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Amyotrophic lateral sclerosis
Transcript of Amyotrophic lateral sclerosis
1 out of 10 cases of ALS are due to a genetic defect. The other times, the cause is unknown.
In ALS, nerve cells (neurons) waste away or die, and can no longer send messages to muscles. This eventually leads to muscle weakening, twitching, and an inability to move the arms, legs, and body. The condition slowly gets worse. When the muscles in the chest area stop working, it becomes hard or impossible to breathe.
ALS affects approximately 5 out of every 100,000 people worldwide.
There are no known risk factors, except for having a family member who has a hereditary form of the disease.
Causes, incidence, and risk factors Symptoms usually do not develop until after age 50, but they can start in younger people. A person with ALS have a loss of muscle strength and coordination that eventually gets worse and makes it impossible to do routine tasks such as going up steps, getting out of a chair, or swallowing.
Breathing or swallowing muscles may be the first muscles affected. As the disease gets worse, more muscle groups develop problems.
ALS does not affect the senses (sight, smell, taste, hearing, touch). It only rarely affects bladder or bowel function, eye movement, or a person's ability to think or reason.
Symptoms The physical exam may show:
•Weakness, often beginning in one area,Muscle tremors, spasms, twitching, or loss of muscle tissue
•Twitching of the tongue (common),Abnormal reflexes,Stiff or clumsy walk,Increased reflexes at the joints,Difficulty controlling crying or laughing (sometimes called emotional incontinence),Loss of gag reflex
Tests that may be done include:Blood tests to rule out other conditions,Breathing test to see if lung muscles are affected,Cervical spine CT or MRI to be sure there is no disease or injury to the neck, which can mimic ALS,Electromyography to see which nerves or muscles do not work properly,Genetic testing, if there is a family history of ALS,Head CT or MRI to rule out other conditions,Swallowing studies,Spinal tap (lumbar puncture)
Signs and tests There is no known cure for ALS. A medicine called riluzole helps slow down the symptoms and lets you live longer.
Treatments to control other symptoms include:
•Baclofen or diazepam for spasticity that interferes with daily activities
•Trihexyphenidyl or amitriptyline for people with problems swallowing their own salivaPhysical therapy, rehabilitation, use of braces or a wheelchair, or other measures may be needed to help with muscle function and general health.hoking is common. Patients may decide to have a tube placed into their stomach for feeding. This is called a gastrostomy.
A nutritionist is very important. Patients with ALS tend to lose weight. The illness itself increases the need for food and calories. At the same time, problems with swallowing make it hard to eat enough.
Breathing devices include machines that are used only at night, and constant mechanical ventilation .Patients should discuss their wishes regarding artificial ventilation with their families and doctors.