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Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS, sometimes called Lou Gehrig's disease) is a progressive, invariably fatal motor neurone disease. In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away (atrophy), and twitch (fasciculations). Eventually, the ability of the brain to start and control voluntary movement is lost. more...

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ALS causes weakness with a wide range of disabilities. Eventually, all muscles under voluntary control are affected, and patients lose their strength and the ability to move their arms, legs, and body. When muscles in the diaphragm and chest wall fail, patients lose the ability to breathe without help from mechanical ventilation. Most people with ALS die from respiratory failure, usually within 3 to 5 years from the onset of symptoms. However, about 10 % of ALS patients survive for 10 or more years.

Epidemiology

As many as 20,000 Americans have ALS, and an estimated 5,000 people in the United States are diagnosed with the disease each year. ALS is one of the most common neuromuscular diseases worldwide, and people of all races and ethnic backgrounds are affected. ALS most commonly strikes people between 40 and 60 years of age, but younger and older people also can develop the disease. Men are affected more often than women.

In 90 to 95 % of all ALS cases, the disease occurs apparently at random with no clearly associated risk factors. Patients do not have a family history of the disease, and their family members are not considered to be at increased risk for developing ALS.

About 5 to 10 % of all ALS cases are inherited. The familial form of ALS usually results from a pattern of inheritance that requires only one parent to carry the gene responsible for the disease. About 20 % of all familial cases result from a specific genetic defect that leads to mutation of the enzyme known as superoxide dismutase 1 (SOD1). Research on this mutation is providing clues about the possible causes of motor neuron death in ALS. Not all familial ALS cases are due to the SOD1 mutation, therefore other unidentified genetic causes clearly exist.

Symptoms

The onset of ALS may be so subtle that the symptoms are frequently overlooked. The earliest symptoms may include twitching, cramping, or stiffness of muscles; muscle weakness affecting an arm or a leg; slurred and nasal speech; or difficulty chewing or swallowing. These general complaints then develop into more obvious weakness or atrophy that may cause a physician to suspect ALS.

The parts of the body affected by early symptoms of ALS depend on which muscles in the body are damaged first. In some cases, symptoms initially affect one of the legs, and patients experience awkwardness when walking or running or they notice that they are tripping or stumbling more often. Some patients first see the effects of the disease on a hand or arm as they experience difficulty with simple tasks requiring manual dexterity such as buttoning a shirt, writing, or turning a key in a lock. Other patients notice speech problems.

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Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a disease that breaks down tissues in the nervous system (a neurodegenerative disease) of unknown cause that affects ...
Survival in amyotrophic lateral sclerosis with home mechanical ventilation : the impact of systematic respiratory assessment and bulbar involvement
Study objectives: To analyze (1) the impact of a protocol of early respiratory evaluation of the indications for home mechanical ventilation (HMV) in ...
Appropriate management of respiratory problems is of utmost importance in the treatment of patients with amyotrophic lateral sclerosis
Respiratory problems (RPs) are the main cause of death in patients with amyotrophic lateral sclerosis (ALS). (1,2) However, nowadays many of these RPs ...
Increasing peak expiratory flow time in amyotrophic lateral sclerosis
Background: Serial measurements of pulmonary function to indicate decreasing respiratory muscle strength in patients with amyotrophic lateral sclerosis ...
Oximetry and indications for tracheotomy for amyotrophic lateral sclerosis
Study objective: To explore the use of oximetry as a guide for using respiratory aids and tracheotomy in the treatment of patients with amyotrophic lateral sclerosis (ALS). Setting: A retrospecti
Efficacy of mechanical insufflation-exsufflation in medically stable patients with amyotrophic lateral sclerosis
Objective: To determine under what circumstances the use of mechanical insufflation-exsufflation (MI-E) can generate clinically effective expiratory ...
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The Department of Veterans Affairs (VA) is developing a nationwide registry of living veterans who have Amyotrophic Lateral Sclerosis (ALS). The goal ...
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ONCE THE DIAGNOSIS OF AMYOTROPHIC lateral sclerosis was made, Bruce Edwards understood why his left hand did what it did. The caddie cleans golf balls ...

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