Amyotrophic lateral sclerosis (ALS), more commonly known as Lou Gehrig's disease (in the US) or motor neuron disease (in the UK), is a neurodegenerative disease, attacking the nerves in the brain and spinal cord and resulting in loss of voluntary muscle control. Over time, ALS causes a complete loss of voluntary movement, which leads to respiratory failure and death.
At Memphis Neurology, our team of board-certified neurologists diagnoses and treats ALS patients at our two convenient locations in Germantown, Tennessee, and Southaven, Mississippi. While there’s currently no cure for the disease, we can help you manage your ALS symptoms to give you the best life possible.
“Motor neurons” travel from the brain through either the brainstem or spinal cord to the muscles that control movement in the arms, legs, throat, mouth, and chest. People who have ALS experience a cell die off in these neurons, and without stimulation, the muscle tissues waste away. ALS, however, doesn’t affect a person's sensory functions or ability to think and reason, meaning they’re fully aware of the disease’s progression, and their minds can become trapped inside of non-functioning bodies.
ALS is usually categorized in one of two ways: Upper motor neuron disease affects nerves within the brain, while lower motor neuron disease affects nerves originating in the spinal cord or brainstem. In both cases, the neurons degrade, and eventually the patient dies of the disease. While the average life expectancy after diagnosis is 2-5 years, some patients can live for years or even decades. The most famous case of longevity was theoretical physicist Stephen Hawking, who lived for more than 50 years after diagnosis.
In the US, about 60% of ALS patients are men, and 93% are Caucasian. That means just over 5,600 people are diagnosed with ALS each year, and as many as 30,000 have the disease at any given time.
Most people develop ALS between 40-70 years old. However, in rare cases, patients can develop the disease in their 20s and 30s.
Nearly all ALS cases are considered sporadic, meaning the disease seems to strike at random, with no clear risk factors and no family history of the disease.
About 5-10% of all ALS cases are familial, which means the patient inherits it from a parent; only one parent needs to carry the disease-causing gene. Researchers have determined that mutations in more than a dozen genes can cause familial ALS. Mutations that cause changes in the processing of RNA molecules, which are involved with protein production and gene activity, are one involved process.
Other mutations suggest there are defects in protein recycling, whereby malfunctioning proteins are broken down and used to build new, correctly working ones; and still others indicate there’s an increased susceptibility in these genes to environmental toxins.
ALS onset can be very subtle, but eventually symptoms develop into obvious weakness in the muscles, and eventually, atrophy.
Early symptoms include:
How the sequence emerges, as well as the rate of progression, varies from one individual to the next. However, all will eventually be unable to stand, walk, or be able to perform activities of daily living.
In addition, difficulty swallowing and chewing food makes it hard to eat. That, combined with the fact that ALS patients burn calories at a faster rate than those without, can lead to rapid weight loss and malnutrition. Eventually, the diaphragm and breathing muscles will become paralyzed, necessitating a ventilator or leading to death.
While there’s no cure for ALS, you can learn to manage your symptoms with various therapies, including:
Many people with ALS, as well as other neuromuscular diseases, opt to take part in clinical research studies investigating new medications and treatments aimed at treating the disease. While personal benefit is low, the potential to help future patients is invaluable.
If you’ve been diagnosed with ALS, or you’re experiencing any of the symptoms, you need to come into Memphis Neurology for an evaluation and treatment plan to help you manage the disease. Give us a call at either of our locations, or book online with us today.