Amyotrophic Lateral Sclerosis (ALS)


Amyotrophic Lateral Sclerosis, or ALS, is a progressive nervous system (neurological) disease that destroys nerve cells and causes disability.

ALS is often called Lou Gehrig's disease, after the famous baseball player who was diagnosed with it. ALS is a type of motor neuron disease in which nerve cells gradually break down and die.

Doctors usually don't know why ALS occurs. Some cases are inherited. ALS often begins with muscle twitching and weakness in a limb, or slurred speech. Eventually, ALS affects control of the muscles needed to move, speak, eat and breathe. There is no cure for ALS, and eventually the disease is fatal.

Symptoms

Early signs and symptoms of ALS include:

  • Difficulty walking or doing your normal daily activities

  • Tripping and falling

  • Weakness in your leg, feet or ankles

  • Hand weakness or clumsiness

  • Slurred speech or trouble swallowing

  • Muscle cramps and twitching in your arms, shoulders and tongue

  • Difficulty holding your head up or keeping good posture

ALS often starts in the hands, feet or limbs, and then spreads to other parts of your body. As the disease advances and nerve cells are destroyed, your muscles progressively weaken. This eventually affects chewing, swallowing, speaking and breathing.

ALS doesn't usually affect your bowel or bladder control, your senses or your thinking ability. It's possible to remain actively involved with your family and friends.

Causes

  • Gene mutation. Various genetic mutations can lead to inherited ALS, which causes nearly the same symptoms as the non-inherited form.

  • Chemical imbalance. People with ALS generally have higher than normal levels of glutamate, a chemical messenger in the brain, around the nerve cells in their spinal fluid. Too much glutamate is known to be toxic to some nerve cells.

  • Disorganized immune response. Sometimes a person's immune system begins attacking some of his or her body's own normal cells, which may lead to the death of nerve cells.

  • Protein mishandling. Mishandled proteins within the nerve cells may lead to a gradual accumulation of abnormal forms of these proteins in the cells, destroying the nerve cells.

Amyotrophic lateral sclerosis (ALS) is difficult to diagnose early because it may mimic several other neurological diseases.

Tests to rule out other conditions may include:

  • Electromyogram (EMG). During an EMG, your doctor inserts a needle electrode through your skin into various muscles. The test evaluates the electrical activity of your muscles when they contract and when they're at rest.

  • Abnormalities in muscles seen in an electromyogram can help doctors diagnose ALS, or determine if you have a different muscle or nerve condition that may be causing your symptoms. It can also help guide your exercise therapy.

  • Nerve conduction study. This study measures your nerves' ability to send impulses to muscles in different areas of your body. This test can determine if you have nerve damage or certain muscle diseases.

Therapies

  • Breathing care. You'll eventually have more difficulty breathing as your muscles become weaker. Doctors may test your breathing regularly and provide you with devices to assist your breathing at night.

  • You may choose mechanical ventilation to help you breathe. Doctors insert a tube in a surgically created hole at the front of your neck leading to your windpipe (tracheostomy), and the tube is connected to a respirator.

  • Physical therapy. A physical therapist can address pain, walking, mobility, bracing and equipment needs that help you stay independent. Practicing low-impact exercises may help maintain your cardiovascular fitness, muscle strength and range of motion for as long as possible.

  • A physical therapist can also help you adjust to a brace, walker or wheelchair and may suggest devices such as ramps that make it easier for you to get around.

    Regular exercise can also help improve your sense of well-being. Appropriate stretching can help prevent pain and help your muscles function at their best.

  • Occupational therapy. An occupational therapist can help you find ways to remain independent despite hand and arm weakness. Adaptive equipment can help you perform daily activities such as dressing, grooming, eating and bathing.

  • An occupational therapist can also help you modify your home to allow accessibility if you have trouble walking safely.

Occupational therapists also have a good understanding of how assistive technology and computers can be used, even if your hands are weak.

  • Speech therapy. Because ALS affects the muscles you use to speak, communication is an issue in advanced ALS. A speech therapist can teach you adaptive techniques to make your speech more clearly understood. Speech therapists can also help you explore other methods of communication, such as an alphabet board or simple pen and paper.

Ask your therapist about the possibility of borrowing or renting devices such as tablet computers with text-to-speech applications or computer-based equipment with synthesized speech that may help you communicate.


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