Primary lateral sclerosis (PLS)
Find out why Mayo Clinic is the right place for your health care. Make an appointment..
Find a directory of doctors and departments at all Mayo Clinic campuses. Visit now..
See how Mayo Clinic research and clinical trials advance the science of medicine and improve patient care. Explore now..
Educators at Mayo Clinic train tomorrow’s leaders to deliver compassionate, high-value, safe patient care. Choose a degree..
Explore Mayo Clinic’s many resources and see jobs available for medical professionals. Get updates..
Your support accelerates powerful innovations in patient care, research and education. Give today..
Primary lateral sclerosis is a type of motor neuron disease that causes muscle nerve cells to slowly break down, causing weakness. 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 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.
Make an appointment to see your doctor if you have persistent problems with stiffness or weakness in your legs, or with swallowing or speaking.
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), the nerve cells in the brain that control movement fail over time. This loss causes movement problems, such as slow movements, balance problems and clumsiness.
The cause of adult primary lateral sclerosis is unknown. In most cases, it’s not an inherited disease, and it’s not known why or how it begins.
Juvenile primary lateral sclerosis is caused by mutations in a gene called ALS2.
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.
Mayo Clinic does not endorse companies or products. Advertising revenue supports our not-for-profit mission.
Check out these best-sellers and special offers on books and newsletters from Mayo Clinic.
Any use of this site constitutes your agreement to the Terms and Conditions and Privacy Policy linked below.
A single copy of these materials may be reprinted for noncommercial personal use only. “Mayo,” “Mayo Clinic,” “MayoClinic.org,” “Mayo Clinic Healthy Living,” and the triple-shield Mayo Clinic logo are trademarks of Mayo Foundation for Medical Education and Research.
This site complies with the HONcode standard for trustworthy health information:
verify here.
Primary lateral sclerosis (PLS)
Research & References of Primary lateral sclerosis (PLS)|A&C Accounting And Tax Services
Source
0 Comments