The (ALS) is a degenerative disease that affects the neurons that transmit nerve impulses from the Central Nervous System to the different muscles of the body: the so-called motoneurons or motor neurons.
It is a chronic, lethal disease. In addition, it is the most frequent pathology within neuromuscular diseases. Every year in Spain, according to data from the , about 700 people begin to develop the symptoms of this disease.
Although there are people who are affected by this disease at very early ages (second or third decade of life) and some cases have even been diagnosed in childhood, very few, the maximum incidence of this disease is between 60 and 70 years.
In patients with ALS, the deterioration and progressive loss of motor neurons will cause them to suffer severe muscular involvement that will cause the patient to progressively paralyze until complete paralysis.
Symptoms of Amyotrophic Lateral Sclerosis
As the doctor explains Alejandro HorgaCoordinator of the SEN Neuromuscular Diseases Study Group, the main symptoms of this disease are:
-
Muscular weakness.
-
Atrophy.
-
Muscle twitching or stiffness.
-
Muscle tension (spasticity).
“This is the most characteristic symptom of this disease, which generally begins in the muscles of the extremities (in 60-85% of cases). Although in up to a third of patients it starts in the bulbar muscles, responsible for speech, speech and chewing”, explains the doctor.
“But, since it is a disease that progresses very quickly, soon other muscle groups will start to be affected, becoming especially dangerous when the disease starts to affect the respiratory muscles,” he concludes.
50% of patients die in less than 3 years
It is precisely the respiratory failure produced by the involvement of the respiratory muscles that is the main cause of death in the majority of patients with ALS. Once symptoms begin, half of patients die in less than 3 years, 80% in less than 5 years, and the majority (95%) in less than 10 years.
And it is this high mortality that currently causes only between 3,000 and 4,000 people to suffer from the disease in Spain, despite the fact that it is the third most common neurodegenerative disease, after Parkinson’s and Parkinson’s, in our country.
The origin of the disease is not yet known.
Until now, investigations have not been able to determine what are the causes that trigger this disease, although experts point to a multifactorial origin.
-
“In most cases it has not been possible to determine the origin of the disease, most likely there is no single explanation, but rather a combination of factors that lead to neurodegeneration in ALS,” explains Dr. Alejandro Horga.
In fact, it is known that between 5 and 10% of cases have a hereditary origin, a very small percentage.
-
“Also, there are studies that point to others such as smoking, having suffered a previous viral infection, regular exposure to heavy metals or pesticides, having carried out regular intense physical activities…”, comments the neurologist.
What does seem clear is that “being a man and having an advanced age are the only risk factors that have been clearly established.”
ALS and sleep disorders
The muscle degeneration caused by ALS in patients makes sleep essential for these patients, due to its regulatory and restorative function. Because sleeping well allows you to recover from muscular and mental fatigue “If you improve sleep, you improve motor functionality and, therefore, the quality of life,” explains the neurologist in charge of the Sleep Unit of the.
And here the problem appears because, according to this expert, “all patients with this disease end up developing sleep disorders, which are motivated by factors of a different nature such as obstructive and/or central apneas or positional discomfort.”
Lack of sleep in turn causes or aggravates the mental or emotional problems that ALS causes in patients. It should not be forgotten that, according to data from the SEN, 30% of them suffer from anxiety and 44% from depression, very high percentages compared to those in general society.
In addition to the physical and mental manifestations of ALS, there is also the “excessive daytime sleepiness or hypersomnia, in addition to cramps and fatigue”, seriously affect the quality of life of people with amyotrophic lateral sclerosis “with the handicap of not being able to prescribe benzodiazepines because they are muscle relaxants”, adds Dr. Pérez.
For all these reasons, this expert insists on the need to develop specific research programs around sleep and ALS, in order to avoid the aggravation of the symptoms of this pathology that causes a lack of rest for those who suffer from it.