Healthy Habits for Bonsall & Fallbrook Folks: Lou Gehrig's disease: Death of the neurons

Special to Village News

Motor neuron disease referred to as amyotrophic lateral sclerosis, also called Lou Gehrig's disease, causes the death of the neurons that control the muscle's voluntary functioning. Lou Gehrig was a famous baseball player who was diagnosed with ALS. Overtime, the nerve cells progressively break down and die. At first onset, muscle twitching might start to occur coupled with weakness in the limbs and slurred speech. Eventually, a person will no longer be able to control their ability to move, speak, breathe or eat. The disease is fatal, and there is currently no cure.

This condition starts to affect the hands and feet first. It will soon spread to other parts of the body, taking over almost everywhere possible. Once full-fledged, a person isn't even able to chew their own food. The only function they can still control is the use of the bathroom. Other side effects include tripping and falling, difficulty holding the head up and keeping good posture. The muscles in the legs become stiff and twitch. There is an overall feeling of fatigue all the time. A person's senses of hearing, vision and touch are no longer the same. ALS patients also have a hard time controlling their emotions.

Another side effect is the inability to control breathing. When this happens, a breathing device similar to those used for sleep apnea may need to be used. Some people opt for surgery, having their tracheostomy surgically reformatted so that there is a whole in the neck leading to the windpipe. Different communication devices and technologies may need to be used once speaking becomes too difficult. Many persons with ALS also develop dementia and are unable to remember or make decisions. When eating becomes too difficult, a feeding tube might need to be inserted. Otherwise malnutrition and dehydration can occur.

There is no known cause for ALS, but it is an inherited disease in five out of 10 cases. Researchers are trying to discover if there are any possible gene mutations, chemical imbalances, immune responses or mishandled proteins that could cause the disease. Some evidence suggests that exposure to environmental toxins might be linked to ALS. In persons with ALS, their children have a 50-50 chance of developing the disease. It is most common in ages 40 to 60.

New research suggested that the gene called membralin plays a key role in ALS causality. It provides a new perspective for therapeutic treatment. Membralin was also discovered to be linked to Alzheimer's disease. Studies have been performed on mice which lack the membralin protein in their brain cells. The mice had clear muscle impairments that mirrored ALS symptoms in humans. When membralin is deficient, the neurotransmitter called glutamate starts to accumulate. This excess glutamate starts to kill neurons, which entails the pathogenesis of ALS. New treatment approaches are therefore being undertaken to be able to boost levels of membralin in humans.

Currently, those diagnosed with ALS have not experienced easy or clear answers to help with their condition. Their lives begin to revolve around a team of doctors and specialists that might include a neurologist, psychiatrist, nutritionist, occupational therapist and a respiratory therapist. Some patients have benefited from joining a community of people who live with ALS for a support system. The ALS Association advocated and aims to empower those affected by the disease. This nonprofit organization was started in 1985 and believes the world is its lab to find a treatment for ALS. Most people only live three to five years after diagnosis.