Despite its low incidence but its high visibility, Amyotrophic lateral sclerosis is, along with many other neurodegenerative conditions, one of the greatest challenges facing the scientific community. Since then, although it was first described in 1869, very little is known about it.
Throughout this article, we will talk about this disease, its main features, and the symptoms that set it apart from other neural diseases. We will also describe its possible causes and the most effective treatment guidelines.
What is amyotrophic lateral sclerosis or ALS?
Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease, Is one of the most famous neurodegenerative diseases in the world. The reason is that famous people who suffer from it, like scientist Stephen Hawking or baseball player Lou Gehrig, have given it great visibility.
Amyotrophic lateral sclerosis is distinguished by causing a gradual decrease in the activity of motor cells or motor neurons, Which eventually stop functioning and die. These cells are responsible for controlling the movement of voluntary muscles. Therefore, when they die, they cause these muscles to weaken and atrophy.
This disease progresses gradually and in a degenerative way, which means that the muscle strength gradually decreases until the patients suffer from total body paralysis which is accompanied by a decrease in inhibitory muscle control.
In most cases, the patient is forced to remain in the wheelchair in a state of total dependence and the prognosis is usually fatal. However, despite this absolute loss of the ability to perform virtually any movement, patients with ALS keep their sensory, cognitive and intellectual capacities intactLike any brain function unrelated to motor skills remains immune to this neurodegeneration. Likewise, control of eye movements as well as sphincter muscles is preserved until the end of a person’s days.
The incidence of amyotrophic lateral sclerosis is around two cases per 100,000 inhabitants belonging to the general population. In addition, it has been observed that ALS tends to affect certain groups of people more, including football players or veterans. However, the causes of this phenomenon have not yet been determined.
Regarding the characteristics of the population most likely to develop this disease, ALS usually appears more widely in people between the ages of 40 and 70, and with much more common in men than in women, Albeit with nuances, as we will see.
What are the symptoms?
The first symptoms of amyotrophic lateral sclerosis are usually involuntary muscle contractions, muscle weakness in a specific limb, or alterations in the ability to speak which, with the development of the disease, also affects the ability to move, eat or speak. breathe. Although these early symptoms may vary from person to person, over time, atrophy of muscle effects leads to great loss of muscle mass and therefore body weight.
In addition, the development of the disease is not the same for all muscle groups. Sometimes the muscle degeneration of some parts of the body is carried out very slowly, and can even stop and remain in some degree of incapacity.
As mentioned above, the sensory, cognitive and intellectual capacities are fully preserved; as well as the control of the sphincter and sexual functions. However, some people affected by ALS they may develop secondary psychological symptoms associated with the condition they are in and of which they are aware, these symptoms are associated with affective alterations such as emotional lability or depressive phases.
Although amyotrophic lateral sclerosis is characterized by development without causing pain in the patient, the appearance of muscle spasms and the gradual decrease in mobility they usually cause discomfort to the person. However, these discomforts can be alleviated with exercise and medication.
Although the causes of amyotrophic lateral sclerosis have not yet been established, it is known that between 5 and 10% of cases are due to an inherited genetic disease.
However, recent studies open up different possibilities for determining the possible causes of ALS:
1. Genetic alterations
According to researchers, there are a number of genetic mutations that can cause amyotrophic lateral sclerosis, which they cause the same symptoms as non-hereditary versions of the disease.
2. Chemical imbalances
ALS patients tend to have abnormally high levels of glutamate, In which it can be toxic to certain types of neurons.
3. Changes in immune responses
Another hypothesis is that which links ALS to a disorganized immune response. As a result, the person’s immune system attacks the cells of the body and causes neuronal death.
4. Poor administration of proteins
Abnormal formation of proteins found in nerve cells could generate one. breakdown and destruction of nerve cells.
Regarding the risk factors traditionally associated with the onset of amyotrophic lateral sclerosis are as follows.
People whose parents have ALS has 50% more chance of developing the disease.
Before the age of 70, men have a higher risk factor for developing ALS. From the 70s, this difference disappears.
The age group between 40 and 60 years is the most prone to the onset of symptoms of this disease.
Smoking is the most dangerous external risk factor during the development of ALS. This risk increases in women aged 45 to 50.
Exposure to environmental toxins
Some studies link environmental toxins, such as lead or other toxic substances found in buildings and homes, In the development of ALS. However, this association has not yet been fully demonstrated.
Certain groups of people
As discussed at the start of the article, there are some specific groups of people who are more at risk of developing ALS. Although the reasons have not yet been established, people who have done their military service are more at risk of contracting ALS; it is assumed that by exposure to certain metals, injuries and intense exertion.
Treatment and prognosis of ALS
No effective cure for amyotrophic lateral sclerosis has been developed to date. Therefore, although treatments cannot reverse the effects of ALS, they can delay the development of symptoms, prevent complications, and improve the patient’s quality of life. Thanks to an intervention with groups of multidisciplinary specialists, a series of physical and psychological treatments can be carried out.
By administering certain specific drugs such as riluzole or edaravone, it can slow the progression of the disease and reduce the deterioration of daily functions. Unfortunately, these drugs do not work in all cases and still have many side effects.
Regarding the other consequences of sclerosis amyotrophic lateral, symptomatic treatment has been shown to be very effective to relieve symptoms such as depression, pain, feeling tired, phlegm, constipation or sleeping problems.
The types of interventions that can be performed with patients with amyotrophic lateral sclerosis are:
- Respiratory care.
- Speech therapy.
- psychological support.
- Nutritional care.
Despite the types of treatments and interventions, the prognosis for ALS patients is quite reserved. With the development of the disease, patients lose the ability to be independent. Life expectancy is limited to 3 to 5 years after the diagnosis of the first symptoms.
However, around 1 in 4 people may survive well for more than 5 years, As is the case with Stephen Hawking. In all of these cases, the patient needs a large number of devices to keep them alive.