“It was in September 2020, in a zoom that we had set up during the pandemic, my tongue slipped. I didn’t give it much importance, but a few days later it happened to me again and that’s when I started investigating. A few months later they confirmed the diagnosis,” explained the former Minister of Education, Esteban Bullrichin dialogue with Juana Viale. In this way, the former legislator began to tell what it is like to live with amyotrophic lateral sclerosis (ALS)a disease of the nervous system that affects the neurons of the brain and spinal cord, and makes you lose muscle control.
In that sense, the doctor Mariana Benderskyphysician and specialist in Neurology, who currently works as an associate professor of Normal Anatomy and professor of the Neurology Specialist Course at the Faculty of Medicine of the University of Buenos Aires, as well as a neurologist (intraoperative neurophysiology) at the Hospital Italian from Buenos Aires, provided details about the current status of the pathology.
According to data from MedlinePlusbelonging to the United States Library of Medicine“ALS affects approximately 5 out of every 100,000 people worldwide”. In this framework, from the Centers for Disease Control and Prevention (CDCfor its acronym in English), also North American, affirm that this pathology “is more common in men than in women”, and that “the majority of people discover that they suffer from it when they are between 55 and 75 years of age, and live 2 to 5 years after symptoms develop.”
“It is a very cruel disease, really. It is degenerative. Special neurons that are used to move muscles die and then neurons are lost in the brain that is responsible for moving the muscles and in the spinal cord. Progressively, you lose control of your muscles, without losing anything else. In other words, you do not lose sensitivity, you do not lose intelligence because you are always aware of what is happening to you, but becomes trapped inside the body“, said the specialist in the program “Having lunch with Juana.”
And he continued: “These neurons begin to die, because toxic clumps of proteins are produced. Then, these neurons suddenly progressively die. We have billions of these neurons, but people begin to notice that they cannot move a foot or a hand or begin to speak poorly.”
This same point was what Bullrich warned in his story to the host: “It was in September 2020, in a zoom that we had set up during the pandemic with former cabinet colleagues from Mauricio (Macri)’s first term in the City, which was My tongue slipped at one point. They spent me on the zoom and I didn’t give it much importance. A few days later it happened to me again and that’s when I started investigating. A few months later they confirmed the diagnosis.”.
At the same time, he highlighted that arriving at the diagnosis was “by discarding.” “There are no markers that clearly tell you that you have the disease. and that is why it is a long process until they confirm that it is ALS. When we found out, we gathered our five children together and told them the diagnosis. “I told them that my body was going to age faster than normal, although it was a very hard time.”
“There is no trigger and there are many genes involved. In fact, there are 10% of those that are genetic and there are some genes that we know produce it and others that predispose you, which is not that you are going to develop it, but that you have a greater predisposition. Oxidative stress has something to do with it and there is a lot of ALS in high-performance athletes, for example, because perhaps they demand too much of these neurons, but there is nothing so proven“said the expert.
And he added: “A few years ago, the ultimate mechanism that produces this death of neurons has been much better discovered. And I’m happy because once that is achieved and they can even make disease models in animals and they can cure them, from there we can see something in humans in a few years. “That’s quite hopeful.”
Regarding the progression of the pathology, Bendersky explained that “each patient is different. The disease is very heterogeneous, there are people who die quickly in months and others live for many years.” At the same time, he highlighted that “there is a peak (of the disease) between the ages of 40 and 60, but there are younger people.”
“There are juvenile cells, especially these genetic forms and they are in younger people, who are much more aggressive. There are 80-year-old people who also have ALS. The issue is that, At some point, (the disease) begins to paralyze the breathing and swallowing muscles. And that’s the risk you have”he added.
Along these lines, Bendersky highlighted her work as director of the laboratory of UBA Living Anatomy, dependent on UBACyT, whose research focuses on the accessory areas of language using functional MRI (Roffo-UBA-CONICET); the development of the skull base using CT (UBA-Garrahan-CONICET-UNLP), the anatomical variants of cortical sulci (UBA-CONICET), and highlighted: “We study living anatomymy lab was always studied with preparations or we expected people to get sick or injured, but now we try to study them earlier, with fMRI methods. We see which part of the brain is working when they perform different tasks”.
Likewise, he highlighted that this research is carried out jointly with artificial intelligence and explained: “We use many algorithms and the processing of the images is with artificial intelligence. We work with engineers and mathematicians, we are no longer just doctors. It’s a team”.
“You have to use artificial intelligence to your advantage, you train it and teach it what to do. In medicine you tell him what and how he is going to diagnose; and it has in its favor that it is very fast and obtains a lot of data faster than a human. But there were many people training her and there always has to be a human behind explaining to her,” she added.
At the same time, he highlighted that, in research, the participants “are patients who offer themselves, sometimes they are control subjects, I am one, who is healthy people. First you need to know what is healthy to understand what is sick. So, there are huge databases of healthy subjects that are available and you can study people of different ages and see what happens. And compare it with your population.”
Finally, Bendersky highlighted the costs of dealing with the disease and explained: “You have a lot of things that are necessary and they cost a lot of money. You need to have a nurse at home 24 hours a day and then the medication, which is also very expensive. The family has to be completely reorganized. Sometimes one of the members has to stop working to dedicate themselves exclusively to patient care. And emotionally it is very hard for everyone.”
“At the hospital we try to give them all possible support, not only medical but also psychological. We have support groups for families and patients. In addition, we try to facilitate access to the necessary treatments and devices, although sometimes it is very difficult due to costs,” the expert highlighted and concluded: “In recent years, much progress has been made in ALS research. New treatments and technologies are being discovered that could improve patients’ quality of life. There is much hope that in a few years we can see significant advances that will make a big difference”.
