Definition

What is amyotrophic lateral sclerosis?

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. 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.

How common is amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis can affect patients at any age. It can be managed by reducing your risk factors. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of amyotrophic lateral sclerosis?

The common symptoms of amyotrophic lateral sclerosis are:

  • 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.

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes amyotrophic lateral sclerosis?

ALS is inherited in 5 to 10 percent of cases, while the rest have no known cause. Researchers are studying several possible causes of ALS, including:

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

Risk factors

What increases my risk for amyotrophic lateral sclerosis?

Established risk factors for ALS include:

  • Heredity. Five to 10 percent of the people with ALS inherited it (familial ALS). In most people with familial ALS, their children have a 50-50 chance of developing the disease.
  • Age. ALS risk increases with age, and is most common between the ages of 40 and 60.
  • Sex. Before the age of 65, slightly more men than women develop ALS. This sex difference disappears after age 70.
  • Genetics. Some studies examining the entire human genome (genomewide association studies) found many similarities in the genetic variations of people with familial ALS and some people with non-inherited ALS. These genetic variations might make people more susceptible to ALS.

Environmental factors may trigger ALS. Some that may affect ALS risk include:

  • Smoking. Smoking is the only likely environmental risk factor for ALS. The risk seems to be greatest for women, particularly after menopause.
  • Environmental toxin exposure.
  • Military service. Recent studies indicate that people who have served in the military are at higher risk of ALS. It’s unclear exactly what about military service may trigger the development of ALS. It may include exposure to certain metals or chemicals, traumatic injuries, viral infections, and intense exertion.

Diagnosis & Treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

 

How is amyotrophic lateral sclerosis diagnosed?

Amyotrophic lateral sclerosis 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.
  • Magnetic resonance imaging (MRI). Using radio waves and a powerful magnetic field, an MRI produces detailed images of your brain and spinal cord. An MRI can spot spinal cord tumors, herniated disks in your neck or other conditions that may be causing your symptoms.
  • Blood and urine tests. Analyzing samples of your blood and urine in the laboratory may help your doctor eliminate other possible causes of your signs and symptoms.
  • Spinal tap (lumbar puncture). Sometimes a specialist may remove a sample of your spinal fluid for analysis. A specialist inserts a small needle between two vertebrae in your lower back and removes a small amount of cerebrospinal fluid for testing in the laboratory.
  • Muscle biopsy. If your doctor believes you may have a muscle disease rather than ALS, you may undergo a muscle biopsy. While you’re under local anesthesia, a small portion of your muscle is removed and sent to a lab for analysis.

How is amyotrophic lateral sclerosis treated?

Treatments can’t reverse the damage of amyotrophic lateral sclerosis, but they can slow the progression of symptoms, prevent complications and make you more comfortable and independent.

Medications

The drug riluzole (Rilutek) is the only medication approved by the Food and Drug Administration for ALS. This drug appears to slow the disease’s progression in some people, perhaps by reducing levels of a chemical messenger in the brain (glutamate) that’s often present in higher levels in people with ALS. Riluzole may cause side effects such as dizziness, gastrointestinal conditions and liver function changes. Your doctor may also prescribe medications to provide relief from other symptoms, including:

  • Muscle cramps and spasms
  • Spasticity
  • Constipation
  • Fatigue
  • Excessive salivation
  • Excessive phlegm
  • Pain
  • Depression
  • Sleep problems
  • Uncontrolled outbursts of laughing or crying

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.
  • 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.
  • 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.
  • Nutritional support. Your team will work with you and your family members to ensure you’re eating foods that are easier to swallow and meet your nutritional needs. You may eventually need a feeding tube.
  • Psychological and social support. Your team may include a social worker to help with financial issues, insurance, and getting equipment and paying for devices you may need. Psychologists, social workers and others may provide emotional support for you and your family.

Lifestyle changes & Home remedies

What are some lifestyle changes or home remedies that can help me manage amyotrophic lateral sclerosis?

The following lifestyles and home remedies might help you cope with amyotrophic lateral sclerosis:

  • Take time to grieve.
  • Be hopeful. Some people with amyotrophic lateral sclerosis live much longer than the three to five years usually associated with this condition. Maintaining an optimistic outlook can help improve quality of life for people with ALS.
  • Think beyond the physical changes. Many people with amyotrophic lateral sclerosis lead rich, rewarding lives despite physical limitations.
  • Join a support group. You may find comfort in a support group with others who have ALS. Your family members and friends helping with your care also may benefit from a support group of other ALS caregivers.
  • Make decisions now about your future medical care. Planning for the future allows you to be in control of decisions about your life and your care.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Review Date: April 22, 2017 | Last Modified: April 22, 2017

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