Although a rare neurologic condition, Amyotrophic Lateral Sclerosis (ALS) is the most common type of Motor Neuron Disease (MND),  a condition that affects the voluntary muscles. This is a progressive disorder that leads to muscle weakness and depletion due to nerve dysfunction.

ALS is also called Lou Gehrig’s disease, named after the football player who had this condition. The literal meaning of Amyotrophic is ‘no muscle nourishment’ which becomes the cause of muscle atrophy. ‘Lateral’ refers to the group of nerves in the spinal cord that sends signals to the muscles. It is these nerves that degenerate, leading to sclerosis in this region. In later stages, this affects the nerves that control breathing and hence can be fatal.

The initial symptoms of ALS include stiffness and muscle weakness, which gradually involves all the muscles under voluntary control. The affected regions and progressive pattern vary from one person to another. Some having difficulty holding a pen or a cup while others finding difficulty speaking, chewing or even talking. Thus, ALS is an ailment that affects daily life and makes simple tasks painful and troublesome.

According to the Center for Disease Control and Prevention (CDC), 14,500 to 15,000 people had ALS in the United States in 2016,  with approximately 5000 people having a confirmed diagnosis for the condition annually. Although the average survival rate is three to five years, patients can live for ten years or more.

Are there different types of ALS?

Amyotrophic Lateral Sclerosis has two types:

Sporadic ALS: this is the most common one and affects 95% of sufferers. This type occurs without a clear cause.

Familial ALS (FALS): This type occurs in 5-10% of sufferers. This type of ALS is genetic and runs in families. This occurs due to abnormal changes to a gene that is then passed in generations. 

What are the symptoms of ALS?

Early signs and symptoms might be unnoticeable and become perceptible after some time. Most clinical signs are evident of upper motor neuron and lower motor neuron lesion. The limb onset ALS (70%) involves initial symptoms in the limbs while the bulbar onset ALS (25%) is characterized by speech and swallowing problems. This is followed by weakness in the limbs later. The remaining 5% of the patients have respiratory involvement in the early period. [5]

Most common symptoms include:

• Muscle weakness in the limbs (distal or proximal)
• Asymmetric progressive muscle wasting
• Difficulty in motor activities like walking, talking, chewing
• Weakness in arms, legs, hands, and feet
• Muscle cramps and twitching
• Slurred speech
• Fatigue
• Emotional liability (episodes of uncontrolled laughing and crying)
• Difficulty in maintaining posture and gait
• Difficulty in breathing and swallowing

With the progression of the disease, symptoms may spread to all parts of the body. In some patients, frontotemporal dementia may occur resulting in poor memory and decision-making abilities.

What Causes ALS?

The exact cause of ALS has not been known by scientists to date. However, research is being carried out to understand what causes ALS. There are several different factors such as: 

Genetic changes

Studies have shown that 5 to 10% of cases of ALS are caused by genetic mutations. For example, changes to the gene that makes SOD1 protein causes damage to motor neurons. 

Environmental factors

No major association has been established between environmental factors like toxins, viruses, diet or physical trauma and the risk of development of ALS. However, there is ongoing research on the subject. Studies have shown that some athletes are at a higher risk of acquiring ALS due to vigorous physical activity. 

Chemical disturbance

Glutamate is the neurotransmitter that is in control of signals to and from the brain. Accumulation of this neurotransmitter within the spaces surrounding the nerves damages them. 

Research has also shown mitochondrial structural and functional abnormalities, as well as defects in axonal structure and transport, could be the causative agents for ALS. 

How Do We Diagnosis ALS?

When it comes to diagnosis, there are no specific tests that can provide a definitive diagnosis for ALS. However, doctors conduct a series of tests to rule other similar diseases. A full medical history check and a neurologic examination are undertaken at regular intervals to assess the progressive worsening of symptoms. 

Running the following Image testing diagnostic tests can help reach a diagnosis:

• Electromyography (EMG) – EMG records the electrical activity of the muscle fibers.
• Nerve Conduction Study (NCS) – NCS assesses the electrical activity of the nerves and muscles.
• Magnetic Resonance Imaging (MRI) – MRI rules out other possible conditions such as a tumor or cyst in the spinal cord, cervical spondylosis, or a hernia in the neck that could be causing the nerve compression.

Laboratory tests such as blood screening and urine tests can also be carried out so that other diseases can be eliminated. 

What Are The Treatment Options & Management Strategies For ALS?

ALS is managed through a multidisciplinary approach.

Unfortunately, there is no definitive cure for the disease at this time. Management of ALS is done through symptomatic treatment to ease the condition of the patients and prevent unnecessary complications:

Support – Physicians, psychologists, speech therapists, nutritionists, and home care assistance all play a vital role in making life easier for patients with ALS.

Medication – Riluzole (Rilutek) and Edaravone (Radicava) are the drugs approved by the U.S Food and Drug Administration (FDA) for treating ALS. Riluzole is believed to reduce glutamate levels thereby, decreasing damage to the motor neurons. Edaravone acts an antioxidant and is believed to expel free radicals and reduce the oxidative stress in the motor neurons.

Lifestyle Habits – Physiotherapists can recommend exercise and physical activity like walking, swimming, and bicycling that may improve muscle strength and help elevate mood without overstressing the muscles.

Speech Therapy – therapists can help patients with ALS to employ strategies to speak clearly. They may also recommend computerized aids such as speech synthesizers and eye-tracking technology to help people learn ways for responding by nonverbal means.

Diet – Nutritionists may formulate a diet plan for patients, which consists of food that is easy to swallow and provides enough nourishment and calories for the patients to maintain adequate energy levels and to prevent excessive weight loss

Breathing Support – Patients with ALS may suffer from shortness of breath and difficulty breathing during physical activity or while lying down. If this is the case, doctors can recommend Non-Invasive Ventilation (NIV) that provides breathing support through the nose or mouth. NIV improves quality of life and increases the number of years of survival for patients.

Is Stem Cell Therapy An Option?

As previously mentioned, there is no curable treatment for ALS available. However, scientists are researching Stem Cell Therapy as the new favorable approach in the treatment of neurologic disorders.

There is a rising interest in Stem Cell Therapy as a promising remedy for curing ALS. Mesenchymal stem cells are particularly believed to be the most suitable ones due to their availability, absence of ethical issues and positive results in various experiments.

Studies and clinical trials have begun to apprehend the benefits of MSC transplantation. They demonstrate that MSCs lead to a partial recovery of motor neurons and a delay in disease progression. Also, there has been no evidence of a major adverse effect after MSC transplantation. 

When testing this newfound research on animals, the lifespan of the subjected animal has increased with MSC transplantation. These positive results have encouraged the administration of MSC in ALS patients. 

However, despite the safe outcomes of MSC transplantation in humans, results show that there is only a partial improvement in ALS sufferers with only a few cases that showed a delay in disease progression. Hence, there is a need for further studies and trials on a higher number of human subjects for a better understanding of MSC effects so that more significant conclusions can be reached.

One of the treatments that the FDA approved for knee arthritis is a Hyalronic Acid (HA) injection, sometimes also known as  Viscosupplementation. It has been incredibly successful for knee arthritis. In fact, so successful that many physicians are starting to use it on other parts of the body, like the hip and shoulder, which the FDA does not approve of.

HA, when injected, works like the fluid that naturally surrounds your joints. This fluid can be like a lubricant for your joints, and absorb shock, allowing bones that otherwise cause arthritis pain cause much less. Over time, it is even absorbed into the joint, which can cause the body to create a more stable cartilage all on its own.

The evidence for this treatment is astounding, with a systematic review of 76 trials, all of which were randomized controller trials. The review noted that HA, when injected, can benefit function, reduce pain, and can be a reliable and effective treatment for knee osteoarthritis.

On the other hand, there is PRP therapy. Platelet Rich Plasma, or PRP, which is a type of blood that has 6 to 10 times more platelets than what is normally found in blood. They even contain many growth factors, such as Epidermal Growth Factor, Connective Tissue Growth Factor, and many more. These can help heal injured parts of the body by using the bodies natural healing tools.

However, PRP is not regulated by the FDA, and devices that are used to make PRP require said approval. Aside from this, multiple studies shoe that PRP can be very effective in the treatment of tendon injuries, as well as for osteoarthritis. This treatment can even help in the reduction of pain. There are even more studies being conducted on whether it can help other things, such as hair regrowth, cardiac muscle repair, and even dermatologic rejuvenation.

So should you use HA injections, or PRP?

In many studies, PRP has bee demonstrated to work just as well, if not better than HA. HA is also only FDA approved for the knee, meaning that it is not approved or covered for the use in any other joint. Also, the risk of infection and rejection is far less while using PRP, as it is a substance that comes from your own body, and contains white blood cells, which can help fight infection.

PRP also saves money in the long run, as using HA in a joint other than the knee is not FDA approved or covered by insurance. As a result, it can cost your patient 1500$ or more. This can even be on top of various other charges, such as doctors visits, and even the injection itself. PRP, on the other hand, only costs from 800 to 1200$ out of pocket.

So PRP has been demonstrated to be just as effective, if not better, than HA injections when it comes to arthritis pain. It does not pose a risk for infection or and auto-immune reaction either, and is even far cheaper than HA. So picking which one to use should be a no-brainer.