So - my niece nominated me to take the ALS/Ice bucket challenge. Im game - but I really think it needs to be about more than just ice over the head. So - Jillian Lee, I accepted your challenge - and I matched your donation in honor of this young man and everyone who is dealing with this horrible disease and the family and friends who are going through it with them. upworthy/the-last-ice-bucket-challenge-you-need-to-see-and-you-really-should-see-it?g=2&c=ufb1 I am not nominating anyone specifically, but if this resonates with you - please feel free to make a donation to any charity that speaks to your heart. What is ALS (amyotrophic lateral sclerosis)? Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrigs disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons)responsible for controlling voluntary muscles (muscle action we are able to control, such as those in the arms, legs, and face). The disease belongs to a group of disorders known as motor neuron diseases, which are characterized by the gradual degeneration and death of motor neurons. Motor neurons are nerve cells located in the brain, brain stem, and spinal cord that serve as controlling units and vital communication links between the nervous system and the voluntary muscles of the body. Messages from motor neurons in the brain (called upper motor neurons) are transmitted to motor neurons in the spinal cord (called lower motor neurons)and from them to particular muscles. In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, and stop sending messages to muscles. Unable to function, the muscles gradually weaken, waste away (atrophy), and have very fine twitches (called fasciculations). Eventually, the ability of the brain to start and control voluntary movement is lost. ALS causes weakness with a wide range of disabilities (see section titled “What are the symptoms?”). Eventually, all muscles under voluntary control are affected, and individuals lose their strength and the ability to move their arms, legs, and body. When muscles in the diaphragm and chest wall fail, people lose the ability to breathe without ventilatory support. Most people with ALS die from respiratory failure, usually within 3 to 5 years from the onset of symptoms. However, about 10 percent of those with ALS survive for 10 or more years. Although the disease usually does not impair a person’s mind or intelligence, several recent studies suggest that some persons with ALS may have depression or alterations in cognitive functions involving decision-making and memory. ALS does not affect a person’s ability to see, smell, taste, hear, or recognize touch. Patients usually maintain control of eye muscles and bladder and bowel functions, although in the late stages of the disease most individuals will need help getting to and from the bathroom. Who gets ALS? Anyone - it does not discriminate. More than 12,000 people in the U.S. have a definite diagnosis of ALS, for a prevalence of 3.9 cases per 100,000 persons in the U.S. general population, according to a report on data from the National ALS Registry. ALS is one of the most common neuromuscular diseases worldwide, and people of all races and ethnic backgrounds are affected. ALS is more common among white males, non-Hispanics, and persons aged 60–69 years, but younger and older people also can develop the disease. Men are affected more often than women. In 90 to 95 percent of all ALS cases, the disease occurs apparently at random with no clearly associated risk factors. Individuals with this sporadic form of the disease do not have a family history of ALS, and their family members are not considered to be at increased risk for developing it. About 5 to 10 percent 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. Mutations in more than a dozen genes have been found to cause familial ALS. About one-third of all familial cases (and a small percentage of sporadic cases) result from a defect in a gene known as “chromosome 9 open reading frame 72,” or C9orf72. The function of this gene is still unknown. Another 20 percent of familial cases result from mutations in the gene that encodes the enzyme copper-zinc superoxide dismutase 1 (SOD1). What are the symptoms? The onset of ALS may be so subtle that the symptoms are overlooked. The earliest symptoms may include cramps, tight and stiff muscles (spasticity), 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 showing early symptoms of ALS depend on which muscles in the body are affected. Many individuals first see the effects of the disease in 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. In other cases, symptoms initially affect one of the legs, and people experience awkwardness when walking or running or they notice that they are tripping or stumbling more often. When symptoms begin in the arms or legs, it is referred to as “limb onset” ALS. Other individuals first notice speech problems, termed “bulbar onset” ALS. Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Individuals may develop problems with moving, swallowing (dysphagia), and speaking or forming words (dysarthria). Symptoms of upper motor neuron involvement include spasticity and exaggerated reflexes (hyperreflexia) including an overactive gag reflex. An abnormal reflex commonly called Babinski’s sign (the large toe extends upward as the sole of the foot is stimulated in a certain way) also indicates upper motor neuron damage. Symptoms of lower motor neuron degeneration include muscle weakness and atrophy, muscle cramps, and fasciculations. To be diagnosed with ALS, people must have signs and symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes. . How is ALS diagnosed? No one test can provide a definitive diagnosis of ALS, although the presence of upper and lower motor neuron signs is strongly suggestive. Instead, the diagnosis of ALS is primarily based on the symptoms and signs the physician observes in the patient and a series of tests to rule out other diseases. Physicians obtain the individual’s full medical history and usually conduct a neurologic examination at regular intervals to assess whether symptoms such as muscle weakness, atrophy of muscles, hyperreflexia, and spasticity are getting progressively worse. Since ALS symptoms in the early stages of the disease can be similar to those of a wide variety of other, more treatable diseases or disorders, appropriate tests must be conducted to exclude the possibility of other conditions. One of these tests is electromyography (EMG), a special recording technique that detects electrical activity in muscles. Certain EMG findings can support the diagnosis of ALS. Another common test is a nerve conduction study (NCS), which measures electrical energy by assessing the nerve’s ability to send a signal). Specific abnormalities in the NCS and EMG may suggest, for example, that the individual has a form of peripheral neuropathy (damage to peripheral nerves) or myopathy (muscle disease) rather than ALS. The physician may order magnetic resonance imaging (MRI), a noninvasive procedure that uses a magnetic field and radio waves to take detailed images of the brain and spinal cord. Standard MRI scans are normal in people with ALS. However, they can reveal evidence of other problems that may be causing the symptoms, such as a spinal cord tumor, a herniated disk in the neck that compresses the spinal cord, syringomyelia (a cyst in the spinal cord), or cervical spondylosis (abnormal wear affecting the spine in the neck). Based on the person’s symptoms and findings from the examination and from these tests, the physician may order tests on blood and urine samples to eliminate the possibility of other diseases as well as routine laboratory tests. In some cases, for example, if a physician suspects that the individual may have a myopathy rather than ALS, a muscle biopsy may be performed. Infectious diseases such as human immunodeficiency virus (HIV), human T-cell leukemia virus (HTLV), polio, West Nile virus, and Lyme disease can in some cases cause ALS-like symptoms. Neurological disorders such as multiple sclerosis, post-polio syndrome, multifocal motor neuropathy, and spinal muscular atrophy also can mimic certain facets of the disease and should be considered by physicians attempting to make a diagnosis. Fasciculations, the fine rippling movements in the muscle, and muscle cramps also occur in benign conditions. Because of the prognosis carried by this diagnosis and the variety of diseases or disorders that can resemble ALS in the early stages of the disease, individuals may wish to obtain a second neurological opinion. What causes ALS? The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others. How Can I Help? Get involved - you can donate your time and your money Help create a world without ALS by getting involved in the way that best suits YOU! When you fundraise with friends and family you take us closer to discovering a cure. DONATE HERE Where can I get more information? alsa.org/ For more information on neurological disorders or research programs funded by the National Institute of Neurological Disorders and Stroke, contact the Institutes Brain Resources and Information Network (BRAIN) at: BRAIN P.O. Box 5801 Bethesda, MD 20824 (800) 352-9424 ninds.nih.gov Information also is available from the following organizations: ALS Association 1275 K Street, N.W. Suite 1050 Washington, DC 20005 [email protected] alsa.org Tel: 202-407-8580 Fax: 202-289-6801 Les Turner ALS Foundation 5550 W. Touhy Avenue Suite 302 Skokie, IL 60077-3254 [email protected] lesturnerals.org Tel: 888-ALS-1107 847-679-3311 Fax: 847-679-9109 Muscular Dystrophy Association 3300 East Sunrise Drive Tucson, AZ 85718-3208 [email protected] mda.org Tel: 520-529-2000 800-572-1717 Fax: 520-529-5300 Project ALS 3960 Broadway Suite 420 New York, NY 10032 [email protected] projectals.org Tel: 212-420-7382 800-603-0270 Fax: 212-420-7387
Posted on: Wed, 20 Aug 2014 23:29:59 +0000
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