In Italy there are around 5,000-6,000 people suffering from ALS, a rare disease that killed the actor Eric Dane.
It was the most frequent of motor neuron diseasesamyotrophic lateral sclerosis (ALS), killed Eric Dane at the age of 53, an American actor known to the general public as Dr. Mark Sloan in the TV series Grey’s Anatomy and Cal Jacobs in Euphoria.
Also known as Charco’s diseaset – who first described it in 1869, 150 years ago – or disease of Lou Gehrig – American baseball legend who fell ill in 1939 – ALS “is a progressive neurodegenerative disease that affects the motor neurons of the anterior horns of the spinal cord, brainstem and motor cortex”, explains the Italian neurology society (Sin).
When this sneaky disease strikes
Per Eric Dane the disease arrived early, but the onset of this pathology usually occurs between the ages of 65 and 75 and is often subtle, experts point out. The motor deficit can initially manifest itself in the upper limbs, the lower limbs, at the level of the muscles with bulbar innervation or the respiratory muscles.
The American actor had made it public less than a year ago I was diagnosed with ALS, choosing to share their journey to help raise public awareness of this serious neurodegenerative pathology, as recalled by Omar Rare Diseases Observatory.
ALS numbers and risk factors
But what is it about? Amyotrophic lateral sclerosis “is the most common motor neuron disease. It is a pathology rare, progressive, which affects the nerve cells responsible for controlling voluntary movements. The course involves a progressive muscular weakening which can involve limbs, phonation, swallowing and breathing”, they recall again about Omar.
In Italy we count around 5,000-6000 sick peopleSin specialists estimate. “Even today, although much less, the male sex has a higher incidence than the female sex. Many environmental risk factors have been studied in association with the disease: cigarette smoking appears to be, together with intense physical activity (sports or work) and there trauma general, the statistically most significant risk factor”.
Furthermore, in recent years an increasingly important role has been recognised genetics as a possible causal or predisposing factor for ALS, mostly considered a sporadic pathology (85-90% of cases), although familial forms exist (10-15% of cases).
Life expectancy from the onset of symptoms is on average 3-5 years, but the course varies from patient to patient.
What stage is research on ALS?
In recent years, scientific research has made significant progress, particularly in terms of genetic bases and molecular mechanisms of the disease. On the treatment front, unfortunately there is still no therapy capable of stopping ALS.
“Some therapeutic options are available that can slow down progression in specific subgroups of patients, while clinical trials continue on innovative therapies, including gene therapy strategies and personalized approaches,” Omar recalls.
The actor’s testimony and the spotlight on the disease
Eric Dane he said he started having symptoms a year and a half before the diagnosis, with sudden weakness in his right hand. But perhaps it was the great English astrophysicist and cosmologist who made this pathology known to the general public Stephen Hawking.
“During his journey with ALS, Eric became an enthusiast supporter from the awareness and of researchdetermined to make a difference for those facing the same struggle. We will miss him deeply and will always remember him with affection” reads a note from the family.
“When a public figure chooses to talk about their experience with a rare or neurodegenerative disease, media attention can help shine a spotlight on unmet needs, inequalities of access and the need for investment in research.
The passing of Eric Dane brings ALS back to the center of public debate, reminding us how urgent it is to continue to support scientific research, strengthen care networks and promote a culture of inclusion and support for people living with this pathology”, conclude Omar.
