Description

Primary lateral sclerosis (PLS) is a type of motor neurone disease. A motor neuron disease affects the nerve cells in the brain that control movement. In PLS , the breakdown of the nerve cells causes weakness in the muscles that control the legs, arms and tongue.

The symptoms typically start with balance problems. People with PLS may move slowly, and are clumsy. Over time, the symptoms evolve to include to have problems with his hands and arms, followed by problems with chewing, swallowing, and speech. Less frequently, the disease can start with problems with speech and swallowing.

This rare disease can develop at any age, but it usually occurs between the ages of 40 and 60. PLS is more common in men than in women. Rarely, PLS can begin in early childhood. This subtype is known as juvenile primary lateral sclerosis. Juvenile PLS is caused by a change in a gene that is passed from parents to a child.

PLS is often confused with another, more common motor neuron disease called amyotrophic lateral sclerosis (ALS). While, possibly related to amyotrophic lateral sclerosis , PLS worse more slowly than the COMMONWEALTH . In the majority of people, PLS is not fatal.

Symptoms

The symptoms of primary lateral sclerosis usually take years to develop and get worse. The symptoms usually begin in the legs. Rarely, PLS begins with weakness in the tongue or hands. Then, the weakness gradually moves down the spinal cord to the legs. However, symptoms vary from person to person. Symptoms may include:

  • The stiffness, weakness, and muscle spasms in the legs. This is known as spasticity. Rarely, spasticity starts on a single leg and moves the arms, hands, tongue, and jaw.
  • Slowness in movements.
  • Shooting, being awkward and having trouble with the balance.
  • Hand clumsiness.
  • A hoarse voice, as well as slow, slurred speech, and drooling.
  • Difficulty chewing and swallowing.
  • Sometimes, frequent, rapid and intense changes in the emotions.
  • Rarely, breathing problems, and bladder problems in advanced disease.

When to see a doctor

Make an appointment to see a health care professional if you have persistent problems with stiffness, or weakness in the legs or arms. Also consult a health care professional if you have difficulty swallowing or speaking.

Take your child to a health professional if your child has muscle spasms, or seems to be losing the balance more often than usual.

Causes

In primary lateral sclerosis, nerve cells in the brain that control movement to slowly break down and stop working. These nerve cells are called upper motor neurons.

As a result, the nerves are not able to activate the motor neurons in the spinal cord that control voluntary muscles. This loss causes problems with movement. People with PLS may have problems with balance, weakness, slowness in movements and clumsiness. PLS also can cause problems with speech and swallowing.

Adult-onset primary lateral sclerosis

The cause of PLS that begins in adulthood is not known. In most people, the disease is not hereditary. It is not known why or how to start.

Juvenile primary lateral sclerosis

Juvenile PLS is caused by changes in a gene called ALS2.

The researchers do not know how this gene causes the disease. But they know that the ALS2 gene gives instructions for the creation of a protein called alsin. Alsin is present in the motor neurons of the cells. When the instructions are changed into someone with minors PLS , the protein alsin becomes unstable and not work properly. This affects the function of the muscle.

Juvenile PLS is an autosomal recessive hereditary disease. This means that both parents must be carriers of the gene to pass to his son. However, they do not need to have the disease.

Risk factors

Have not been established environmental risk factors for primary lateral sclerosis.

Complications

It may take up to 20 years to primary lateral sclerosis to progress and become worse. The symptoms vary from person to person. Some people continue to walk, but others might require the use of wheelchairs or other assistive devices.

For most people, adult PLS is not intended to shorten the life expectancy. But you can gradually affect the quality of your life as more muscles of the disability. Weak muscles can cause you to fall, which may result in injury. Problems with chewing and swallowing can result in poor nutrition. The performance of the activities of daily living, such as bathing and dressing, it can be hard.

People with PLS can develop problems with their way of thinking, known as cognitive impairment. Or they may have changes in their behavior. For some people, its symptoms overlap with the symptoms of frontotemporal dementia.

Diagnosis

There is No single test that confirms the diagnosis of primary lateral sclerosis (PLS). PLS can have symptoms similar to those of other neurological diseases such as multiple sclerosis and amyotrophic lateral sclerosis . For this reason, it is likely that you will have several tests to rule out other diseases.

A health care professional first, it takes a careful record of your medical history and family history and perform a neurological examination. Then you may have the following tests:

  • Analysis of blood. The blood tests check for infections or other possible causes of muscle weakness.
  • The magnetic resonance imaging. An mri or other imaging tests of your brain or the spine can reveal signs of neural cells have been broken. An mri also may show other causes of symptoms, such as structural problems, multiple sclerosis or spinal cord tumors.
  • Electromyogram (EMG).The test evaluates the electrical activity of your muscles when they contract and when they are at rest. This test also measures the participation of lower motor neurons. This may help to explain the difference betweenPLS⸺ a disease of the upper motor neuron ⸺ andALS, which affects the upper and lower motor neurons. During anEMG, the doctor inserts a needle electrode through your skin into various muscles.
  • Nerve conduction studies. This test can determine if you have nerve damage. A low amount of electric current measures of your nerves, the ability to send impulses to the muscles in different areas of your body.
  • Lumbar puncture, also known as a spinal tap. A lumbar puncture can help to rule out multiple sclerosis, infections, and other diseases. A thin, hollow needle inserted into the spinal canal removes small samples of cerebrospinal fluid. This is the fluid that surrounds the brain and the spinal cord. The samples are analyzed in a laboratory.

Electromyogram (EMG). The test evaluates the electrical activity of your muscles when they contract and when they are at rest. This test also measures the participation of lower motor neurons. This may help to explain the difference between PLS ⸺ a disease of the upper motor neuron ⸺ and ALS , which affects the upper and lower motor neurons.

During an EMG , your doctor inserts a needle electrode through your skin into various muscles.

Sometimes it takes 3 to 4 years before a diagnosis can be made. This is because the first COMMONWEALTH may seem PLS to other symptoms of the surface of a couple of years later. You can ask to repeat EMG's more than 3 to 4 years before a PLS diagnosis is confirmed.

Genetic testing can be done when the juvenile PLS is suspected. Genetic counseling can also be recommended.

Treatment

There are No treatments to prevent, halt, or reverse the primary lateral sclerosis. The treatment focuses on relieving the symptoms and the preservation of the function.

Medicine

You can take medication to relieve these symptoms:

  • Muscle spasms, known as spasticity.Muscle spasms can be relieved by baclofen (Fleqsuvy, Ozobax, Lyvispah), tizanidine (Zanaflex), or clonazepam (Klonopin). These medications are taken by mouth. If the muscle spasms can not be controlled with oral medications, your health care professional may recommend surgery to implant a pump of medicine. The delivery of the pump baclofen directly into the spinal fluid.
  • The emotional changes. You may be prescribed an antidepressant if you have frequent, rapid and intense changes in the emotions.
  • Drooling. Drooling can be helped by amitriptyline or other drugs.

Muscle spasms, known as spasticity. Muscle spasms can be relieved by baclofen (Fleqsuvy, Ozobax, Lyvispah), tizanidine (Zanaflex), or clonazepam (Klonopin). These medications are taken by mouth.

If the muscle spasms can not be controlled with oral medications, your health care professional may recommend surgery to implant a pump of medicine. The delivery of the pump baclofen directly into the spinal fluid.

Physical and occupational therapy

Stretching and strengthening exercises can help to maintain muscle strength, flexibility and range of motion. The exercises may also help prevent the immobility of the joint.

Speech and language therapy

Speech therapy can help with communication and swallowing if your facial muscles are affected by PLS .

Nutritional support

If you have difficulty chewing and swallowing, a dietitian can offer tips for food, nutritional supplements, or special methods of feeding. This can help you to maintain your body weight when the symptoms become difficult to eat.

Assistive devices

Like PLS symptoms get worse, you may need an assistive device. Physical therapists, occupational therapists can assess on a regular basis to determine if you need a brace, cane, walker, or wheelchair. Assistive technology devices can also help with the communication.

Coping and support

It is expected to have a range of emotions when you have primary lateral sclerosis. To cope with the illness and its effects, keep these tips in mind:

  • Seek emotional support. Family and friends can be a great source of comfort and support. It might be helpful to see how others cope with illness by joining a support group. Because PLS is not common, it can be a challenge to find a local support group. But some online discussion groups are available. Your health care team may have suggestions.
  • Seek professional help if you need it. Living with a chronic illness can be overwhelming at times. Seek professional advice for the other point of view or if you have depression and need advice on treatment.
  • Know and use the resources available to you. If PLS is the restriction of their activities, talk to a health professional about devices that are designed to help you stay independent. Social services also works with people with disabilities of all kinds. Try to learn all you can about the resources available to you. Sometimes, trusting in his community to help can strengthen the ties in new ways.
Symptoms and treatment of Primary lateral sclerosis (PLS)