Amyotrophic lateral sclerosis: what is it and what are the latest advances to combat it

Every June 21, since 1997, the World Amyotrophic Lateral Sclerosis (ALS) Day, a neurodegenerative disease characterized by progressive atrophy of all the muscles in the body except the heart and the muscles that control the motility of the eyes and sphincters (bladder and anal), without producing changes in sensitivity.

On this day, a large number of activities are organized around the world with the aim of keeping alive the search for the cause, the treatment and the cure of this disease.

It’s a degenerative neurological disease that affects motor neurons in the brain and spinal cord and prevents muscles from functioning. The underlying causes are unknown and at the moment there is no cure. The average survival of patients is 3 to 5 years. In exceptional cases, the disease can stop, like that of astrophysicist Stephen Hawking, who survived with ALS for several decades..

“When the motor neurons are damaged, they stop sending messages to the muscles, so the muscles cannot function. Anyone Can Suffer From It but mainly the symptoms generally start around 50-70 years ”, explained the doctor Eugenie Conti, staff physician of the neurology division, section of neuromuscular diseases of the Hospital de Clínicas.

Epidemiological studies have estimated that the incidence or number of people who will develop ALS each year is approximately 2 in 100,000 in the general population. The prevalence or number of people living with ALS at one time is approximately 7 out of 100,000 inhabitants. In Argentina, both incidence and prevalence are estimated to be consistent with epidemiological data published in the rest of the world.

“Until now little is known about the origin of the pathology, different mechanisms involved in the death of the neuron have been described but it has not been possible to determine if one of them is the origin. or the consequence of another mechanism still unknown “, adds the professional of the Clinics.

Some patients confuse multiple sclerosis (MS) with ALS, but these two entities are completely different, from their origin to their form of course and treatment.. MS is an inflammatory disease that occurs with relapses and remissions and the treatments are aimed at reducing inflammation, on the other hand, ALS is a progressive degenerative disease in which we are looking to find a method that manages to stop the cascade. which causes this degeneration.

“Symptoms, which can be very varied, are a consequence of the weakness and may affect the ability to walk, dress, write and other activities.. Es poco probable que sucedan todos al mismo tiempo o que lleven un orden específico Es una enfermedad progresiva, en la mayoría de los casos, los síntomas avanzan y se van agregando nuevos a medida que pasa el tiempo, existen igual casos descritedos en donde la enfermedos has stopped. It is not yet clear what could be the reason for this varied course in different patients, ”said Dr Conti.

Treatments

According to Conti, “the current therapeutic strategy is to support the patient in an interdisciplinary manner, to form teams comprising professionals in neurology, pneumology, psychology, physiatry, kinesiology, occupational therapy, speech therapy, etc. depending on the symptoms that are experienced or appearing in the patient. There are drugs available that work by slowing the progression of the disease, but so far no drug has stopped it. Many studies try to decipher the origin of the disease. It is estimated that when we find it, we will be faced with the initial kick to achieve a cure “

“Survival in ALS patients is between 3 and 5 years from the onset of average symptoms, of course it can be less or sometimes much longer. Regarding the fact that there are patients who are cured, it is controversial to say so. There are cases that have been arrested (like that of Stephen Hawking, who survived decades with the disease) but there are very few. There are no statistics on it, we do not know why either and we even suspect that it could be other diseases which are not yet known ”, declared the professional of the Hospital. by Clínicas.

Until in Argentina there are two drugs approved for patients with ALS, the Rilzole and the Edaravone, the mechanism of action of these treatments indicates various phenomena which occur in the neuron and which are part of the events involved in the death of the neuron.

“As we have said before, multiple events occur and it is not known in which order or which is the first, so the treatments that we have today have not yet resulted in a cure.. There are currently many ongoing studies that seek to demonstrate efficacy in patients with ALS. We expect promising results, ”concluded the specialist.

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