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Amyotrophic Lateral Sclerosis (ALS): symptoms and treatment

Amyotrophic Lateral Sclerosis (ALS): symptoms and treatment

January 18, 2023

Despite its low incidence but great visibility, Amyotrophic Lateral Sclerosis is, along with many other neurodegenerative conditions, one of the biggest challenges to which the scientific community faces. Since, although it was first described in 1869, there is still very little knowledge about it.

Throughout this article we will discuss this disease, its main characteristics and the symptoms that distinguish it from other neuronal diseases. Also, we will describe its possible causes and the most effective treatment guidelines.

  • Related article: "Neurodegenerative diseases: types, symptoms and treatments"

What is Amyotrophic Lateral Sclerosis or ALS?

Amyotrophic Lateral Sclerosis (ALS), also known as motor neuron disease , is one of the most known neurodegenerative diseases worldwide. The reason is that celebrities who suffer from it, such as scientist Stephen Hawking or baseball player Lou Gehrig, which have given him great visibility.


Amyotrophic Lateral Sclerosis is distinguished by causing a gradual decrease in the activity of motor or motor neurons , which eventually stop working and die. These cells are responsible for controlling the movement of the voluntary musculature. Therefore, when dying they cause weakening and atrophy of these muscles.

This disease progresses in a gradual and degenerative manner, which means that muscle strength gradually decreases until patients suffer total paralysis of the body to which a reduction in inhibitory muscular control is added.

In most cases the patient is forced to remain in a wheelchair in a state of total dependence and whose prognosis is usually fatal. However, despite this absolute loss of ability to perform virtually any movement, patients with ALS preserve their sensory, cognitive and intellectual abilities intact , since any brain function not related to motor skills remains immune to this neurodegeneration. In the same way, the control of the eye movements, as well as the sphincter muscles are preserved until the end of the person's days.


  • Maybe you're interested: "Motor neurons: definition, types and pathologies"

Prevalence

The incidence of Amyotrophic Lateral Sclerosis is about two annual cases of every 100,000 people belonging to the general population. In addition, it has been observed that the ELA tends to affect to a greater extent certain groups of people, among which are soccer players or war veterans. However, the causes of this phenomenon have not yet been determined.

In terms of the characteristics of the population most likely to develop this disease, ALS usually appears to a greater extent in people between 40 and 70, and with much more often in males than in females , although with nuances, as we will see.

What symptomatology does it present?

The first symptoms of amyotrophic lateral sclerosis are usually involuntary muscular contractions, muscle weakness in a particular limb or alterations in the ability to speak which, with the development of the disease, also affect the ability to perform movements, eat or breathe . Although these early symptoms may vary from one person to another, with the passage of time, the atrophy of affects the muscles leads to a large loss of muscle mass and, therefore, of body weight.


In addition, the development of the disease is not the same for all muscle groups. Sometimes, the muscular degeneration of some parts of the body is carried out very slowly, and can even stop and maintain a certain degree of disability.

As mentioned above, the sensory, cognitive and intellectual capacities are totally preserved; as well as toilet training and sexual functions. However, some people affected by the ELA can develop secondary psychological symptoms associated with the state in which they are and of which they are fully aware, these symptoms are associated with alterations of affectivity such as emotional lability or depressive phases.

Although Amyotrophic Lateral Sclerosis is characterized by developing without causing any type of pain in the patient, the appearance of muscle spasms and the progressive decrease of mobility They usually generate discomfort in the person. However, these annoyances can be alleviated with physical exercises and medication.

Causes

Although, for the moment, the causes of Amyotrophic Lateral Sclerosis have not been established with certainty, it is known that between 5 and 10% of the cases are caused by a hereditary genetic alteration .

However, recent studies open different possibilities when determining the possible causes of ELA:

1. Genetic alterations

According to the researchers, there are a number of genetic mutations that can cause Amyotrophic Lateral Sclerosis, which cause the same symptoms as non-hereditary versions of the disease .

2. Chemical imbalances

It has been proven that ALS patients tend to present abnormally high levels of glutamate , which can be toxic to certain types of neurons.

  • Maybe you're interested: "Glutamate (neurotransmitter): definition and functions"

3. Alterations of immune responses

Another one of the hypotheses is the one that relates the ELA with a disorganized immune response . As a result, the person's immune system attacks the cells of the body itself and causes neuronal death.

4. Poor protein administration

The abnormal formation of the proteins that are inside the nerve cells could generate a degradation and destruction of nerve cells .

Risk factor's

Regarding the risk factors traditionally associated with the appearance of Amyotrophic Lateral Sclerosis are the following.

Genetic heritage

People with some of their parents with ELA It has a 50% more chance of developing the disease.

Sex

Before age 70, male sex is a higher risk factor for developing ALS. After 70, this difference disappears.

Age

The age range between 40 and 60 years is the most prone to the appearance of the symptoms of this disease.

Habits with tobacco

Smoking is the most dangerous external risk factor at the time of developing ELA. This risk increases in women from 45-50 years.

Exposure to environmental toxins

Some studies link the environmental toxins, such as lead or other toxic substances present in buildings and homes , to the development of ELA. However, this association has not yet been fully demonstrated.

Certain groups of people

As discussed at the beginning of the article, there are certain specific groups of people who are more likely to develop ALS. Although the reasons have not yet been established, people who present military service have a higher risk of ALS; it is speculated that by exposure to certain metals, injuries and intense effort .

Treatment and prognosis of ELA

At the moment, an effective cure for amyotrophic lateral sclerosis has not been developed. Therefore, although treatments can not reverse the effects of ALS, they can delay the development of symptoms, prevent complications and improve the quality of life of the patient . By means of the intervention with groups of multidisciplinary specialists, a series of physical and psychological treatments can be carried out.

By administering some specific medications such as riluzole or edaravone, It can delay the progression of the disease, as well as reduce the deterioration in daily functions . Unfortunately, these medications do not work in all cases and still have numerous side effects.

Regarding the rest of the consequences of Amyotrophic Lateral Sclerosis, symptomatic treatment has proven to be highly effective when it comes to relieving symptoms such as depression, pain, tiredness, phlegm, constipation or sleep problems.

The types of interventions that can be carried out with patients suffering from amyotrophic lateral sclerosis are:

  • Physiotherapy .
  • Respiratory care
  • Occupational therapy
  • Talk therapy.
  • Psychological Support .
  • Nutritional attention

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 autonomous. Life expectancy is restricted to 3 to 5 years after the diagnosis of the first symptoms.

However, around 1 out of 4 people can survive much more than 5 years , as is the case with Stephen Hawkins. In all these cases the patient requires a large number of devices that keep them alive.


Diagnosis and Treatment of ALS-Mayo Clinic (January 2023).


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