ALS, WHAT IS AMYOTROPHIC LATERAL SCLEROSIS?
ALS, what is it the disease of mother of Carlotta Ferlito? The 29-year-old former gymnast and influencer is currently experiencing the drama of her mother Roberta, hospitalized in a hospice due to the progression of this degenerative condition and, waiting to tell her experience this afternoon in front of the “Verissimo” cameraswe can discover something more about a pathology for which there is no cure and which is highly disabling since it leads to the atrophy of voluntary muscles up to a progressive paralysis. What is the definition of ALS, also known as “Lou Gehrig’s disease”what are the symptoms and the instrumental tests to recognize it and, finally, how can the progression of the pathology be slowed down with the latest medical discoveries?
First of all we can say that the ALS, or amyotrophic lateral sclerosisit’s a disabling neurodegenerative disease given that it compromises motor neurons, i.e. those brain nerve cells responsible for stimulating muscle contraction which makes not only movement possible but also some vital functions such as breathing, phonation and also swallowing. Once the motor neurons degenerate, the voluntary muscles no longer receive impulses from the brain and those affected by this disease suffer from paralysis. Wanting to historicize studies and discoveries around this pathology, we can remember how it is also known as Lou Gehrig’s disease, an American baseball player who represented one of the best-known cases of affected patients together with Stephen Hawking, or precisely motor neuron disease: in fact this affects the central and peripheral ones found in the spinal cord and brainstem.
CAUSES AND SYMPTOMS OF ALS: THERE IS NO CURE AND DEATH OCCURS BY…
ALS (the first description of which dates back to 1824 with Charles Belle and then con Jean-Martin Charcot in 1874 using the expression “amyotrophic lateral sclerosis”) is essentially characterized by muscular rigidity and reduction of muscle size themselves as well as by the progressive difficulty not only in breathing but also in swallowing and pronouncing words, as it is neurodegenerative and disabling: according to some studies, amyotrophic lateral sclerosis usually affects people aged between 40 and 70 (but the average debut It’s around 60) and, remaining in Italy alone, there would be around 6 thousand affected patients with an incidence rate of 2-3 new diagnoses per 100 thousand inhabitants every year. As regards the triggering causes (in 90% the cause is not known), science still questions itself on the matter but it is believed that ALS is due to some very specific risk factors such as genetic predisposition, lifestyle and other environmental factors. As regards the diagnosis, there are no specific instrumental tests but we can get there with tests periodic functional and clinical testsand excluding other pathologies.
As mentioned, to date there is no cure for ALS (amyotrophic lateral sclerosis) and even yours speed of progression is variable and it depends from person to person: riluzole (which is part of the benzothiazole class) is used which helps to extend life expectancy, in addition to artificial ventilation which significantly improves the quality of life of patients who have now difficulty breathing. According to what is published on the Ministry of Health website, in a quarter of cases the patient dies within two years of the onset of the first symptoms, while it can also happen that some subjects survive eight or more years; furthermore, as explained on the “Mario Negri” Institute website, often death occurs due to paralysis of the voluntary respiratory muscles. An aspect made even more dramatic by the fact that ALS does not inhibit the patient’s thinking and relational capacity, whose mind thus finds itself living ‘prisoner’ of a body over which you no longer have control.
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