Amyotrophic lateral sclerosis (ALS) is a progressive nervous system disorder. It gradually destroys the nerves responsible for muscle movement. Over time, ALS leads to total paralysis of muscle movement, including respiration.
Prognosis is poor in most cases because of the progressive nature of the condition due to eventual respiratory failure. After diagnosis is made, lifespan ranges from 2-5 years. The 5 year survival rate is 25% and up to 10% of patients will survive more than 10 years. In general, the younger the age of onset, the slower the disease progresses.
The cause of ALS is not known, but it appears that genes may play a role in a very small number of cases. Also, research has shown that there may be a reduced response to cell stress, as well as cell toxicity, related to certain protein build-up in the brain.
A risk factor is something that increases your chance of getting a disease or condition.
Risk factors include:
Having a family member with ALS
Military personnel may have an increased risk
Persons with certain genetic mutations
Progressive weakness in arms and legs (at first often on only one side) over weeks to months without changes in sensory abilities.
Initial presentation may be a wrist or foot drop
Trouble holding things without dropping them
Frequent tripping while walking
Unpredictable changing emotions
Trouble chewing and swallowing, resulting in frequent choking and gagging
Weight loss due to trouble eating
Excess salivation, drooling
Cognition is intact
Sensation is intact
Trouble coughing, resulting in development of
The doctor will ask about your symptoms and medical history, and perform a physical exam. There are no tests that definitively diagnose ALS, but tests may be used to rule out other medical conditions.
For you and your family, a multidisciplinary approach may work best. This approach may include:
Working with therapists and joining a support group
Participating in religious and social activities
Treatments options include:
The drug, called
, has been approved for ALS with a clinical trial revealing a modest lengthening of survival. The drug may slightly improve functioning, but it doesn't stop the disease from progressing.
A study, though, showed that the addition of
(a medication used to treat mood disorders) to riluzole may slow the progression of ALS and prolong survival.
Other drugs are also being studied.
Your doctor may prescribe these medications for symptom
Physical therapy—This is used to reduce pain associated with muscle cramping and spasticity
Respiratory care—In some cases, you may need to receive a mixture of air and oxygen from a machine. If you cannot move enough air in and out of your lungs, you may need
to have a tube inserted into your airway.
Nutritional care—Your doctor may make changes to your diet. In some cases, getting nutrition through
Speech therapy—Speech therapy may be used to optimize communication. Therapy can include exploring alternative methods of communication.
There are no guidelines for preventing ALS because the cause is unknown.
Please be aware that this information is provided to supplement the care
provided by your physician. It is neither intended nor implied to be a
substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER
IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the
advice of your physician or other qualified health provider prior to
starting any new treatment or with any questions you may have regarding a