What is the prognosis for ALS, Coach Juan Carlos Unzué's illness?

Three days after World Day against amyotrophic lateral sclerosis (ALS), on June 21, the former player and coach Juan Carlos Unzué has announced at Camp Nou that he suffers amyotrophic lateral sclerosis (ALS), a degenerative neurological disease that is still there is no cure and in a large percentage of cases, life expectancy after diagnosis is five years.

What does the disease consist of?

It mainly affects the nerve cells (neurons) responsible for controlling the movement of the voluntary muscles. These muscles are what allow movements like chewing, walking, and talking.

How does it evolve?

The disease is progressive, which means that symptoms get worse over time. Both the upper and lower motor neurons degenerate or die and stop sending messages to the muscles, making them unable to function, gradually weakening, starting to contract, and atrophy. Over time, the brain loses its ability to initiate and control voluntary movements. This progressive muscular weakness advances towards the total paralysis of the patient, the ability to speak, chew, swallow and breathe also being affected. On the other hand, functions such as sensitivity and intelligence are not affected (the clear case of Stephen Hawking) and neither are eye movements, since these are motor neurons that have more resistance.

There are cases in which in less than six months the affected person suffers complete paralysis and dies, and others, however, in which the progression is slower.

Is there a cure?

Currently, there is no cure for ALS, nor is there an effective treatment capable of stopping or reversing the course of the disease. 50% of people diagnosed with ALS die in less than three years. Of the rest, 20% live for five years and only between 5 and 10% exceed the decade.

The disease was described by the French neurologist Jean-Martin Charcot, but effective therapy for its treatment is still lacking today. The only currently authorized drug for ALS is riluzole (Rilutek), of which few and insufficient benefits have been demonstrated that extend the life of the patient from 2 to 3 months.

What are the symptoms?

The disorder - also known as Lou Gehrig's disease by the famous American baseball player who suffered from it - causes a progressive deterioration of motor neurons, the nerve cells that control the voluntary movement of muscles. The patient usually begins to notice weakness, muscle stiffness or coordination problems in some of his extremities, speech disorders or swallowing difficulties. Spasms, cramps, or abnormal loss of body mass may also occur. The person loses strength and the ability to speak, eat, move, and even breathe. However, the patient does not lose his sensory capacity or, in general, his cognitive abilities. Most people with ALS die from respiratory failure, usually.

Who does it affect?

ALS mainly affects adults between 50 and 70 years, although there are many cases in younger people. In these cases in which the disorder starts at younger ages, it has often been seen that the progression of the disease is slower, probably due to the particularities of the genetic alterations that cause the disease in those cases. It is more common in males. Every year 2 cases of ALS are diagnosed for every 100,000 inhabitants that in Spain would be around 900 new cases every year.

Are the causes known?

In 90% -95% of those affected, ALS occurs sporadically and with no known origin, but there is also a type of hereditary familial ALS. In this case, the most described cause resides in a mutation that affects the antioxidant enzyme Superoxide Dismutase 1 (SOD1). Sporadic ALS cases have also been found with this mutation.

Currently, it is known that there are many candidate genes to be involved in the disease and other pathogenic mechanisms such as excitotoxicity (produced by the excitatory neurotransmitter glutamate), oxidative stress (oxidative radicals, mitochondrial dysfunction), failure of axoplasmic transport are also being studied. or autoimmunity (antibodies against calcium channels) and neuroinflammation.

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