Primary Lateral Sclerosis

When your voluntary muscles feel weak it is called primary lateral sclerosis (PLS). These voluntary muscles such as those you use to control your arms, tongue and legs. This is a type of motor neuron disease that can cause your muscle nerve cells to break down slowly which leads to weakness. Mostly this occurs between ages 40 and 60 but it can happen to anyone at any age.

Juvenile primary lateral sclerosis develops in childhood and the cause is an abnormal gene that was passed to children by their parents.

Sometimes PLS is mistaken to a disease called amyotrophic lateral sclerosis (ALS) that is the more common motor neuron disease but PLS in mostly is not fatal.

The signs and symptoms of primary lateral sclerosis (PLS) include:

• stiffness, spasticity and weakness in your legs,
• tripping, difficulty with balance,
• clumsiness as the leg muscles weaken,
• hoarseness,
• reduced rate of speaking,
• slurred speech and drooling as the facial muscles weaken,
• stiffness and weakness progressing to your trunk then your arms, hands, tongue and jaw,
• difficulty with breathing,
• difficulty with swallowing.

Often times PLS begin in your hands or tongue then progress down to your spinal cord to your legs. Consult your doctor if you have persistent problems with stiffness or weakness with speaking and swallowing or in your legs. Consult your child’s pediatrician if he develops involuntary muscle spasms or he is losing balance.

The causes of primary lateral sclerosis (PLS) vary depending on its type.

When nerve cells in the brain that is controlling the movement fails over time PLS occurs. This will cause movements problems such as balance problems, clumsiness and slow movements.

Adult primary lateral sclerosis cause is unknown. Mostly they do not know where it begins and it is not inherited.

A mutation in a gene called ALS2 is the cause of juvenile primary lateral sclerosis. Researchers only know that the ALS2 is responsible for creating a protein called alsin but they do not understand how this gene causes this disease. The protein alsin doesn’t work properly and becomes unstable when the instructions are changed in a person with juvenile PLS; this will damage normal muscle function.

Juvenile primary lateral sclerosis is an autosomal recessive disease in which both parents have to be carriers of the gene for the child to inherit it.

There is no single test for primary lateral sclerosis (PLS) diagnosis. Some of the signs and symptoms are similar to the signs and symptoms of other neurological diseases such as ALS or multiple sclerosis.

You may undergo different tests to rule out other conditions such as:

• blood work – to check for infections or other possible causes of muscle weakness;
• electromyogram (EMG) – this is done by inserting electrode through your skin into your muscles, your doctor will see the electrical activity of your muscles when they are at rest or contract. This can also check the lower motor neutrons to differentiate between ALS and PLS;
• MRI – this can reveal signs of nerve cell degeneration and to check for other possible causes of your symptoms such as multiple sclerosis;
• nerve conduction studies – to measure your nerve’s ability to send impulses to muscles and can determine if you have nerve damage;
• evoked potentials – this is done by attaching electrodes in your scalp and in different parts of your body to record your brain’s response to muscle stimulation, this can also evaluate degenerative nerve conditions;
• spinal tap or lumbar puncture – your doctor will remove tiny samples of the cerebrospinal fluid for laboratory analysis and this can rule out infections, multiple sclerosis and other conditions;
• position emission tomography (PET) – to check for degenerative changes in the brain and diagnose PLS.

Sometimes early diagnosis of ALS can look just like PLS until additional symptoms will develop after a few years later, it will be after three to four years until PLS is confirmed.

There are no treatments to cure or prevent primary lateral sclerosis (PLS), the treatment will only focus on relieving the symptoms and preserving function:

• medication – such as baclofen, tizanidine (Zanaflex) or clonazepam (Klonopin) to relieve muscle spasms or spasticity which is taken orally. If the oral medicine does not work, your doctor may suggest surgically implanting a medication pump to give baclofen to your spinal fluid (intrathecal baclofen). Your doctor may give you antidepressants for depression and amitriptyline to help the drooling problems.
• physical therapy – strengthening and stretching exercises to maintain flexibility, muscle strength and range of motion, this can also be done to prevent immobility.
• speech therapy – to help you with your facial muscle problems and speech.
• assistive devices – an occupational or physical therapist will check if you need assistive devices such as cane.

There are no preventions that might stop or slow the progress of primary lateral sclerosis (PLS).

There are no homeopathic remedies for primary lateral sclerosis (PLS).

Lifestyle modifications are necessary in order to cope with primary lateral sclerosis (PLS).

You can follow some of these tips to preserve your muscle function:

• Stay active – join exercise programs, try to stay active to keep your existing function and can help in slowing the progression of the disease but be careful while doing activities.
• Eat a healthy and nutritious diet – this is done to avoid excessive weight gain that can add pressure to your joints.

Dealing with this disease can be challenging and scary, follow some of these tips to cope with this disease:

• Seek emotional support from your family and friends, they can be great source of comfort. You can also join online discussion groups because local support group may be hard to find because this disease is uncommon.
• Get professional counseling or if you are struggling with anxiety and depression or if you need advice on treatments;
• consult your doctor about devices designed to help you stay independent.
• There are also social services available for people with disabilities of all kinds.

There are several risks and complications associated with primary lateral sclerosis (PLS).

y variable effects from person to person although the average progression of primary lateral sclerosis lasts up around 20 years. Others will need to use wheelchairs but others may continue to walk.

Having PLS will not shorten your life expectancy but it will affect your everyday life as more of the muscles become disabled.