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Amyotrophic Lateral Sclerosis (ALS) is a disease of the regions of the
nervous system that control voluntary movement. It usually strikes in late
middle age, although may also occur in young adults, children, and the elderly.
The overall prevalence is 4-6/100,000 with a slight male predominance (1.2/1).
In most cases, the cause is unknown, although certain genetic risk factors
exist. Gradually, motor nerve cells lose their function, resulting in weak
muscles and paralysis. Death usually occurs from respiratory complications,
typically three to five years after diagnosis.
Typical symptoms of ALS include stiffness, weakness, and wasting of limb and
trunk muscles. Muscle twitching and cramping are also common. Patients
eventually develop difficulties with speaking, swallowing, chewing, shortness
of breath, and ambulation. Numbness, tingling, and pain are rare, although
affected body parts may feel cold. Hearing, vision, touch, eye movements, and
intellect are unaffected.
Unfortunately, medicinal treatment is limited to one medication (Rilutek),
which extends survival just a few months. Other treatment modalities include
occupational, physical, and speech therapies. Many patients eventually require
feeding tube placement. Some utilize ankle-foot-orthoses (AFOs), walkers,
scooters, and wheelchairs. As respiration becomes compromised, tracheotomy may
be required.
Researchers are actively trying to identify the causes and potential treatments
for ALS. Possible causes range from viral infections, excessive "free radical"
cell toxins, to environmental toxins and mitochondrial defects. A variety of
potential treatments are being evaluated, including hormonal therapy, nerve
growth factors, antibiotics, and antioxidants.
ALS remains a progressively debilitating, and ultimately fatal disease
frequently striking those in the prime of their lives. However, research
continuously sheds new light on its possible causes and treatments, and may one
day produce a cure.
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