Amyotrophic lateral sclerosis (ALS) is a disease of the nerve cells in the brain, brain stem and spinal cord that control voluntary muscle movement.
In ALS, motor nerve cells (neurons) waste away or die, and can no longer send messages to muscles. This eventually leads to muscle weakening, twitching and an inability to move the arms, legs and body. The condition slowly gets worse over time. When the muscles in the chest area stop working, it becomes hard or impossible to breathe.
There are 2 different types of ALS.
- Sporadic ALS (SALS), the most common form, occurs in people without a family history of ALS.
- Familial ALS (FALS) occurs in 5 to 10 percent of cases and there is usually a clear family history of the disease.
Symptoms usually do not develop until after age 50 but they can start in younger people. ALS symptoms usually start with painless weakness developing in a hand or foot and can be mistaken for more common problems, such as carpal tunnel syndrome or a pinched nerve. The muscle weakness slowly gets worse. Other symptoms and signs include:
- Muscle stiffness and wasting
- Muscle cramps and twitching
- Slurred speech or swallowing problems
- Behavior changes which can interfere with decision-making
- Becoming more emotional - crying and laughing more easily
ALS mostly affects upper and lower motor neurons, which are the nerve cells that control movements of the arms, legs, face, diaphragm, throat and tongue.
Automatic movements (such as the gut moving food and the heart beating), sensation and eye movements are generally not affected. ALS usually has minimal impact on a person’s intellect and bowel/bladder control. ALS is usually not painful.
The cause of ALS is unknown and most scientists believe that a complex interaction of genetics and environmental factors cause sporadic ALS. There is no evidence that any particular toxin, occupational exposure or nutritional deficiency causes ALS.
Who is at Risk?
In the United States, there are approximately 5,000 people diagnosed each year with ALS and 30,000 people with the disease at any given time.
Most people afflicted with ALS are between the ages of 40 and 70, but it can affect younger adults and the elderly. Men are affected slightly more frequently than women.
Having a family member who has a hereditary form of the disease is a risk factor of ALS. Other risk factors include military service.
The average life expectancy from the beginning of symptoms is around 3 years, but about 25 percent of patients live 5 years and 10 percent will survive 10 or more years.
There is no single test used to establish a diagnosis of ALS. Your physician will perform a physical exam and ask about your medical history and symptoms.
You may be referred to a neurologist. He or she will perform a neurological exam to test muscle strength, reflexes, sensation, walking, speech, swallowing and other functions. Certain abnormalities are frequently found in patients with ALS:
- Brisk reflexes
- Stiff arms and legs
- Uninhibited laughing or crying
- Wasting of muscles
- Muscle twitching
Other tests include:
- Electromyography (EMG) involves a needle being placed into a muscle to evaluate its function.
- In nerve conduction studies, integrity of nerves is examined through electrical stimulation.
- Blood is drawn and evaluated to eliminate other diseases which mimic ALS.
- Magnetic Resonance Imaging (MRI) images the brain or spinal cord.
There is no known cure for ALS. Riluzole is the only FDA-approved medication for ALS, but its effect is quite modest. It slows the progression of the disease slightly and is generally safe with tolerable side effects.
Other medications can be prescribed, and suggested home remedies can minimize ALS symptoms such as muscle cramps, spasticity (stiffness), excess saliva, behavior changes, depression, constipation, insomnia, anxiety and weight loss.
Wake Forest Baptist ALS Clinic
The ALS Clinic aims to extend longevity, maximize independence and improve quality of life by offering specialized services to patients diagnosed with ALS. Established in March 1998, the ALS Clinic is an ALS Association certified Center of Excellence - one of only 33 such centers in the country. Patients receive care from a multidisciplinary team of ALS specialists which research has shown results in a longer lifespan and a higher quality of life.