Lou Gehrig's Disease (ALS)

Signs and Symptoms

At first, symptoms of ALS may be barely noticed. But over time, the disease worsens. As nerve cells die, the muscles they control stop acting and reacting correctly.

• Muscles in the arms, shoulders and tongue may cramp or twitch.
• One or more limbs may lose strength and coordination.
• Feet and ankles may become weak, so walking may become clumsy or stiff, and feet may drag.
• Swallowing, speaking or breathing may become difficult.
• You may become fatigued.

Eventually, ALS weakens muscles, including muscles used for breathing, until they become paralyzed. Unable to swallow, patients with ALS may aspirate or inhale food or saliva into their lungs. In fact, most people with ALS die of respiratory failure. The ability to think, see, hear, smell, taste and touch, however, usually is not affected.

Diagnosis

To determine if you have ALS, your doctor will perform an evaluation that includes a physical exam, medical history and lab tests such as blood and urine tests. Electromyography (EMG) may also be used. This test is very sensitive in detecting lower motor neuron disease. There are no standard laboratory tests for upper motor neuron disease. But certain signs may be interpreted by your doctor as signs of ALS such as spasticity, a specific type of stiffness; abnormally brisk tendon reflexes; Babinski's sign, an unusual flexing of the toes during examination; and diminished fine motor coordination.

Treatment

There is no known cure for ALS. The drug riluzole has been approved for treatment and may slow progression of the disease but it is expensive and only modestly effective. Generally, treatment is designed to help control symptoms.

Drugs such as baclofen or diazepam may help control spasticity. Trihexyphenidyl or amitriptyline may help patients swallow saliva. Gabapentin may be prescribed to help control pain. Because choking is common as ALS progresses, patients may need a tube called a percutaneous endoscopic gastrostomy (PEG) tube or feeding tube to be placed into the stomach for feeding. A referral to an otolaryngologist, an ear, nose and throat specialist, may be advised.

Physical therapy, rehabilitation, use of appliances such as braces or a wheelchair or other treatments may help muscles work as well as possible and improve your general health.

ALS patients gradually lose the ability to function and care for themselves. They may survive from two to 10 years after the onset of the disease, with about 20 percent of ALS patients living more than five years after diagnosis.