- Every 90 minutes someone is diagnosed with ALS.
- Every 90 minutes someone loses their battle with ALS.
- ALS can strike anyone at anytime; it serves no racial, ethnic or socioeconomic boundaries.
- The average life expectancy for a person with ALS is 2-5 years.
- There is no cause or cure for ALS.
- ALS most commonly strikes people between 40 and 60 years of age, but younger and older people can also develop the disease.
- Men are affected slightly more often than women.
- As many as 30,000 Americans have ALS and an estimated 5,000 people in the
- ALS is one of the most common neuromuscular diseases worldwide.
- ALS is considered a blind or orphan disease.
Amyotrophic Lateral Sclerosis (ALS), more commonly known as Lou Different Types of ALS Sporadic ALS In 90 to 95 percent 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. Sporadic ALS can strike anyone at anytime. Familial ALS 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. About 20 percent 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, however, not all familial ALS cases are due to the SOD1 mutation; therefore there are other unidentified genetic causes out there. The onset of ALS may be so subtle that the symptoms are frequently overlooked. The earliest symptoms may include muscle twitching, cramping, or stiffness; muscle weakness affecting an arm or a leg; slurred and nasal speech, difficulty to project voice; or difficulty chewing or swallowing such things as saliva and liquids rather than solid foods. Excessive salivation and difficulty swallowing may cause drooling. A few other common symptoms may include tripping, dropping things, abnormal fatigue of the arms and/or legs, twitching of the tongue, and shortness of breath or uncontrollable periods of laughing or crying. These general symptoms then develop into more obvious weakness or atrophy that may cause a physician to suspect ALS. The rate at which ALS progresses, can be quite variable from one person to another. The parts of the body affected by early symptoms of ALS depend on which muscles in the body are damaged first. There are two different onsets of ALS. First, and more common, is limb-onset. Limb-onset is used to characterize a newly diagnosed ALS patient whose main symptoms begin either in the upper or lower limbs. These symptoms can include weakness in the hands and wrist or legs and ankle. In some cases, symptoms initially affect one of the legs. 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. The second type of onset is: bulbar-onset. The muscles of the throat, tongue, jaw, and face are known as bulbar because the area of the brain that controls these muscles – the lower brainstem – was once known as the bulb. Because patients with bulbar-onset have difficulty swallowing and may choke or aspirate frequently, it has been found generally that these patients carry a poorer prognosis than those with the limb-onset. However, much longer courses of bulbar-onset have been reported. Typically 75 – 85% of patients diagnosed with limb-onset will eventually develop signs and symptoms of bulbar-onset. 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. Not all people with ALS experience the same symptoms or the same sequences or patterns of progression, but progressive muscle weakness and paralysis are universally experienced. Although the sequence of emerging symptoms and the rate of disease progression vary from person to person, eventually patients will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. Difficulty swallowing and chewing impair the patient's ability to eat normally and increase the risk of choking. Maintaining weight will then become a problem, therefore limbs begin to look "thinner" as muscle tissue atrophies. Because the disease usually does not affect cognitive abilities, patients are aware of their progressive loss of function and may become fearful, anxious and/or depressed. A small percentage of patients may experience problems with memory or decision-making, and there is growing evidence that some may even develop a form of dementia. Health care professionals need to explain the course of the disease and describe available treatment options so that patients can make informed decisions in advance. In later stages of the disease, patients have difficulty breathing because the weakening and paralysis continue to spread to the muscles of the trunk of the body. Patients eventually lose the ability to breathe on their own and must depend on ventilatory support for survival. Patients also face an increased risk of pneumonia during later stages of ALS. To be diagnosed with ALS, patients must have signs and symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes. ALS is difficult to diagnose because the symptoms are similar to those of other neuromuscular disorders, many of which are treatable. To date, there is no one test or procedure to ultimately establish the diagnosis of ALS. It is done through a clinical examination and series of diagnostic tests, often ruling out other diseases that mimic ALS. Since there are several diseases that mimic ALS, many of which are treatable, it is highly recommended that a person given an ALS diagnosis seek a second opinion, an opinion from an ALS “expert”, someone who diagnoses and treats many ALS patients. To determine if you have ALS, your doctor will perform an evaluation that includes a physical exam, medical history, blood tests may be done to detect the presence of heavy metals such as lead in the blood; and urine tests may detect abnormal proteins or hormone levels associated with other neurological diseases, Nerve Conduction Velocity (NCV), which tests the function of the of the motor and sensory nerves in the body; an Electromyography (EMG), which measures nerve impulses within the muscles and is sensitive in detecting lower motor neuron disease. Symptoms of lower motor neuron disease include muscle weakness, cramps and twitching or fasciculation’s. Although there are no standard tests for upper motor neuron disease, there are tests that may show signs of upper neuron damage such as the Babinski’s sign, an abnormal reflex where the large toe extends upward as the sole of the foot is stimulated in a certain way. Another test is done through the patients reflexes. The muscles may be so stiff that when the neurologist moves them, they continue to move abnormally afterward. When the neurologist tests the "knee jerk" reaction, the movement is abnormally quick. This shows signs of muscle weakness and atrophy, either localized or widespread, depending on the extent of the disease. A MRI (Magnetic Resonance Imaging) scan may also be used to rule out spinal cord or brainstem disease and a lumbar puncture or spinal tap may be performed to analyze the cerebrospinal fluid for genetic abnormalities. Because ALS affects the skeletal, voluntary muscles, the neurological exam usually does not reveal abnormalities in the sensory reflexes (i.e., vision, hearing, taste, smell, touch, or bowel and bladder control) Other treatments for ALS are designed to relieve symptoms and improve the quality of life for patients. Physicians can prescribe medications to help reduce fatigue, ease muscle cramps, control spasticity, and reduce excess saliva and phlegm, help patients with pain, sleep disturbances and constipation. Physical therapy is a very important part of treatment to help relieve muscle cramping and pain. Other therapies, such as occupational therapy and speech therapy, are also used in treatment. ALS patients require a diet of high-energy foods that are easy to swallow, therefore may benefit from a nutritionist. If the patient is not able to maintain adequate nutrition, a feeding tube is usually inserted. This has been shown to prolong life in ALS patients who are losing weight. Some ALS patients may also need pulmonary consultants and respiratory therapists to assist breathing. Fewer than 5% of patients use long-term ventilation support. Noninvasive forms of breathing assistance are available as well, such as a CPAP (continuous positive airway pressure or a BiPAP (bilevel positive airway pressure), to improve the patient’s quality of life. Depression is very common among ALS patients, so therefore, antidepressant medication and counseling can help patients and their families cope This supportive care is best provided by health care professionals such as physicians; pharmacists; physical, occupational, and speech therapists; nutritionists; social workers; and home care and hospice nurses. Working with patients and caregivers, these teams can design an individualized plan of medical and physical therapy and provide special equipment aimed at keeping patients as mobile and comfortable as possible.
a progressive, fatal, neurodegenerative disease that affects nerve cells in the brain and spinal cord, caused by the degeneration of motor neurons; the nerves in the central nervous system that control voluntary movement, and reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. 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. As a motor neuron disease, the disorder causes muscle weakness throughout the body, as both the upper and lower motor neurons degenerate, failing to send messages to muscles. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. Unable to function, the muscles gradually weaken, develop twitches (fasciculations) because of denervation, and eventually atrophy because of that denervation. The patient may ultimately lose the ability to initiate and control all voluntary movement except for the eyes.
ALS causes weakness with a wide range of disabilities. Eventually, all voluntary muscles are affected, and the patients lose their strength and their ability to move their arms, legs and body. When the muscles in the diaphragm and chest wall fail, patients lose the ability to breathe without ventilatory support. Most people with ALS die from respiratory failure, usually within 2 to 5 years from the onset of symptoms. However, about 10 percent of ALS patients survive for 10 or more years, and some may only survive a few short months. Although the disease usually does not impair a person's mind or intelligence, several recent studies suggest that some ALS patients may have alterations in cognitive functions such as depression and problems with 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 patients will require help getting to and from the bathroom.
A-myo-trophic comes from When the Greek language. "A" means no or negative. "Myo" refers to muscle, and "Trophic" means nourishment–"No muscle nourishment." When a muscle has no nourishment, it "atrophies" or wastes away. "Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates it leads to scarring or hardening ("sclerosis") in the region.
Symptoms of ALS
ALS Diagnosis
Treatment
There is no cure for ALS. In 1995, the FDA approved the first drug treatment, Riluzole (Rilutek®). Rilutek® is one of the few drugs so far proven to be effective against ALS and may prevent progression and possibly prolong life for a few months. It is believed to reduce damage to motor neurons by decreasing the release of glutamate, typically patients with ALS have an excess of glutamate. Glutamate is a part of protein that cells in the body use to help break down food and build up body tissues. In the central nervous system, nerve cells (neurons) use glutamate to communicate with one another. Rilutek® does not reverse the damage already done to motor neurons, and patients taking the drug must be monitored for liver damage and other possible side effects. However, this first disease-specific drug offers hope that the progression of ALS may one day be slowed by new or combinations of medications.
