Doctor's Notes on Amyotrophic Lateral Sclerosis (ALS)
Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron disease that affects the part of the nervous system that controls voluntary movements. It is also referred to as Lou Gehrig's disease for the famous baseball player who died of the disease. The muscles become progressively weaker, and the condition eventually leads to paralysis and death. The effects of ALS are not reversible and there is no cure.
Early symptoms of amyotrophic lateral sclerosis may be subtle and include
- weakness,
- awkwardness,
- atrophy in one or more limbs, or
- difficulty swallowing or speaking.
In addition, common symptoms of ALS include
- difficulty standing,
- walking, or running;
- clumsiness;
- difficulty with fine hand motions such as buttoning,
- writing,
- turning a key in a lock; atrophy of hand muscles,
- atrophy of the tongue,
- difficulty chewing food,
- oversensitive gag reflex,
- difficulty forming words,
- weakness and atrophy in specific muscles,
- stiff muscles,
- muscle cramps, and
- muscle twitching visible under the skin.
Depression and anxiety may occur in ALS patients and are believed to be part of the disease process. Some patients may have symptoms such as involuntary, uncontrolled outbursts of crying or laughter, which is different than their underlying mood. As the disease progresses, ALS causes the loss of ability to carry out everyday activities and as respiratory muscles weaken, breathing becomes more difficult. Most people with ALS die within five years of the onset of symptoms.
What Is the Treatment for Amyotrophic Lateral Sclerosis (ALS)?
Treatment of amyotrophic lateral sclerosis (ALS) is aimed at slowing the progression and assisting with the symptoms that cause complications. Supportive care and physical assistive devices make up many of the treatments available for ALS.
Medications approved to treat ALS include:
- Edaravone (Radicava)
- Riluzole (Rilutek)
- Dextromethorphan HBr and quinidine sulfate (Nuedexta)
Other medications are used for the symptoms of ALS such as pain medications, anti-spasm mediations, and antidepressants.
Supportive care and assistive devices are the mainstays of treatment for the progressive symptoms of ALS. Assistive devices most commonly needed are:
- PEG tube or feeding tubes for nutrition and hydration
- Mobility devices
- Cane
- Wheelchair
- Computer-assisted communication devices
- Oxygen devices
- Breathing machines
- Masks
- Tracheostomy
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Dementia in ALS
Amyotrophic lateral sclerosis (ALS) affects the voluntary nervous system that causes muscles to weaken, leading progressively to paralysis and death, usually within five years of the first symptoms. ALS, or Lou Gehrig's disease, usually doesn't cause cognitive impairment, but is sometimes accompanied by dementia due to destruction of cells in the frontal lobe of the brain. -
Dysphagia (Difficulty Swallowing)
Dysphagia (difficulty swallowing) has many causes. Dysphagia can be caused by diseases of the brain, diseases or conditions that affect muscle function, diseases of the esophagus, or obstructions of the upper digestive tract and esophagus. Symptoms of dysphagia generally is a mild to severe form of difficulty swallowing. Treatment of dysphagia depends on the cause. -
How Do People Get ALS?
Amyotrophic lateral sclerosis (ALS, also called Lou Gehrig's disease) is a rare disease that damages the nerves that control muscles. Over time, the muscles gradually weaken, twitch (fasciculate), and atrophy, and the brain loses the ability to control voluntary movements. -
Muscle Cramps (Muscle Spasms)
Muscle cramps occur when a muscle undergoes a sustained, forceful, and involuntary contraction. The cramp may cause a visible or palpable hardening of the muscle. Dehydration, fatigue, and certain medications may cause muscle cramps. The primary treatments for muscle cramps include relaxing, stretching, massaging, and applying heat to the cramp. -
Pick Disease
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REFERENCE:
Kasper, D.L., et al., eds. Harrison's Principles of Internal Medicine, 19th Ed. United States: McGraw-Hill Education, 2015.