Amyotrophic Lateral Sclerosis (ALS)
What is amyotrophic lateral sclerosis?
Amyotrophic lateral sclerosis (ALS, sometimes called Lou Gehrig's Disease) is a progressive, fatal, neurodegenerative disease caused by the degeneration of motor neurons, the nerve cells in the central nervous system that control voluntary muscle movement. The disorder causes muscle weakness and atrophy throughout the body as both the upper and lower motor neurons degenerate and die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, atrophy, and develop fasciculations (twitches) because of denervation. Eventually, the brain completely loses its ability to initiate and control voluntary movement. The disease does not necessarily debilitate the patient's mental functioning in the same manner as Alzheimer's disease or other neurological conditions. Rather, those suffering advanced stages of the disease may retain the same memories, personality, and intelligence they had before its onset.
In the United States and most other parts of the world, 1 to 2 people per 100,000 develop ALS each year. Men are affected slightly more often than women. Although ALS may occur at any age, it is most common in middle-aged and older adults.
What causes ALS?
The cause of ALS is unknown. About 5% to 10% of people with ALS have an inherited form of the disease. ALS is not contagious.
What are the symptoms?
The symptoms of amyotrophic lateral sclerosis (ALS) include:
It is important to remember that having muscle weakness, fatigue, stiffness, and twitching doesn't necessarily mean that you have ALS.
The first sign of ALS is usually slight weakness in one leg, one hand, the face, or the tongue. Other problems may include increasing clumsiness and difficulty performing tasks that require precise movements of the fingers and hands. Muscle twitching may also occur. The weakness slowly spreads to the arms and legs over a period of months or years. As the nerves continue to waste away and decrease in number, the muscle cells that would normally be stimulated by those nerves also start to waste away, and the muscles weaken.
ALS is a progressive, disabling disease. Walking, speaking, eating, swallowing, breathing, and other basic functions become more difficult with time. These problems can lead to injury, illness, and other complications. Respiratory problems are the most common serious complication of ALS. As the muscles in the throat and chest area become weak, swallowing and coughing become more difficult, which may sometimes cause food and saliva to be inhaled into the windpipe (aspiration). This may lead to pneumonia. Breathing problems tend to get worse as the disease progresses, increasing the risk of infection and respiratory failure.
Pneumonia, pulmonary embolism, lung failure, and heart failure (probably due to lack of adequate breathing as the chest muscles weaken) are the most common causes of death among people with ALS. In most cases, death will occur within 3 to 6 years after symptoms begin, although some people live for many years, even decades.
How is ALS diagnosed?
A diagnosis of amyotrophic lateral sclerosis (ALS) is based on a careful medical history, a physical exam of the nervous system, and tests that evaluate nerve and muscle function.
If your health professional suspects ALS, you may be referred to a neurologist for diagnosis and treatment. A medical history and detailed physical exam of the nervous system can usually help the neurologist diagnose your condition. Tests needed to confirm the diagnosis include electromyogram (EMG) and nerve conduction studies. EMG helps measure how well and how quickly the nerves and muscles are functioning. Nerve conduction studies test nerve function.
Depending on your doctor's findings during the nervous system exam and the results of EMG or nerve conduction studies, other tests may be needed to rule out other possible causes of your symptoms. These tests may include a muscle biopsy (tissue sample), blood tests, or imaging tests, such as a computed tomography (CT) scan or magnetic resonance imaging (MRI).
ALS may be difficult to diagnose, and the diagnosis may not become clear until symptoms have progressed or until additional testing and observation have taken place. If ALS is suspected, evaluation by a specialist who has experience treating and diagnosing ALS may lead to an earlier diagnosis.
Although it does not affect the course or outcome of the disease, an early diagnosis may:
How is it treated?
While there is no cure for amyotrophic lateral sclerosis (ALS), treatment can help you maintain strength and independence, manage symptoms, and avoid complications for as long as possible. Treatment also focuses on providing emotional support as disability increases.
Physical therapy and occupational therapy may help you maintain strength and function and make the most of your remaining abilities. Speech therapy can help maintain your ability to communicate as speaking problems develop.
Medications may be used to relieve symptoms and make you more comfortable. These include:
If you are having problems with food and saliva being inhaled into the windpipe, a tube can be inserted through the skin into the stomach (percutaneous endoscopic gastrostomy, or PEG). The tube provides an easier way to get adequate nutrition and take medications.
Breathing devices (ventilators) may be needed as the muscles of the chest become affected. Initially, extra oxygen can be given through a nasal cannula, a flexible plastic tube that is placed in the nostrils. For people who have mild breathing problems, nonsurgical forms of ventilation may be used. These include bilevel positive airway pressure (BiPAP) and noninvasive positive pressure ventilation (NPPV), which deliver air or extra oxygen through a face mask. However, in some cases, an opening in the neck leading to the windpipe (tracheostomy) may have to be created and oxygen delivered through a tube in the opening.
At first, breathing devices may be needed only part of the time, such as when you are sleeping. As the disease gets worse and breathing problems increase, you may need to be on a breathing machine all the time.
Riluzole
A medication called riluzole (Rilutek) may prolong survival by a few months. It is not known exactly how the medication works, but it may slow the release of certain brain chemicals (neurotransmitters) that are believed to play a role in ALS. Riluzole is the only medication approved for the treatment of ALS.
There are some disadvantages to taking riluzole. While it has been shown to prolong survival for a few months, it does not improve symptoms or quality of life in ways that people with ALS, their caregivers, or their doctors have been able to detect. Most people tolerate riluzole very well, but it can cause side effects, including nausea, vomiting, weakness, dizziness, and coughing. Because riluzole can cause liver problems, people taking the drug need to have their liver function checked on a regular basis. Treatment with riluzole is also expensive, and it may not help some people who have certain forms of ALS.
What decisions might I face as ALS progresses?
If you (or someone in your family) have ALS, learn as much as you can about the disease and your options for care. What you value and want are important factors in making choices about your care. As ALS progresses, you may face decisions about how aggressively you want to treat complications, such as breathing problems, difficulty eating, or pneumonia. As you make these decisions, remember that what's right for one person with ALS may not feel right for another person.
As the muscles that control swallowing become weaker, some people have problems with food and saliva being inhaled into the windpipe. If this happens, you may choose to have a tube surgically inserted through the skin into the stomach (percutaneous endoscopic gastrostomy, or PEG) as a way to get adequate nutrition and take medications.
Help with breathing may be needed as the muscles of the chest become weaker. Initially, extra oxygen can be given through a nasal cannula, a flexible plastic tube that is placed in the nostrils. For mild breathing problems, machines that deliver air or extra oxygen through a face mask may be used. These machines are called bilevel positive airway pressure (BiPAP) or noninvasive positive pressure ventilation (NPPV) machines. For severe breathing problems, a surgical opening in the neck leading to the windpipe (tracheostomy) may be created so oxygen can be delivered through a tube in the tracheostomy. As ALS gets worse and breathing problems increase, some people choose to support their breathing by being on a breathing machine, or ventilator, all the time.
How aggressively to treat the problems from ALS is a personal decision that only you and your loved ones can make. Regardless of your choices, your comfort can be maintained. It is important to discuss treatment options and share your concerns with your doctor.
Another important decision is whether to pursue litigation. Brain injury lawyers in Pennsylvania and New Jersey can help. Personal Injury attorneys are available.
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