Also known as Lou Gehrig's Disease or Motor Neuron Disease
by Rosalyn Carson-DeWitt, MD
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 Nervous System
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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
- Progressive weakness in arms and legs (at first often on only one side)
- Initial presentation may be a wrist or foot drop
- Trouble holding things without dropping them
- Frequent tripping while walking
- Shrunken muscles
- Twitchy muscles
- Unpredictable changing emotions
- Overactive reflexes
- Slurred speech
- Trouble chewing and swallowing, resulting in frequent choking and gagging
- Weight loss due to trouble eating
- Trouble breathing
- Excess salivation, drooling
- Cognition is intact
- Sensation is intact
- Trouble coughing, resulting in development of pneumonia
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.
Tests may include:
- Electromyogram (EMG) -to look for progressive muscle weakness and twitching
- CT Scan -a type of x-ray that uses a computer to make pictures of the structures inside the head
- MRI Scan -a test that uses magnetic waves to make pictures of the structures inside the head
- Blood Tests-to rule out metablic, heavy metal exposure, or rarely infections such as Lyme or HIV disease
- Lumbar Puncture-a procedure to collect cerebrospinal fluid (CSF)
- Urine Tests
There is currently no cure for ALS. The drug, called riluzole, has been approved for ALS with a clinical trial revealing a modest lengthening of survival. It may slightly improve functioning for ALS patients, although it doesn't stop disease progression. Other drugs are also being studied.
Treatments for the symptoms of ALS include:
Valium, Baclofen, or Dantrolene
Valium, Baclofen, or Dantrolene are used to reduce spasticity.
Nonsteroidal Anti-inflammatory Drugs (Such as Ibuprofen) and Other Pain Medications
These drugs are used to reduce pain from muscle cramping.
Physical therapy is used to reduce pain associated with muscle cramping and spasticity.
Atropine, Scopolamine, or an Antihistamine
These drugs are used to reduce heavy drooling.
Respiratory care with non-invasive positive pressure ventilation, with eventual trachea intubation may be used.
Nutritional care with dietary changes, with eventual feeding tube placement may be used.
Speech therapy may be used to optimize communication, including exploration of non-vocal options once speech is lost.
Antidepressants, Anxiolytic Drugs, and Psychotherapy
These drugs are used to treat depression and anxiety .
A multidisciplinary approach must be taken for not only the patient, but the patient's family. This may include medications and psychological, social, religious, and ALS support groups.
There are no guidelines for preventing ALS because the cause is not known.
American Academy of Neurology
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Miller RG, Mitchell JD, Lyon M, et al. Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). The Cochrane Library . Issue 3. Chichester, UK: John Wiley & Sons, Ltd.; 2005.
National Institute of Neurological Disorders and Stroke website. Available at www.ninds.nih.gov
Accessed October 12, 2005.
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