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What Comes First In Als Muscle Weakness Or Twitching

by Kristin Beck
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What Comes First In Als Muscle Weakness Or Twitching

What Comes First In Als Muscle Weakness Or Twitching

“ALS is an incurable degenerative disease that destroys nerve cells in the brain and spinal cord causing motor neurons to die off. The cause is unknown but it has been linked with genetic factors, environmental toxins, infections, head trauma, and autoimmunity. It affects about one person out every 5,000 worldwide. There are two types – 1) Primary lateral sclerosis (PLS), which accounts for 90 percent of all cases; 2) progressive muscular atrophy (PMA), also known as pseudobulbar palsy, which accounts for 10 percent of all cases.
Most people who develop ALS have no family history of the disease. Some rare forms can run in families. About half of those diagnosed with ALS do not have any family members with the condition. Symptoms usually begin between the ages 40-60 years old but children under 20 rarely get ALS. Men are affected more than women by a ratio of 4:1. Most patients survive from six months to several years after their diagnosis. Those who live longer than five years average three additional years of life.
Muscles involved in ALS gradually weaken over time until they become weak and flaccid. This results in loss of movement control, balance problems, poor coordination, trouble walking, speaking, eating, and eventually paralysis. As muscles waste away, spasticity occurs when muscles tighten up involuntarily making them hard and rigid. Spasms affect muscles throughout the body including legs, arms, neck, and shoulders.
People with ALS often experience drooling due to lack of saliva production. Excessive sweating can occur on the face, upper lip, and forehead. Breathing becomes labored because diaphragm movements cannot move air through the lungs. Swallowing is difficult because nerves controlling this function are damaged. Speech becomes impaired leading to frequent mispronunciations and misspellings. These symptoms can lead to severe depression and anxiety.
About 30 percent of those diagnosed with ALS will experience dementia. Difficulty breathing during sleep leads to snoring and sleeping pills can help some sufferers fall asleep. Others develop constipation and bowel disorders. Diarrhea and fecal incontinence are common. Many people with ALS lose bladder control. They must wear diapers or use catheters. Loss of sexual desire is commonly experienced. Sexual dysfunction is common in both men and women.
Symptoms associated with ALS typically worsen over time. Therefore, early detection is vital. If you think you might have ALS, contact your doctor immediately. Diagnosis is made most often through neurological examination. Blood tests and imaging studies such as MRI’s are done to rule out other conditions that could mimic ALS. A lumbar puncture is used to collect fluid around the spine for further testing. Electrodiagnostic studies such as EMG’s are performed to detect damage to peripheral nerves. Genetic testing can confirm the diagnosis if necessary.
Treatment options vary depending upon where the illness begins in the nervous system. Medications can improve symptoms and slow progression. Physical therapy can improve strength, mobility, and range of motion. Assistive devices such as braces, walkers, wheelchairs, and scooters can provide needed support. Respiratory assistance devices such as CPAP machines, bi-level positive airway pressure machines, oxygen tanks, and respiratory assistive devices are helpful. Speech therapists can teach individuals how to speak using alternative methods like sign language. Occupational therapists and physical and rehabilitation assistants can suggest activities suitable to ones needs and abilities. Social workers and psychologists can aid in dealing with emotional stress and loss. Hospice care can ease suffering at end stages of the disease.
No cure exists for ALS. However, research continues to identify potential therapies that target various aspects of ALS such as genetics, neuroprotection, and stem cell transplantation. Scientists continue to search for ways to stop or slow down the progress of ALS. Recent breakthroughs in the field of gene therapy have led researchers to explore whether inserting healthy genes into the DNA of patients can reverse or halt the progress of ALS. One promising new approach involves modifying TDP-43, a protein that normally binds together RNA molecules and helps create messenger ribonucleoproteins (mRNPs). When mutated, TDP-43 appears in areas of the brain that control motor functions. Researchers hypothesize that introducing the normal version of TDP-43 back into these areas might restore lost function. Another group is studying a protein called valosin-containing protein (VCP) which has been found to play a role in ALS. VCP activates proteins within a cell called ubiquitin. Researchers want to know if activating VCP can prevent the formation of abnormal proteins that contribute to ALS.
ALS remains a debilitating disorder without a cure. Early detection is critical. With proper treatment, many sufferers can maintain quality of life well beyond diagnosis. For more information see http://www.alsinfo.org/.”

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