My sister was diagnosed with amyotrophic lateral sclerosis last year. She had been experiencing symptoms for about a year before that. Please tell me about ALS and any new developments toward a cure. — Tucson
Amyotrophic lateral sclerosis, also called Lou Gehrig's disease after the famous baseball player who developed the condition in the 1930s, attacks motor neurons — the nerve cells in the brain and spinal cord that control voluntary muscles.
The onset of ALS is generally noticed through weakness in the upper or lower limbs, problems with walking, decreased speech clarity or trouble swallowing. The weakness tends to begin in a specific area of the body, such as an arm or leg, and slowly spreads to other regions.
ALS usually spares the intellect, as well as the senses of sight, hearing, smell, taste and touch. Also, it doesn't affect involuntary muscles, such as those that control the heartbeat and the functioning of the bladder and bowels.
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The cause of ALS is not yet known, though research suggests that several mechanisms, perhaps in combination, are involved. One factor may be excessive levels of the neurotransmitter glutamate, which can cause neurons to die. Researchers also are studying viruses, immune-system abnormalities, environmental toxins, cell suicide (apoptosis), defects in the energy-producing parts of a cell (mitochondria) and the buildup of certain proteins known as neurofilaments.
In about 10 percent of ALS victims, the condition is caused by an inherited trait.
There is no cure for ALS, nor is there a treatment capable of restoring lost function or stopping the progression of weakness. ALS unfortunately remains a disabling illness that gradually tends to produce significant impairments, causing potentially life-threatening problems with swallowing and respiration.
In surveys of ALS patients, the average survival tends to be less than five years after diagnosis, although this may vary significantly from person to person.
Treatment mainly involves efforts to make patients more comfortable and independent, and to slow the progression of symptoms — that is, to preserve the quality of life as much as possible. Riluzole (Rilutek brand name) is the first and only medicine to be approved by the Food and Drug Administration for treatment of ALS. The drug has been shown in research studies to slow progression modestly, perhaps by reducing glutamate levels.
— Dr. Kevin B. Boylan, neurology, Mayo Clinic, Jacksonville, Fla.