ALS is a disease with no known cause and no cure yet —Prof. Owolabi

Professor Mayowa Owolabi is a neurologist and Dean, Faculty of Clinical Sciences, University of Ibadan and also the Regional Director (Sub-Saharan Africa), World Hypertension League. In this report by Sade Oguntola , he speaks on ALS, a rare disease that gradually shuts down its patient’s ability to move, including swallowing and movement of food down the intestines. Excerpt:

 

A colleague’s friend recently died of what doctors diagnosed as Amyotrophic Lateral Sclerosis (ALS). What is ALS and what are its symptoms?

Amyotrophic Lateral Sclerosis (ALS) is the most common type of  motor neuron disease resulting into hardening of neurons from the brain through the spinal cord, and eventual wasting and weakness of muscles in the body. What do we mean by motor neuron disease? First, it is important to understand what motor neurons are. Generally, there are three types of neurons. The sensory neurons, the motor neurons and intermediary neurons. The sensory neurons help us to perceive our environment.

The motor neurons are those that help our body to respond to the environment through movements. These include the movement of any body parts, including the hand, legs, intestine, eyelids or even the pupils in the eye. The movements of the parts of the body are due to the activities of the motor neurons on our muscles. Muscles become wasted and weak when they are not being stimulated by motor neurons.

Basically, when there is a problem that affects only motor neurons, it is referred to as a motor neuron disease.

There are two major types of motor neurons: the upper and lower motor neurons. The lower motor neurons are the ones that go into the muscles directly. The upper motor neurons are the ones that originate most of the time from the brain, which travel through the brainstem and/or spinal cord, and end up on the lower motor neuron within the brainstem and/or spinal cord. But, unlike the lower motor neurons, it does not end in the muscles.

So, any disease of motor neuron, whether it is an upper motor neuron or lower motor neuron is called motor neuron disease.

It is a major problem because it affects muscle contractions in different parts of  the body, thereby impairting movement, including swallowing and movement of food down the intestines as well.

It can affect the upper motor and the lower motor neurons at the same time, at multiple levels. For instance, at the level of the head, swallowing, breathing and muscles of the face may be affected. Also, movements of the hands and legs are affected. There will be cramps, weakness of the limbs such that mobility becomes really difficult.

But that is not all. It is called Amyotrophic lateral sclerosis because a portion of the spinal cord is also hardened, that is the portion where upper motor neurons from the brain travel through.

 

Generally what are its common symptoms, you already indicated movement?

Very rarely sometimes, it can affect memory and behaviour. Some experience depression too. Chewing is affected. The tongue, in some instances, becomes shrunken; even the speech sometimes becomes slurred as if the tongue is stuck to the floor of the mouth or as if the patient is eating a hot meal. In addition, while trying to swallow, food might come out from the nose. If food goes into the lungs, it causes infection.

 

How treatable is it, can it be made to go into remission?

It does not, unfortunately. Once it starts, it is a progressive disease. Once the motor neurons are affected, the damage continues. Nobody knows exactly why it happens. There are so many factors responsible for motor neurons damage. In some instances, it is due to viruses. For instance, polio virus causes motor neuron disease. Paralytic polio affects only the lower motor neurons, causing paralysis and wasting of the limbs.

In some instances, environmental toxins and pollutants, including exposure to heavy metals, pesticides and petrol fumes are contributory to causing motor neuron disease. Very rarely, it is due to genetic problems. About five per cent of cases are due to a genetic problem. Certainly ensuring protection from environmental pollutants may reduce an individual’s chances of developing a motor neuron disease, but science is yet to understand its causes.

Currently, there are only two drugs that have been found to slow down its progression and prolong life.  Alongside the drugs, symptomatic treatment and rehabilitation are given. Physiotherapy is provided for weak muscles. For breathing difficulty, chest physiotherapy and oxygen therapy are required. Cognitive therapy is provided for memory issues or depression; speech therapy for speech problems; and painkillers for pain and to reduce muscle spasms.

 

Is there anything that one can do to prevent it?

It is very difficult. I think the best that can be done is for everyone to do a lot about our environment, and what we eat; to ensure that we breathe clean air; we have potable  water that doesn’t have heavy metals or any other toxins just to be sure that we are not exposed to environmental toxins as much as possible.

 

How common is this disease in Nigeria?

It is rare anywhere in the world; it is not very common. The chances of having it are 1 in 400 in a lifetime. It’s usually more in the middle-aged between 20 and 40. It is very rare in the elderly. It generally affects more males than females.

 

How easy is its diagnosis? Is it a disease that is easily mistaken for another one?

It is not a disease that is easily picked up because there are so many other causes of muscle weakness, wasting and spasms.  A neurologist is required to make its diagnosis. It will require running different medical tests to rule out other medical conditions that may look like Amyotrophic Lateral Sclerosis. This would include such tests as a brain and spine scan, electromyography and so on.

 

Is treatment available for ALS in Nigeria?

It can be managed here in Nigeria. Basically it is about neuro-rehabilitation and symptomatic treatment. The only two medications that have been found to be useful can be imported, so that is not a problem. Actually, a neurorehabilitation facility where you have the full complement of speech therapists, psychotherapists, physiotherapists, and so on, in Nigeria, will handle the condition.

 

What is the chance that a person diagnosed with the disease will still be alive in 5 years?

Motor neuron disease is like an umbrella for so many diseases that end up that way. So the prognosis is variable, but usually when it happens and you’ve established a diagnosis, the life expectancy, so to speak, is just a matter of years. If the person is lucky, maybe up to 10 years; and extremely rarely maybe 20 years if it’s of childhood onset, with good care, including respiratory support and all sorts of medical and nursing care. Every other thing is symptomatic treatment. It is very rare to have it and live for 10 years and beyond. Though a few live up to 30 years.

If the person can afford good care, including if possible respiratory support, skincare, treating spasms, treating infection, preventing infection, blood clots and all that, a person can live an improved quality of life. But in a weak health system, it may be tougher. It depends on if the person can afford what is required.

 

Why is it that people don’t know about this disease, we talk more about stroke and other diseases?

It is not a common disease. So that’s one of the reasons why people don’t know. It is not a disease that people do not have in Nigeria. It is just that they are few, and they are likely to be diagnosed at a tertiary centre where a neurologist assesses it.