Primary lateral sclerosis (PLS)
Primary lateral sclerosis (PLS)
Primary lateral sclerosis (PLS) causes weakness in your voluntary muscles, such as those you use to control your legs, arms and tongue. Primary lateral sclerosis is a type of motor neuron disease that causes muscle nerve cells to slowly break down, causing weakness.
Primary lateral sclerosis can happen at any age, but it usually occurs between ages 40 and 60. A subtype of primary lateral sclerosis, known as juvenile primary lateral sclerosis, begins in early childhood and is caused by an abnormal gene passed from parents to children.
Primary lateral sclerosis is often mistaken for another, more common motor neuron disease called amyotrophic lateral sclerosis (ALS). However, primary lateral sclerosis progresses more slowly than ALS, and in most cases isn't fatal.
Signs and symptoms of primary lateral sclerosis (PLS) usually take years to progress. They include:
Less commonly, PLS begins in your tongue or hands and then progresses down your spinal cord to your legs.
When to see a doctor
If your child develops involuntary muscle spasms or seems to be losing balance more often than usual, make an appointment with a pediatrician for an evaluation.
In primary lateral sclerosis (PLS), nerve cells in the brain that control movement fail over time. This loss causes movement problems, such as slow movements, balance problems and clumsiness.
Adult primary lateral sclerosis
Juvenile primary lateral sclerosis
Although researchers don't understand how this gene causes the disease, they know that the ALS2 gene is responsible for giving instructions for creating a protein called alsin, which is present in motor neuron cells.
When the instructions are changed in someone with juvenile PLS, the protein alsin becomes unstable and doesn't work properly, which in turn impairs normal muscle function. Adults who get primary lateral sclerosis don't appear to have the same gene mutation.
Juvenile primary lateral sclerosis is an autosomal recessive disease, meaning that both parents have to be carriers of the gene to pass it to their child, even though they don't have the disease themselves.
Although the average progression of primary lateral sclerosis (PLS) lasts around 20 years, the disease has highly variable effects from person to person. Some people may be able to continue to walk, but others will eventually need to use wheelchairs or other assistive devices.
Adult PLS isn't thought to shorten life expectancy, but it may gradually affect the quality of your life as more muscles become disabled. Weaker muscles may cause you to fall more, which may result in injuries.
Tests and diagnosis
There is no single test that confirms a diagnosis of primary lateral sclerosis (PLS). In fact, because the disease can mimic signs and symptoms of other neurological diseases such as multiple sclerosis and ALS, your doctor may order several tests to rule out other diseases.
After taking a careful record of your medical history and family history and performing a neurological examination, your doctor may order the following tests:
After other diseases are ruled out, your doctor may make a preliminary diagnosis of PLS.
Sometimes doctors wait three to four years before giving a diagnosis, because early amyotrophic lateral sclerosis (ALS) can look just like PLS until additional symptoms surface a few years later. You may be asked to return for repeat electromyography testing over three to four years before the PLS diagnosis is confirmed.
Treatments and drugs
The treatments for primary lateral sclerosis (PLS) focus on relieving symptoms and preserving function. There are no treatments to prevent, stop or reverse PLS. Treatments include:
Lifestyle and home remedies
Although there's no cure for primary lateral sclerosis, you can make a few lifestyle choices to preserve muscle function for as long as possible:
Coping and support
Periods of feeling down about having primary lateral sclerosis are expected and normal. Dealing with the reality of an incurable, progressive disease can be challenging. To cope with the disease and its effects, consider these tips:
Last Updated: 2013-07-12
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