Overview
Amyotrophic lateral sclerosis, commonly called “Lou Gehrig’s Disease” is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells or motor neurons responsible for controlling voluntary muscles. The disease belongs to a group of disorders known as “motor neuron diseases,” which are characterized by the gradual degeneration and death of motor neurons.
ALS causes weakness with a wide range of disabilities. Eventually, all muscles under voluntary control are affected, and patients lose their strength and the ability to move their arms, legs, and body. When muscles in the diaphragm and chest wall fail, patients lose the ability to breathe without ventilation support.
Most people with ALS die from respiratory failure, usually within 3 to 5 years from the onset of symptoms. However, about 10 percent of ALS patients survive for 10 or more years. Although the disease usually does not impair a person’s mind or intelligence, several recent studies suggest that some ALS patients may have alterations in cognitive functions such as depression and problems with decision-making and memory.
ALS does not affect a person’s ability to see, smell, taste, hear, or recognize touch. Patients usually maintain control of eye muscles and bladder and bowel functions, although in the late stages of the disease most patients will need help getting to and from the bathroom.
According to their webpage, the Department of Veterans Affairs presumes amyotrophic lateral sclerosis (ALS) diagnosed in all Veterans who had 90 days or more continuous active military service is related to their service.
ALS Types
Sporadic — Nearly all cases of ALS are considered sporadic, meaning the disorder seems to happen at random with no clearly associated risk factors and no family history. Although family members of people with ALS are at an increased risk for the disorder the overall risk is very low—most won’t develop ALS.
Familial — About 10% of all ALS cases are familial (also called inherited or genetic). Mutations (changes) in more than a dozen genes have been found to cause familial ALS.
ALS Signs and Symptoms
Symptoms of ALS vary from person to person and depend on which nerve cells are affected. ALS generally begins with muscle weakness that spreads and gets worse over time.
Early symptoms include:
- Muscle twitches in the arm, leg, shoulder, or tongue
- Muscle cramps
- Tight and stiff muscles (spasticity)
- Muscle weakness affecting an arm, a leg, or the neck
- Slurred and nasal speech
- Difficulty chewing or swallowing
As the disorder progresses, muscle weakness and atrophy spread to other parts of the body. People with ALS may develop problems with:
- Chewing food and swallowing (dysphagia)
- Drooling (sialorrhea)
- Speaking or forming words (dysarthria)
- Breathing (dyspnea)
- Unintended crying, laughing, or other emotional displays (pseudobulbar symptoms)
- Constipation
- Maintaining a healthy weight and getting enough nutrients
ALS Risk Factors
Established risk factors for ALS include:
- Genetics — For about 10% of people with ALS, a risk gene was passed down from a family member. This is called hereditary ALS. In most people with hereditary ALS, their children have a 50% chance of inheriting the gene.
- Age — Risk increases with age up to age 75. ALS is most common between the ages of 60 and the mid-80s.
- Sex — Before the age of 65, slightly more men than women develop ALS. This sex difference disappears after age 70.
Environmental factors, such as the following, have been associated with an increased risk of ALS.
- Smoking — Evidence supports that smoking is an environmental risk factor for ALS. Women who smoke seem to be at even higher risk, particularly after menopause.
- Environmental toxin exposure — Some evidence suggests that exposure to lead or other substances in the workplace or at home might be linked to ALS. Much study has been done, but no one agent or chemical has been consistently associated with ALS.
- Military service — Studies indicate that people who have served in the military are at higher risk of ALS. It’s not clear what about military service might trigger ALS. It might include exposure to certain metals or chemicals, traumatic injuries, viral infections, or intense exertion.
ALS Treatment
The Food and Drug Administration has approved two medicines for treating ALS:
- Riluzole (Rilutek, Exservan, Tiglutik) — Taken by mouth, this medicine can increase life expectancy by about 25%. It can cause side effects such as dizziness, gastrointestinal conditions and liver problems. Your health care provider typically monitors your liver function with periodic blood draws while you’re taking the medicine.
- Edaravone (Radicava) — This medicine may reduce the speed of decline in daily functioning. It’s given through a vein in your arm or by mouth as a liquid. The effect on life span isn’t yet known. Side effects can include bruising, headache and trouble walking. This medicine is given daily for two weeks each month.
Your health care provider also might prescribe treatments for symptoms relief, including:
- Muscle cramps and spasms
- Constipation
- Fatigue
- Excessive saliva and phlegm
- Pain
- Depression
- Sleep problems
- Uncontrolled outbursts of laughing or crying
- An urgent need to urinate
- Leg swelling
Therapies — When ALS affects your ability to breathe, speak and move, therapies and other forms of support can help, including:
- Breathing care
- Physical therapy
- Occupational therapy
- Speech therapy
- Nutritional support
- Psychological and social support
Other Resources and Support
https://www.publichealth.va.gov/exposures/radiation/diseases.asp
https://www.ninds.nih.gov/health-information/disorders/amyotrophic-lateral-sclerosis-als
https://www.ninds.nih.gov/health-information/disorders/motor-neuron-diseases