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Also see - Treating Respiratory Problems In ALS Introduction Amyotrophic lateral sclerosis (ALS) is a serious neurological disease that attacks the nerve cells that control voluntary muscles. It may begin with muscle twitching or weakness in an arm or leg, or with slurring of speech. Eventually, amyotrophic lateral sclerosis affects your ability to control the muscles needed to move, speak, eat and breathe. In the United States, amyotrophic lateral sclerosis is often called Lou Gehrig's disease, after Hall of Fame baseball player Lou Gehrig of the New York Yankees, who died of the disease in 1941. The cause of amyotrophic lateral sclerosis remains elusive, as does any treatment that can reverse the relentless progression of the disease. Treatment of amyotrophic lateral sclerosis typically focuses on efforts to relieve symptoms and maintain quality of life in the years after diagnosis. Signs and symptoms Signs of amyotrophic lateral sclerosis are typically subtle at first. The disease commonly begins in one part of the body and progresses to other areas. Gradual death of nerve cells saps muscles of their control. Early signs and symptoms of ALS include:
The disease frequently begins in your hands, feet or limbs, and then spreads to other parts of your body. It eventually affects chewing, swallowing, speaking and breathing — which are known as bulbar functions after the bulb-shaped part of the brainstem that houses nerve cells controlling these functions. Less commonly, ALS begins with these bulbar symptoms. As the disease advances, your muscles become progressively weaker until they're paralyzed. Eventually, amyotrophic lateral sclerosis paralyzes the muscles needed to breathe. Most people with ALS die of respiratory failure, usually within three to five years after symptoms begin. Sometimes, people with amyotrophic lateral sclerosis develop pneumonia because they can't swallow and they inhale (aspirate) food and oral secretions into their lungs. ALS usually leaves intellect intact and spares the senses of sight, hearing, smell, taste and touch. It doesn't affect involuntary muscles, such as the muscles that control heartbeat and bladder and bowel function.
Causes The basic units of your central nervous system — which includes your brain and spinal cord — are nerve cells (neurons). Each neuron consists of a cell body, a major branching fiber (axon) and numerous smaller branching fibers (dendrites). Nerve cells communicate with adjacent nerve cells at contact points called synapses. They also send and receive signals throughout your body. Nerve signals provide information to your brain through your senses. They communicate with your involuntary muscles — the ones that control activities such as digestion, sexual function and heartbeat. Nerve signals also activate your voluntary muscles. Amyotrophic lateral sclerosis is a disease that involves death of motor neurons — the nerve cells that control voluntary muscles. These are the muscles you use to move your limbs, face, neck and torso and to talk, chew, swallow and breathe. In ALS, both upper motor neurons, located in your brain, and lower motor neurons, located in your spinal cord, gradually die. As a result, your voluntary muscles no longer receive messages. These muscles become weak and begin to waste away (atrophy). Researchers aren't sure what causes motor neuron deterioration, but amyotrophic lateral sclerosis appears to fall into two categories:
One possible cause of amyotrophic lateral sclerosis is excess glutamate — one of the many chemicals (neurotransmitters) that neurons use to send signals to one another. People with ALS appear to have too much glutamate in their nervous systems, which can cause neurons to die. Researchers are studying other factors that may contribute to amyotrophic lateral sclerosis, including 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. Some researchers believe that a combination of more than one factor may trigger ALS. Risk factors Although most cases of amyotrophic lateral sclerosis have no apparent cause, a small percentage of people who have ALS inherit it. Inherited ALS may strike at a younger age and will affect consecutive generations. If you're the first person in your family to have amyotrophic lateral sclerosis, you likely don't have an inherited form, and your children aren't at a greater risk. Noninherited ALS often develops between ages 40 and 60, although the disease affects people of all ages. ALS affects more men than women. When to seek medical advice If you've noticed weakness in one of your limbs or twitching and cramping in your muscles, see your doctor to determine if there may be a neurological cause such as amyotrophic lateral sclerosis. Although there's no treatment to reverse the course of ALS, other neurological conditions that may mimic some of the signs and symptoms of ALS are treatable. It's easy to ignore the early, sometimes subtle signs of amyotrophic lateral sclerosis. In fact, it takes about a year before most people are diagnosed with ALS. But the sooner you see your doctor, the sooner a diagnosis and treatment decisions can be made. Screening and diagnosis Amyotrophic lateral sclerosis is difficult to diagnose early because it may appear similar to other neurological diseases. Your doctor may refer you to a doctor who specializes in brain and nervous system disorders (neurologist). Diagnosing amyotrophic lateral sclerosis first involves reviewing your family's medical history and your signs and symptoms. You'll then have a physical examination that may include some basic in-office testing of your nerves and muscles. Additionally, you'll undergo a variety of tests, including tests to rule out other conditions. These diagnostic tests may include:
Treatment Because there's no reversing the course of amyotrophic lateral sclerosis, treatments mainly involve efforts to make you more comfortable and independent and to slow the progression of symptoms. Treatments may involve many professionals, including doctors, therapists, dietitians, social workers and hospice nurses, and may include:
As breathing muscles weaken further, you may need to decide whether or not to use the full-time help of a respirator that inflates and deflates your lungs. A respiratory therapist or lung specialist (pulmonologist) can help you choose devices that will work for you. New treatments Researchers are investigating many other medications for their usefulness in treating amyotrophic lateral sclerosis. Examples include the antibiotic minocycline (Minocin), the breast cancer drug tamoxifen, the antioxidant coenzyme Q10 and a nerve-nourishing drug called insulin-like growth factor (IGF-I). Increasingly, researchers are studying drug "cocktails" — which are combinations of medications. Stem cell therapy is another avenue of research. Early studies show that stem cells may have the ability to repair or replace the motor neurons damaged by ALS, but clinical use or clinical trials with stem cell therapy is still a long way off. Only time will tell if these new treatments will be useful. It's important to work closely with a doctor who specializes in amyotrophic lateral sclerosis, who will be informed as new discoveries and treatments may become available. Some health care centers offer ALS clinics where people with ALS can work with physical therapists, occupational therapists, speech therapists, nurses, social workers and other people with ALS — all in the same place. Coping skills No one chooses to live with amyotrophic lateral sclerosis. Yet many have managed to do it with daring, dignity, humor and a hopeful attitude. If you have ALS, you can, too.
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