Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig's Disease)

Introduction

Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig's disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles. Alternate Names include Aran-Duchenne, Gehrig's Disease, Lou Gehrig's Disease, Motor Neuron Disease.

The U.S. Social Security Administration (SSA) has included Amyotrophic Lateral Sclerosis (ALS) as a Compassionate Allowance to expedite a disability claim.

Main Item

In ALS, the upper and lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away, and twitch. Eventually, the ability of the brain to start and control voluntary movement is lost.

Individuals with ALS lose their strength and the ability to move their arms, legs, and body. When muscles in the diaphragm and chest wall fail, individuals lose the ability to breathe without ventilatory support. The disease does not affect a person's ability to see, smell, taste, hear, or recognize touch and does not usually impair their thinking or other cognitive abilities. However, several recent studies suggest that a small percentage of patients may experience problems with memory or decision-making, and there is growing evidence that some may even develop a form of dementia.

Early Symptoms:

Males are affected more often than women, and the onset of ALS may be so subtle that the symptoms are frequently overlooked. The earliest symptoms of Lou Gehrig's Disease may include:

• Tripping and falling
• Hand weakness or clumsiness
• Weakness in your leg, feet, or ankles
• Slurred speech or trouble swallowing
• Difficulty walking or doing your normal daily activities
• Difficulty holding your head up or keeping good posture
• Muscle cramps and twitching in your arms, shoulders, and tongue
• Eventually, ALS weakens muscles, including muscles used for breathing, until they become paralyzed.

Cause(s)

The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others. There is no one test or procedure to establish the diagnosis of ALS.

The diagnosis of ALS is based on history, neurological findings consistent with the diagnosis of ALS, and electro-physiological and neuroimaging testing to rule out other impairments that may cause similar signs and symptoms. The diagnosis may be supported by electrophysiological studies (Electromyogram [EMG] and nerve conduction study [NCS], but these tests may be negative or only suggestive of the diagnosis.

Treatment(s)

No cure has yet been found for ALS. However, the FDA has approved the first drug treatment for the disease-riluzole (Rilutek).

Clinical trials with ALS patients showed that riluzole prolongs survival by several months. 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. However, this first disease-specific therapy offers hope that the progression of ALS may one day be slowed by new medications or combinations of drugs.

Other treatments for ALS are designed to relieve symptoms and improve the quality of life for patients.

• Multidisciplinary teams of health care professionals can design an individualized medical and physical therapy plan and provide special equipment to keep patients as mobile and comfortable as possible.
• Physicians can prescribe medications to help reduce fatigue, ease muscle cramps, control spasticity, and reduce excess saliva and phlegm.
• Drugs also are available to help patients with pain, depression, sleep disturbances, and constipation.

Regardless of the part of the body first affected by the disease, muscle weakness, and atrophy spread to other parts of the body as the disease progresses. Most individuals with ALS die from respiratory failure, usually within 3 to 5 years from the onset of symptoms.