ALS stands for amyotrophic lateral sclerosis, which is also known as motor neuron disease (MND) or Lou Gehrig's disease. It is a progressive neurodegenerative disease. According to disease fact sheets, the symptoms of this medical condition are 20% more common in men than in women, and 15 new cases are detected everyday in the US. It has been observed that 93% of patients in the ALS CARE database are Caucasian. The exact causative agent of ALS has not yet been determined. Genetics are factored in for the cause. A flaw associated with chromosome 21 is believed to be responsible in 20% of genetic cases. Investigations are still underway to unravel the contributing factors. Genetics or environmental factors are still fettered to the scope of conjecture.
Amyotrophic means 'no muscle nourishment'. Due to lack of nourishment, a muscle atrophies (withers or wastes away). The word 'lateral' describes the location. The commencement of this debility starts with the degeneration of the particular areas in a person's spinal cord. The important sections of the nerve cells that signal and control the muscles are located in the affected or degenerating area. Degeneration of this area results in scarring (hardening), and is therefore referred to as sclerosis.
Motor neurons carry signals from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. In case of an individual affected by ALS, the nerve cells in the brain and the spinal cord are affected. The progressive degeneration of the motor neurons ultimately ends in the death of the neurons. As a result, the brain is not able to initiate and control muscle movement. In the initial stages, progressive muscle weakness is usually experienced while in the later stages of the disease, patients may become totally paralyzed. It is assumed that mutations in the genes, elevated levels of glutamate in the serum and spinal fluid (reason behind this is not yet known), and autoimmune responses (the body's immune system itself starts destroying its healthy cells) can lead to ALS.
Advancement of the Symptoms
The early symptoms are usually overlooked or go unnoticed. They may vary from person to person. These can include tripping over carpet edges, trouble lifting grocery bags, or even slurred speech. The progression of symptoms varies from patient to patient. In very few people, ALS is known to go into remission or to stunt. Researchers are trying their best to find out the reasons for stunted progression. The advancement of the disease can be divided into stages:
- The initial symptoms might be as simple as muscle weakness in one or both hands, arms, or legs.
- Gradually, the person starts to feel increasing weakness in the muscles, expense of additional effort to speak, and slurred speech is noticed. This might generally be followed by difficulty in swallowing or breathing.
- Twitching (fasciculation) and cramping of muscles, particularly in the hands and feet is experienced.
- Losing the ability to use arms and legs, and difficulty in projecting the voice are the symptoms noticed in advanced stage.
- Emotional incontinence can be noticed, which is described as a condition wherein the person is not able to control his emotions like laughing and crying.
- In the advanced stage, the patient has no control at all, over his voluntary movements and has to stay bedridden.
- Some of the advanced symptoms of ALS are shortness of breath or difficulty in breathing and swallowing.
- Eventually, a permanent ventilator support is required in order to survive.
- A pulmonary induced infection results in pneumonia, setting the final phase in ALS, which might lead to death if timely precautions are not taken.
It should be kept in mind that all patients of ALS will not experience the same pattern of progression of ALS symptoms. Moreover, all the patients may not experience the same symptoms. But, progressive muscle weakness and paralysis are common. Though no treatment can halt or reverse ALS today, there is one FDA-approved drug, riluzole, that has shown some positive results regarding slowing down the progression of ALS. Several other drugs are being tested, and they will be made available in markets soon. According to the statistics, 50% of ALS-affected patients live at least for three or more years after diagnosis. About 20% patients live for five years or more; and up to 10% live more than ten years. Recent trend shows that people with ALS are living longer, for which the credit goes to clinical management interventions, riluzole, and some other compounds and drugs being developed. The example of Stephen Hawking, the pioneering astrophysicist who has survived ALS for over 40 years, is a motivating example.
Disclaimer: The information provided in this article is solely for educating the reader. It is not intended to be a substitute for the advice of a medical expert.