Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic Lateral Sclerosis (ALS) is a progressive disease of the nervous system. Motor neurons that control movement throughout the body are rapidly and progressively destroyed.

The onset of ALS usually occurs between age 40 and 70, and more men are diagnosed with ALS than women. The cause of ALS is not known. It can run in families, but most cases of ALS are random and can affect anyone.

ALS is always fatal, but medications and treatments can improve symptoms and prolong survival.

ALS is also known as “Lou Gehrig’s Disease” in memory of one of its most famous victims.

ALS destroys cells of the nervous system. Motor neurons transmit messages from your brain and spinal cord to your voluntary muscles throughout the body.

Usually, the first symptoms that people notice are mild muscle problems including:

• Trouble walking or running
• Tripping
• Dropping things
• Trouble writing
• Speech problems
• Uncontrollable episodes of laughing or crying

Eventually, people with ALS lose strength and cannot move. ALS only affects motor neurons, so it does not affect a person's ability to see, smell, taste, hear, or recognize touch, nor does it impair a person's mind or intelligence. Patients usually maintain control of eye muscles and bladder and bowel functions. Eventually, muscles in the chest fail, making breathing impossible. A breathing machine can help, but most people with ALS die from respiratory failure.

The cause of ALS is unknown. Genetics and environmental factors may contribute to the disease, and research is underway to confirm potential causes and contributing factors.

A very small portion of cases are a familial form of the disease that is inherited, but most cases occur at random and there are no identified risk factors for the disease.

ALS is difficult to diagnose. There is no single test or procedure to diagnoses ALS, but, instead, diagnosis involves a clinical examination and a series of diagnostic tests to rule out other diseases that mimic ALS. A diagnostic workup includes many procedures, such as electomyography (EMG) and nerve conduction velocity (NCV) tests, blood and urine studies, thyroid and parathyroid hormone levels, spinal tap, x-rays, magnetic resonance imaging (MRI), muscle and/or nerve biopsy, and a thorough neurological examination.

Several diseases have some of the same symptoms as ALS and most of these conditions are treatable. Therefore, if you are diagnosed with ALS, seek a second opinion from an ALS "expert" - someone who diagnoses and treats many ALS patients and has training in this medical specialty.

ALS is a progressive disease, so symptoms will gradually worsen. The sequence of emerging symptoms and the rate of disease progression vary from person to person. Difficulty swallowing and chewing impair the person’s ability to eat normally and increase the risk of choking. Maintaining weight becomes a problem for most patients with ALS.

Cognitive abilities remain relatively intact, so people with ALS are aware of their progressive loss of function and may become anxious and depressed. In later stages of the disease, individuals have difficulty breathing as the muscles of the respiratory system weaken. They eventually lose the ability to breathe on their own and must depend on ventilatory support for survival. Affected individuals also face an increased risk of pneumonia during later stages of ALS.

Physical therapy can improve an individual’s independence and safety. Gentle, low-impact aerobic exercise such as walking, swimming, and stationary bicycling can strengthen unaffected muscles, improve cardiovascular health, and help patients fight fatigue and depression. Range of motion and stretching exercises can help prevent painful spasticity and shortening (contracture) of muscles. Occupational therapists can suggest devices such as ramps, braces, walkers, and wheelchairs that help individuals conserve energy and remain mobile.

People with ALS who have difficulty speaking may benefit from working with a speech therapist. Patients can learn adaptive strategies to help them speak louder and more clearly. As ALS progresses, speech therapists can help people develop ways for responding to yes-or-no questions with their eyes or by other nonverbal means and can recommend aids such as speech synthesizers and computer-based communication systems. These methods and devices help people communicate when they can no longer speak or produce vocal sounds.

Nutritional support is an important part of the care of people with ALS and individuals and caregivers can learn how to plan and prepare healthful that provide enough energy and nutrients and how to avoid foods that are difficult to swallow.

ALS is always fatal, though some medications can be used to help reduce fatigue, ease muscle cramps, control spasticity, and reduce excess saliva and phlegm. Drugs can also help patients with pain, depression, sleep disturbances, and constipation.

Riluzole (Rilutek) is the only drug approved for the treatment of ALS. This drug reduces damage to motor neurons by decreasing the release of a chemical that transmits messages among nerve cells. Riluzole prolongs survival by several months, mainly in people who have difficulty swallowing, and it extends the time before an individual needs ventilation support. Riluzole does not reverse the damage already done to motor neurons, and patients taking the drug must be monitored for liver damage and other possible side effects.