What Is Amyotrophic Lateral Sclerosis?
Amyotrophic lateral sclerosis (ALS) is a devastating nervous system disease that attacks the brain and spinal cord nerve cells. It is also known as Lou Gehrig’s disease in reference to the famous American football player Lou Gehrig after he got affected by amyotrophic lateral sclerosis. The term amyotrophic refers to the gradual weakening of muscles. And this weakening happens from the slow and gradual destruction of nerves (neurodegeneration). Eventually, the affected individual may be unable to eat, speak, move around, or breathe.
How Does One Get Amyotrophic Lateral Sclerosis?
Most people acquire the disease roughly at forty to sixty years of age. Although the condition is rare, affecting two in one lakh individuals, men have more chances of acquiring the disease than women.
The definite cause of amyotrophic lateral sclerosis is still unknown. Sometimes, doctors see it running in families as an inherited disorder. But most cases happen randomly among the population with no positive family background.
What Is the Cause Behind Amyotrophic Lateral Sclerosis?
Nerve cells or neurons are tiny structures that send messages and commands back and forth from the body to the brain and vice versa. For making a voluntary activity or movement with our muscles, the brain, and spinal cord send specific commands passed on to the muscles by these nerves. The voluntary muscles in the body (whose movements we can control) obey these commands and perform the activity in the mind. All these happen in a fraction of a second.
For amyotrophic lateral sclerosis-affected individuals, the long nerve fibers that connect the brain and spinal cords to the arms and legs degenerate over time. And neurologists think of it as a disorder that affects the upper motor neurons (nerves present in the brain) and the lower motor neurons (those in the brainstem and spinal cord). One needs both of these to have the muscles move voluntarily. Gradually, the muscles become smaller and weaker (muscular atrophy) when they no longer receive the signals to twitch or contract.
What Are the Symptoms of Amyotrophic Lateral Sclerosis?
Like other neurological diseases, there is no significant amount of pain or trouble with sensations like numbness or tingling with amyotrophic lateral sclerosis. Also, there are no headaches, memory issues, thinking inabilities, or vision problems. It is just a weakness at the beginning of the disease, which progresses and worsens over time. Some common symptoms would be:
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Fasciculations or muscle twitching one can see under the skin, coupled with weakness and atrophied muscles.
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Swallowing problems, tend to choke on food often.
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Bladder and bowel problems, trouble emptying the bowel and bladder, and inability to hold the urine and feces at one’s will.
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Troubles with speech.
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Breathing problems.
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Paralysis.
How Does the Disease Progress in Amyotrophic Lateral Sclerosis?
From the onset of the first symptom, the disease progresses as follows:
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Initially, amyotrophic lateral sclerosis is presented as mild muscle weakness. One may have trouble walking, running, writing, or speaking. Patients begin with weakness in one part of the body, probably on one foot, causing a foot drop or foot slap (foot hitting the ground on walking).
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And starts to have weakness in the hand with trouble gripping things or doing things with the hands. Occasionally one may start feeling weakness in the face as well.
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And they have trouble talking and sometimes chewing. The speech may be a bit slurred and hard to understand. Eventually, one loses strength and turns immobile.
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With the progression of the disease, the individual loses the ability to perform even the basic day-to-day activities, and they lose control over their bodily functions. With speech function impairment, one cannot communicate effectively. But the individual has full mental awareness, which makes it emotionally hard to go through all this.
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There is obvious paralysis in the long run. It progresses to a stage where the muscles in the chest get affected, and one cannot breathe. The affected individual will need to get the help of a breathing machine. In most cases, one may die from respiratory failure.
How Is Amyotrophic Lateral Sclerosis Diagnosed?
On average, it takes about a year to get the diagnosis made because initially, the symptoms can be hard to pinpoint to a diagnosis for sure. Sometimes, the tests that are done early on do not always show a definitive diagnosis. But with amyotrophic lateral sclerosis progressing over time, people become weaker in some parts of the body. Initially, it is the arm, leg, or face, and with time it starts to progress to the other parts of the body. And the real disability happens when the disease progresses throughout the body. Initial diagnosis should be made by a team of specialists. An initial evaluation includes a number of different tests, such as:
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Neurological examination to evaluate the level of muscle function, coordination, and strength.
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An electromyogram (EMG) may be conducted. It is a study where electric shocks are given to the nerve to measure how the nerves are working. And then use little needles under the skin into the muscles to assess how the muscles are working.
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One may need blood tests and magnetic resonance imaging (MRI) studies to evaluate the level of nerve degeneration.
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Those with speech trouble should meet a speech pathologist for evaluation.
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Those with swallowing problems should undergo an assessment regarding that.
What Is the Treatment Strategy for Amyotrophic Lateral Sclerosis?
Unfortunately, Amyotrophic lateral sclerosis has no cure so far. No treatment can reverse the condition, but medicines can definitely help relieve the symptoms. And the right treatment can improve the life expectancy of an affected individual. With the help of a rehabilitation specialist, one can get directions for physical therapy, occupational therapy, and speech therapy. Swallowing experts can help with chewing and swallowing food. For those with breathing problems, mechanical breathing machines can be installed.
Conclusion:
Amyotrophic lateral sclerosis is a rather rare but highly debilitating condition. Strangely, the disease does not affect the mental faculties of the affected individual. Hence the intellectual capacity remains intact. One of the all-time brilliant physicists, Dr. Stephen Hawking, was suffering from this condition in his twenties but made commendable contributions to society in his lifetime. With the aid of professionals from multiple medical specialties, amyotrophic lateral sclerosis-affected individuals can do the needful to improve their living standards and prolong their survival to a possible extent.
