Amyotrophic Lateral Sclerosis (ALS)
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What is Amyotrophic Lateral Sclerosis?
Amyotrophic Lateral Sclerosis, also known as Lou Gehrig’s Disease, is a rapidly progressive as well as fatal neurodegenerative disorder attacking motor nerve cells of the brain as well as the spinal cord to destroy muscular movements of the human body. The literal meaning of the disease is the wastage of the voluntary muscles due to lack of nourishment.
The disease does not affect a patient’s emotional or intelligence quotient, although a person may experience severe depression or alterations in the cognitive functions affecting memory or behavior. Although, it has not been observed to affect the sensory neurons of the person maintaining his ability to touch, smell, taste, hear, etc.
How prevalent is ALS?
The Center for Disease Control has suggested that close to 15,000 people in the United States have reported to be suffering from the deadly disease. On an average, the general prevalence rate of ALS has been estimated to be approximately 2.3 per 1,000,000. This ratio increases every year in overall reported cases. The data is also proportional to the age, as the age increases the prevalence of the disease also has been observed to increase.
People between the ages of 18 – 39 have the lowest prevalence rate of 0.5 per 1,000,000 people, whereas the age group between 65-80 years have reported to have the highest prevalence rate of 17 per 1,000,000 patients. More specifically, males have a higher prevalence than females with the reported ratio of males to females is 1.56.
In the ethnic database, it has been reported that whites accounted for almost 79% of the reported cases as compared to the blacks with 6.5%.
Overall about 5-10% of the cases have been reported within the families with same equalities among men and women. This occurrence has been linked up with the mutated gene chromosome 9.
Factors responsible for ALS
Although the exact cause of its occurrence is still unknown different studies have revealed different facts about its occurrence such as genetic mutations or environmental exposure. Many other studies suggest the disease is caused by an unusual diet or injury.
Changes in gene mutations can cause ALS. This has been confirmed by the National Institute of Neurological Disorder and Stroke. The mutated gene that produces the enzyme SOD1 is found to be associated with the occurrence of the disease.
Some research has also linked ALS with toxic environmental factors such as lead exposure or infectious agents, as well as physical trauma or behavioral and occupational factors.
Symptoms Associated with the ALS
In general, the initial symptoms have been observed to be very subtle that sometimes they can be overlooked. Some of them can be fasciculation, cramps, tights and stiff muscles, muscle weakness affecting arm or leg, slurred or nasal speech or difficulty in chewing or swallowing. These initial symptoms may aggravate into major atrophy causing a physician to suspect ALS. Apart from the initial symptoms, the progressive symptoms of the upper motor neuron include spasticity and exaggerated reflexes including an overactive reflexes including the large toe extended upward as the sole of the foot is stimulated in certain ways.
The diagnosis of ALS is difficult as no actual medical test is available to confirm the disease. Currently, clinical tests are advised to rule out the possibility of the other degenerative disorders. Some of the comprehensive diagnostic tests include blood and urine studies, spinal tap, X ray analysis, myelogram, muscle or nerve biopsy.
What goes wrong in ALS
Neurons are the cells mainly located in the brain, spinal cord and the lower stem of the brain. These cells are known as the messengers connecting the brain with the other parts of the body through the spinal cord. They pass on messages from the brain to different organs of the body including voluntary muscles. These messages are passed on through signals from neurons to neurons.
Due to genetic mutations or environmental factors, if anything goes wrong with the passage of the information then the faulty information will be processed to the voluntary parts of the muscles resulting in severe progressive degeneration. Due to a lack of functional processing, the voluntary muscles of the body gradually weaken and waste away.