Today, June 21 is the International Day of Amyotrophic Lateral Sclerosis (ALS), a fatal neurodegenerative disease characterized by the progressive loss of motor neurons in the spinal cord and cerebral cortex. The Spanish Society of Neurology (SEN) estimates that ALS has an incidence in Spain of 1 to 2 new cases per 100,000 inhabitants per year, which means that each year, about 700 people they will begin to develop the first symptoms of this disease.
This high incidence makes ALS the disease more common neuromuscularas well as in tthird most common neurodegenerative disease (after Alzheimer’s and Parkinson’s) in our country, despite the fact that due to its high mortality the number of people who currently suffer from ALS in Spain is around 3,000. Most patients die in 3-5 years after start of the disease, mainly due to respiratory failure.
“Although this disease affects each person differently, since not all symptoms are experienced or appear in the same sequence, having progressive weakness in the muscles of the extremities or the muscles of speech, swallowing or breathing, are usually the most common symptoms. Although they also present abnormal muscle movements and muscle cramps (especially in hands and feet) and loss of reflexes, “he explains. Alejandro Horga, of the Spanish Society of Neurology.
«In recent years, there has been an improvement in symptomatic treatments, as well as in diagnosis times, which has led to a temporary improvement in the quality of life of many patients.
But even so, since there is no curative treatment, in a short term patients will go from a situation of normalcy to one of complete dependence. It is therefore a disease that entails a great dependency and that therefore it is not surprising that it is among the main causes of disability according to the last INE survey».
In addition, the disability generated by ALS is not only due to the severe muscle involvement that causes the progressive paralysis of the patient until his complete paralysis, but also to the fact that in more than 50% of the cases the patients show traits of dysexecutive dysfunction in neuropsychological studies, that between 5-10% of patients present an associated dementia -in general of the fronto-temporal type, which can precede, appear simultaneously or after the onset of ALS-, or at discharge rates of anxiety (30%) and depression (44%) presented by these patients, well above the rates of the general population.
«It is, therefore, a complex pathology that requires a multidisciplinary approach and high specialization. Public health has the means to care for these patients with the implementation in recent years of Multidisciplinary Units for the management of patients with ALS, present in most of the autonomous regions.
Research is also generated in these units and international clinical trials are carried out, but they are still insufficient. For this reason, for years, the SEN Neuromuscular Diseases Study Group has been recommending that there be a Specialized unit for every million inhabitants and that all referral hospitals have one, ”says Horga.
The investigation It is also, without a doubt, one of the aspects in which improvement is needed, not only in the search for more effective treatments, but in achieving advances in the knowledge of this disease. In 5-10% of cases some type of hereditary or genetic pattern is present, but in the rest the origin of this disease has not yet been identified and it has not been possible to establish clearly. Which are the risk factors.
Although some studies suggest that it may be due to a multifactorial disease in having suffered certain infections, having been exposed to tobacco or heavy materials and / or pesticides or have made Intense physical activities on a continuous basis can influence its appearance, being a man and being over 60 years old are the only risk factors that have been clearly established.
And is that the mean age of onset of ALS is between 60-69 years, and in all Western series, the incidence in men is slightly higher than in women. This also translates into more than 50% of cases affect people of working agefully productive, which makes the socio-sanitary impact of this disease is even greater.
It was already pointed out before that the main cause of death of patients with ALS is respiratory complicationsTherefore, it is a population at risk of mortality from COVID-19, as well as an especially vulnerable group given the consequences that social distancing can have on their daily care.
Although there is still no data on the impact that Covid-19 could have on Spanish patients, a report recently published in the journal Neurology among a group of patients from Catalonia points out that people with ALS have not only had problems at the time of contact with medical professionals, but also when it comes to keep your caregiver, something especially important on an emotional and organizational level.
«And in other European countries an increase in anxiety and feelings of loneliness has also been reported in ALS patients and their relatives / caregivers during the pandemic.
Something also worrisome if we take into account that, before the pandemic, psychiatric symptoms in patients with ALS they were already well above the rates of the general population, ”explains Horga.
Since in Spain the vast majority of patients with ALS are over 60 years old, it is expected that a large percentage of these people have already completed the vaccination schedule against Covid-19. In the event that it has not yet been carried out, the SEN will recommends vaccination for both patients and their caregivers.
Furthermore, the studies that have been published to date do not suggest that ALS increases the risk of side effects of vaccines and since the treatments do not involve the immune system, they should not affect the effectiveness of the vaccine.