Approximately 5,600 adults are diagnosed with ALS (Amyotrophic Lateral Sclerosis) each year and about 30,000 Americans are living with the disease at any given time. ALS is more commonly known Lou Gehrig's Disease, named in 1939 after famed baseball player Lou Gehrig, whose diagnosis of ALS was confirmed on his 36th birthday.
ALS is not contagious, but it is terminal. While 60 percent of ALS patients are men and 90 percent are Caucasian, it does not discriminate against gender or ethnicity.
The ALS Association describes Amyotrophic Lateral Sclerosis as: A progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.
There are three types of ALS:
- 90 to 95 percent of cases are Sporadic (experts don't know what causes or triggers it)
- 5 to 10 percent of cases are Familial and may be caused by a genetic link
- Guamanian cases are named for the high concentration of ALS cases that took place in Guam in the 1950s.
Since ALS only affects motor neurons (the nerves associated with movement), it does not generally impair thinking, vision, senses of smell and taste, hearing, and bladder control. While the patient may not be able to move or communicate, his brain is usually as active as it was prior to diagnosis.
Early symptoms of ALS vary and can be subtle such as frequent muscle twitches, difficulty lifting feet, or tripping over uneven surfaces. 60 percent of patients complain of muscle weakness. Speech may be slurred or hand movements might appear uncoordinated. Common early symptoms include:
- Weakness in the muscles of the hands, arms, or legs or the muscles used to speak, swallow, or breathe
- Twitching and cramping of muscles, especially in hands and feet
- Difficulty using or coordinating arms and legs
- Thick, quiet, or slurred speech
As the disease progresses, patients become unable to walk, talk, breathe, swallow, or move unassisted. Patients will eventually require respiratory ventilation. Death is inevitable as extensive damage to motor neurons progresses. Most deaths associated with ALS are caused by respiratory failure. While most deaths occur within a few years of diagnosis, half of patients live longer than three years, 20 percent live longer than five years, and 10 percent live with quality of life for ten years or longer.
ALS is difficult to diagnose since there's no single test that positively identifies the disease. Instead, it's diagnosed when other diseases are ruled out and through the use of diagnostic tests including:
- Electomyography (EMG) and nerve conduction velocity (NCV)
- Blood and urine collection
- Spinal tap
- X-rays
- MRI
- Myelogram of cervical spine
- Muscle and/or nerve biopsy
- Thorough neurological examination
Currently, there's no cure for ALS and only one medication used to treat it. Riluzole slows the progression of ALS. Many patients report only a few months of life extension with Riluzole, while others report living better for longer periods of time. Other treatments associated with ALS focus on maintaining quality of life, symptom treatment, and life support.
Medical experts and researchers are looking for new ways to treat ALS and for clues about its causes. Ultimately, they're looking for a cure for a disease that strikes men and women in the prime of their lives.
Sources:
ALS Association http://www.alsa.org/
National Institute of Neurologic Disorders and Stroke
http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_ALS.htm
