What Is Lou Gehrig's Disease (ALS)?
Amyotrophic lateral sclerosis (ALS) — commonly known as Lou Gehrig's disease — is a progressive, degenerative motor neuron disease of the nervous system. Motor neurons are the nerve cells that send messages to your muscles to function.
Over time, the muscles that control movement, speech, swallowing, and breathing progressively weaken and eventually become paralyzed.
Types of ALS
There are two types of ALS: sporadic and familial.
- Sporadic ALS. In about 90 percent of ALS cases, there is no family history of the disease.
- Familial ALS. Some 10 percent of people with ALS inherited a genetic mutation that causes the disease.
Signs & Symptoms of ALS
The signs and symptoms of ALS may vary depending on how far the disease has progressed.
Early ALS symptoms
- Muscle twitches and stiffness
- Loss of control of the hands and arms
- Dropping objects
- Slurred speech
Advanced ALS symptoms
- Challenges with movement
- Difficulty swallowing
- Labored speech
- Difficulty breathing
The onset of ALS symptoms may differ between men and women. Men most often develop ALS, which affects the spinal region, while in women, the disease more often starts in a part of the brain called the bulbar region, which controls muscles in the neck and face. Women may experience worse speech and swallowing symptoms due to the role of the bulbar region. Men are more likely to first experience problems with their hands and feet.
What Causes ALS?
In most cases, the cause of ALS is unknown. In other cases, causes may include:
- Family history. In people with familial ALS, the disease is caused by an inherited mutation in a dominant gene, meaning the person only needs to inherit it from one parent to develop symptoms.
- New genetic mutations. People with sporadic ALS may display spontaneous genetic mutations that caused their ALS, which are not hereditary.
- Smoking. Some studies suggest that smoking may raise ALS risk, particularly in women after menopause.
Risk Factors for ALS
While no single risk factor has been identified for ALS, studies suggest the following factors raise ALS risk:
- Genetics. A family history of ALS increases the chance that someone may develop the disease.
- Age. While ALS can affect anyone of any age, symptoms commonly begin between 55 and 75.
- Gender. Men are slightly more likely than women to develop ALS, but this difference diminishes with older age.
- Race and ethnicity. Non-Hispanic white individuals are more likely to develop ALS.
- Military service. Research suggests that military veterans are about 1.5 to 2 times more likely to develop ALS. Although the reason for this is unclear, possible risk factors for veterans include exposure to lead, pesticides, and other environmental toxins.
As the disease progresses, people with ALS frequently develop debilitating complications, such as:
- Labored breathing. Some people use a bilevel positive airway pressure (BiPAP) device to support breathing while sleeping at night. As the respiratory muscles become paralyzed, many people may eventually require a ventilator when they cannot breathe on their own.
- Speaking difficulties. Patients may rely on communication technologies if their speech becomes too difficult for others to understand.
- Eating problems and malnourishment. Difficulty with chewing and swallowing can lead to weight loss and dehydration. People with ALS are also more prone to aspirating saliva, liquids, or food into the lungs, which can cause pneumonia. Many individuals eventually get a feeding tube to reduce these risks and promote their wellbeing.
- Dementia and other cognitive challenges. A small percentage of people with ALS may develop problems with decision-making and language, and some of them develop a form of dementia called frontotemporal dementia — a group of disorders that mainly affect the frontal and temporal lobes of the brain, which are associated with behavior, personality, and language.
Trust NewYork-Presbyterian for ALS Care
The Eleanor and Lou Gehrig MDA/ALS Multidisciplinary Care Center at NewYork-Presbyterian/Columbia University Irving Medical Center is one of the nation’s most extensive comprehensive care programs for the diagnosis and treatment of patients with ALS and their families.
Our dedicated ALS experts offer state-of-the-art neurological evaluation, promote optimal functioning, support independence, and help maintain quality of life throughout the course of each patient’s disease. Call us to make an appointment for a consultation.