Amyotrophic lateral sclerosis

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Stephen Hawking, a physicist who has ALS.

Amyotrophic lateral sclerosis (ALS, sometimes called Lou Gehrig’s disease or motor neurone disease) is a chronic, progressive, almost always fatal neurological disease. ALS is marked by slow but steady death of the nerve cells in the central nervous system that control voluntary muscle movement. That is, people with ALS can no longer move, but their heart still beats. The disorder causes muscle weakness and muscle shrinking (atrophy) throughout the body. In ALS, both the upper motor neurons and the lower motor neurons die, making them stop sending messages to the muscles. Unable to function, the muscles gradually weaken and waste away (atrophy). Eventually, the ability of the brain to start and control voluntary movement is lost. However, even patients in later stages of the disease may still have the same intelligence, memory, and personality they had before it started.

Symptoms[change | change source]

ALS does not show many symptoms, making it very hard to diagnose. It usually affects people ages 40-60. The earliest symptoms may include twitching, cramping, or stiffness of muscles; muscle weakness affecting an arm or a leg; slurred and strange-sounding nasal speech; or having a difficult time chewing or swallowing. These general symptoms then develop into clear weakness or atrophy that may cause a physician to believe that a person has ALS.

The parts of the body affected by early symptoms of ALS depend on which muscles in the body are affected first. About 75% of people have limb onset ALS. In some of these cases, symptoms first affect one of the legs, and patients have awkwardness when walking or running or they notice that they are tripping or stumbling more often. Other limb onset patients first see the effects of the disease on a hand or arm as they have a difficult time with simple tasks requiring hand dexterity, or ability to move small things, such as buttoning a shirt, writing, or turning a key in a lock.

About 25% of cases are bulbar onset ALS. These patients first have difficulty speaking clearly. Speech becomes hard to understand and slurred. Speaking through the nose and softer talking are often the first symptoms. Difficulty swallowing, and loss of tongue movement follow. Eventually total loss of speech and ability to keep the airway clear when swallowing are experienced.

Treatment[change | change source]

No cure has been found for ALS. However, the Food and Drug Administration (FDA) has approved the first drug treatment for the disease: Riluzole (Rilutek). Riluzole is believed to make damage smaller to motor neurons by decreasing the release of glutamate, a substance that is found in most ALS patients. Clinical trials with ALS patients showed that riluzole gives a better chance of survival by several months, mainly in those with a hard time swallowing. The drug also allows more time before a patient needs breathing help. Riluzole does not heal the damage already done to motor neurons, and patients taking the drug must be watched for liver damage and other side-effects. However, this first ALS therapy offers hope that the assault of ALS may one day be slowed by new medications or combinations of drugs.

Other treatments for ALS are designed to make symptoms less painful and improve the quality of life for patients. This care is best given by teams of health care professionals such as physicians; pharmacists; physical, occupational, and speech therapists; nutritionists; social workers; and home care and hospice nurses. Working with patients and people who take care of the patient, these teams can make a personalized plan of medical and physical therapy and give special equipment that can be used to keep patients as mobile and comfortable as possible.