Lou Gehrig disease


Lou Gehrig disease

Description, Causes and Risk Factors:

Alternative Name: Amyotrophic lateral sclerosis (ALS).

Lou Gehrig's disease is a progressive neuromuscular disease characterized by the progressive degeneration of motor nerve cell in the brain (upper motor neurons) and spinal cord (lower motor). Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. As motor neurons degenerate, they can no longer send impulses to the muscle fibers that normally result in muscle movement.

The cause of Lou Gehrig disease is not known, and scientists do not yet know why Lou Gehrig disease strikes some people and not others. Research believes that mutations in the gene that produces the SOD1 enzyme were associated with some cases of familial Lou Gehrig disease. . This enzyme is a powerful antioxidant that protects the body from damage caused by free radicals. Free radicals are highly reactive molecules produced by cells during normal metabolism. If not neutralized, free radicals can accumulate and cause random damage to the DNA and proteins within cells. Although it is not yet clear how the SOD1 gene mutation leads to motor neuron degeneration, researchers have theorized that an accumulation of free radicals may result from the faulty functioning of this gene. In support of this, animal studies have shown that motor neuron degeneration and deficits in motor function accompany the presence of the SOD1 mutation.

Studies also have focused on the role of glutamate in motor neuron degeneration. Glutamate is one of the chemical messengers or neurotransmitters in the brain. Scientists have found that, compared to healthy people, Lou Gehrig disease patients have higher levels of glutamate in the serum and spinal fluid. Laboratory studies have demonstrated that neurons begin to die off when they are exposed over long periods to excessive amounts of glutamate. Now, scientists are trying to understand what mechanisms lead to a build up of unneeded glutamate in the spinal fluid and how this imbalance could contribute to the development of Lou Gehrig disease.

Autoimmune responses—which occur when the body's immune system attacks normal cells—have been suggested as one possible cause for motor neuron degeneration in Lou Gehrig disease. Some scientists theorize that antibodies may directly or indirectly impair the function of motor neurons, interfering with the transmission of signals between the brain and muscles.

In searching for the cause of Lou Gehrig disease, researchers have also studied environmental factors such as exposure to toxic or infectious agents. Other research has examined the possible role of dietary deficiency or trauma. However, as of yet, there is insufficient evidence to implicate these factors as causes of ALS.

Symptoms:

The early symptoms of Lou Gehrig diseasemay seem rather vague. They can include tripping, dropping things, slurred or "thick" speech, and muscle cramping, weakening, and twitching. Some people with these early symptoms may tend to assume that they are normal signs of aging. As the disease progresses, the muscles of the trunk of the body are affected. Weakness of the breathing muscles develops slowly over months or years.

For some people, the muscles for speaking, swallowing or breathing are the first to be affected. This is known as Bulbar ALS. The term “bulbar” refers to the motor neurons located in the bulb region of the brain stem, that control the muscles used for chewing, swallowing, and speaking.

The rate of muscle loss can vary significantly from person to person, with some patients having long periods with very slow degeneration. Although the average life expectancy of newly diagnosed people is between 3 and 5 years, 20% live more than 5 years, and 10% live more than 10 years. Being a progressive disease, Lou Gehrig disease may spread throughout the body over time, and at some point in this process, may involve the muscles required for breathing.

Diagnosis:

Lou Gehrig disease can be difficult to diagnose in the early stages, when symptoms may mimic other conditions. However, there are clinical signs that can be indicative of a wasting of motor neurons in either the upper or lower portion of the spine.

In addition to a physical examination, people are often given an EMG, blood tests, a MRI (Magnetic Resonance Imaging), and other tests to search for the presence of other diseases that can look like Lou Gehrig disease.

Doctors who do not usually see people with Lou Gehrig disease may be reluctant to suggest it as a possible diagnosis. They normally refer the person to a neurologist. Telling someone and his or her family that the person may have Lou Gehrig disease requires a sensitive, understanding and compassionate manner.

Treatment:

There is no cure for Lou Gehrig disease. Treatment focuses on relieving symptoms and maintaining an optimal quality of life.

Pharmacological Treatment Options May Include:

    Riluzole (Rilutek®) is one of the few drugs so far proven to be effective against Lou Gehrig disease and may prevent progression and prolong life for a few months or so. Riluzole decreases the release of glutamate. Side effects may include the following dizziness, elevated liver enzymes, reduced leukocytes in the blood (granulocytopenia), and weakness.

  • Baclofen (Lioresol®) or tizanadine (Zanaflex®) may relieve spasticity. Side effects include increased weakness, sedation, and dizziness.

  • Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen or naproxen may relieve general discomfort. Due to potentially severe gastrointestinal and cardiovascular side effects, NSAIDs should only be used as instructed.

  • Tramadol (Ultram®) is often prescribed for pain relief.

Note: Risk and benefits of the drugs must be carefully discussed with your neurologist.

Physical therapy is an important part of treatment and helps to relieve cramping and muscular pain. Passive stretching helps to avoid permanent contraction of muscles (contractures) that may cause joint problems.

Other therapies, such as occupational therapy and speech therapy, are also used in treatment. Lou Gehrig disease patients require a diet of high-energy foods that are easy to swallow. Patients may benefit from a nutritionist. If the patient is not able to maintain adequate nutrition, a percutaneous endoscopic gastrostomy (PEG), or feeding tube, is usually inserted. This has been shown to prolong life in Lou Gehrig disease patients who are losing weight.

Some Lou Gehrig disease 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 (e.g., continuous positive airway pressure [CPAP], bilevel positive airway pressure [BiPAP]) may be used to improve the patient's quality of life).

Depression is very common among Lou Gehrig disease patients. Antidepressant medication and counseling can help patients and their families cope.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

Reference and Source are from:

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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