Amyotrophic Lateral Sclerosis, or ALS, is a devastating neurodegenerative disease. Bringing it to the world's attention can get it deserved recognition and support.
What is ALS?
Amyotrophic Lateral Sclerosis, also known as ALS or Lou Gehrig's disease, is a neurodegenerative disease that hinders and then eventually destroys the communication between nerves and voluntary muscles. The meaning of "amyotrophic" is "no muscle nourishment." Motor neurons, which direct muscle movement from the brain to the muscles via the spinal cord, degenerate and eventually die. In those with ALS, this causes lack of communication from the brain to the voluntary muscles, rendering them useless. Voluntary muscles in the body do not include the heart and those in the digestive system (automatic), but they do include those within the limbs, the torso, and the face. They also include those involved with breathing.
Demographics
- Approximately 60% of those with ALS are men
- 93% affected are Caucasian
- Approximately 3,600 new cases of ALS are diagnosed in the US annually
- Roughly 30,000 Americans currently have it
- The average age of diagnosis is 55
- The development of ALS symptoms generally occurs between ages 40 and 70
- As people age, the percentage of men and women who are diagnosed becomes almost equal
- Life expectancy is 3+ years for 1/2 of people after diagnosis
- 20% of people live 5+ years after diagnosis
- 10% live 10+ years
- Dr. Stephen Hawking, a remarkably prolific man, has lived for decades with ALS
- 90-95% of those with ALS have sporadic, or random disease, with no genetic factor
- 5-10% have the genetic variety
Symptoms
Symptoms of ALS can vary by person and vary in progression and severity. Initial symptoms of Lou Gehrig's disease can be subtle, and therefore overlooked. They can include tripping, "lead" or heavy legs and arms, dropping things, muscle rigidity and/or spasticity. Emotional symptoms may include unusual crying and/or laughing. Later in the disease, the muscles of the face and/or torso become affected. Speech, swallowing, chewing, and possibly breathing may be compromised. People affected with ALS experience progressive paralysis thoughout the body. The rate at which this happens varies from person to person.
Diagnosis
The diagnosis of ALS is very tricky because it involves the elimination of other neurological disease diagnoses which present similarly. A thorough neurological exam detects the ALS patient's muscular weakness. This weakness can be localized or more diffuse thoughout the body. Muscle stiffness can also be observed.
Patients may undergo nerve tests such as an EMG or NCV. NCV, or nerve conduction velocity, reveals the speed at which nerves transmit information to the muscles. EMG, or electromyography, tests nerve impulses within the muscles. Weak responses can signify ALS.
Some other tests to diagnose ALS include MRI, or magnetic resonance imaging, which can rule out other neurological disorders, and bloodwork and/or spinal taps, which rule out such autoimmune diseases as lupus or multiple sclerosis. For further reference: www.ALSA.org and http://www.neurologychannel.com/als.
*****For more information on ALS, read ALS Support, Dr. Stephen Hawking and ALS and Lou Gehrig's Story.
*****For related neurological information, visit the Neurological ARTICLE INDEX and Blog.
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