Primary Lateral Sclerosis (PLS)

What is Primary Lateral Sclerosis?

Primary Lateral Sclerosisis also called PLS and central motor neuron disease of voluntary muscles. The cause of PLS is unknown but symptoms usually begin when the patient is between 40 to 60 years old. Patients are usually men. Just why men are affected more than women is still unknown.

Doctors are not quick to diagnose PLS. The National Institute of Neurological Diseases and Stroke note that doctors may take as long as 4 years in order to diagnose a PLS patient. This long time to reach a diagnosis is for two reasons:

The first is that symptoms come on so gradually that other conditions may be suspected.

The second is that the first symptoms are similar to other conditions, including other kinds of neurological diseases.

What are the Symptoms of Primary Lateral Sclerosis?

The good news is that PLS is mostly painless physically. Coordination is gradually lost in the body. The legs are usually the first affected. They become weak and stiff so walking or standing is difficult. For some patients, the tongue may be first affected. For others it is the hands. Wherever it starts, moving the affected body part is difficult. As the disease slowly progresses, other body parts are affected.

In the worse cases, the face, tongue, hands and legs are difficult to move or coordinate. They may jerk about spastically. The patients may no longer be able to talk, suffer urine incontinence. The worst symptom is the inability to swallow and the person loses the ability to eat. Liquid food is given via a tube directly to the stomach.

How is Primary Lateral Sclerosis Treated?

There is no cure for PLS. However, unlike many other types of neuromuscular disease, PLS is not fatal because it does not interfere with breathing. Most patients go on to live out their full life span. Some even retain their ability to walk without even a cane to support them. Others are able to live by themselves without the need to hire a live-in nurse.

Individual symptoms are treated depending on the needs of each patient. Speech therapy helps some retain the ability to communicate. Wheelchairs, rollators, braces and canes help others to move about. Spastic motions may be treated with a type of medications called muscle relaxants.

It is normal for a PLS patient to become depressed before or after diagnosis. Anti-depressants are often prescribed to help the patient cope with the emotional impact of PLS.

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Last Reviewed:
October 09, 2016
Last Updated:
August 29, 2017
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