![]() muscle weakness affecting an arm, a leg, neck or diaphragm įor many individuals the first sign of ALS may appear in the hand or arm as you try to do simple tasks, such as buttoning a shirt, writing, or turning a key in a lock.muscle twitches in the arm, leg, shoulder, or tongue.Gradually, however, these symptoms develop into more obvious weakness or atrophy that may cause a physician to suspect ALS. ![]() Some individuals carrying this mutation may show signs of both motor neuron and dementia symptoms (ALS-FTD).Īnother 12 to 20 percent of familial cases result from mutations in the gene that provides instructions for the production of the enzyme copper-zinc superoxide dismutase 1 ( SOD1).ĪLS can be so subtle that you may not notice the first symptoms. Interestingly, the same mutation can be associated with atrophy of frontal-temporal lobes of the brain causing frontal-temporal lobe dementia. About 25 to 40 percent of all familial cases (and a small percentage of sporadic cases) are caused by a defect in a gene known as “chromosome 9 open reading frame 72,” or C9ORF72. Mutations in more than a dozen genes have been found to cause familial ALS. The familial form of ALS usually only requires one parent to carry the gene responsible for the disease. Familial (Genetic) ALSĪbout five to 10 percent of all ALS cases are familial, which means that an individual inherits the disease from his or her parents. This means the disease seems to occur at random with no clearly associated risk factors and no family history of the disease.Īlthough family members of people with sporadic ALS are at an increased risk for the disease, the overall risk is very low and most will not develop ALS. The majority of ALS cases (90 percent or more) are considered sporadic. ALS is recognized as a service-connected disease by the U.S. Although the reason for this is unclear, possible risk factors for veterans include exposure to lead, pesticides, and other environmental toxins. Some studies suggest that military veterans are more likely to develop ALS. Most likely to develop the disease are Caucasians and non-Hispanics. However, as age the difference between men and women disappears. Men are slightly more likely than women to develop ALS. Although the disease can strike at any age, symptoms most commonly develop between the ages of 55 and 75. There are several potential risk factors for ALS including: It affects people of all races and ethnic backgrounds. If you have ALS, you lose your strength and the ability to speak, eat, move, and even breath.ĪLS is a common neuromuscular disease worldwide. Gradually all muscles under voluntary control are affected. Early Signs of ALSĮarly symptoms of ALS usually include muscle weakness or stiffness. Eventually, the brain loses its ability to initiate and control voluntary movements. Unable to function, the muscles gradually weaken, start to twitch (called fasciculations), and waste away (atrophy). In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, and stop sending messages to the muscles. They they move from the spinal cord and motor nuclei of brain to a particular muscle or muscles. Messages from motor neurons in the brain (called upper motor neurons) are transmitted to motor neurons in the spinal cord and to motor nuclei of brain (called lower motor neurons). These motor neurons provide communication links between the brain and the voluntary muscles. They also extend to muscles throughout the body. Motor neurons are nerve cells that extend from the brain to the spinal cord. The gradual deterioration (degeneration) and death of motor neurons causes these diseases. Currently, there is no cure for ALS and no effective treatment to halt, or reverse, the progression of the disease.ĪLS belongs to a wider group of disorders known as motor neuron diseases. The disease is progressive, meaning the symptoms get worse over time. Voluntary muscles produce movements like: These diseases mainly involve the nerve cells (neurons) that are responsible for controlling voluntary muscle movement. Amyotrophic lateral sclerosis (ALS) is a rare group of neurological diseases.
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