Asked by Sandee Hengst, Las Vegas, Nevada
Is there a cure or treatment for ALS?
Dr. Otis Brawley
Chief Medical Officer,
American Cancer Society
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive, neurodegenerative disorder that causes muscle weakness. It is incurable and eventually fatal. It occurs at a very low incidence, about one to three cases per 100,000 people. While it is seen among young adults, it is most common among people in their 60s and 70s. Ten percent of cases that occur are familial or inherited. The cause is unknown in 90 percent.
The disease is primarily a deterioration of the upper motor neurons of the brain and the lower motor neurons of the spine. Motor neurons are the nerves that give out the signals for muscles to contract and move. ALS most commonly presents with weakness of an arm or leg. The examiner may find other symptoms such as exaggerated deep tendon reflexes (hyper-reflexia), and spasms. There is also eventual loss of muscle mass (atrophy) and small muscle twitching (fasciculations).
When a patient's initial symptoms are hand weakness, they may notice that they are dropping things and have difficulty performing fine motor tasks such as pinching, writing, typing, managing buttons or zippers and picking up small objects such as coins and paper clips. Patients with shoulder weakness may have trouble using their arms above the head in activities such as combing their hair or lifting things.
Patients with lower extremity weakness most often notice a foot drop. They have a "slapping" gait and are prone to trip. Some with weakness in the thigh and hip will have difficulty climbing stairs and rising from chairs.
About one in five are initially diagnosed because they have difficulty swallowing (dysphagia) or slurred speech (dysarthria). These symptoms are due to weakness of muscles in throat and mouth.
As the disease progresses, weakness spreads to other parts of the body. Difficulty breathing can lead to the need for mechanical ventilation. Infection, especially pneumonia, is a common problem with end stage ALS. The median survival from the time of diagnosis is three to five years. About 10 percent of ALS patients can live 10 years or more. Survival beyond 20 years is rare.
A number of drugs have been tested for this disease and several are in clinical trials. Neurology departments at medical schools are the best place to find these trials. Riluzole is the only drug that has impact on survival. It does slow ALS progression but only marginally. Those who are most likely to benefit from riluzole have had symptoms for less than five years and have very little respiratory impairment.
There are a number of diseases that can be mistaken for ALS. Among them are cervical disk disease (cervical radiculomyelopathy) post-polio syndrome, inflammatory myopathy, myasthenia gravis and hyperthyroidism. Physicians considering the diagnosis of ALS work hard to exclude those diseases that have very effective treatments.
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