive processes (such as thinking, learning, memory, and comprehension) nor behavior is affected. However, researchers have increasingly begun to recognize that in some cases, people with ALS can experience cognitive changes that are severe enough to be called dementias.  Dementias are a type of severe brain disorder that interferes with a person’s ability to carry out everyday activities.  Research on the relationship between ALS and dementias is ongoing.

The relationship between dementia and ALS is not well understood, but is thought to be reflect damage to cells in the frontal and temporal lobe of the brain. The frontal lobe is the part of the brain from the forehead back to the ears, while the temporal lobe is found on the sides of the brain that correspond to the temples. 

While dementia can occur in some ALS patients, it is uncertain whether ALS is its cause, or a concurring problem.  Some researchers believe that there may be a common origin to explain why a subset of people with ALS have cognitive symptoms that are similar to that seen in fronto-temporal dementia. However, the cause or causes of dementia in people with ALS is yet to be pinpointed.

Dementia is rare in ALS, but when it occurs, it crosses ethnic and gender boundaries. People ages 55 to 65 are most likely to be affected.

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