June 21 is World ALS Day, a neurodegenerative disease of unknown origin, which generates a great dependency of the patient who suffers from it. The reason for this date is to give visibility to ALS, as well as to continue promoting research to find both the cause and the cure for the disease.
For this occasion, we have the collaboration of the prestigious Dr. Povedano Panades, specialist in Neurophysiology of the Neuromuscular Diseases Unit of iMove Traumatology at Clínica Mi Tres Torres.
What is ALS?
Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease that affects nerve cells in the brain and spinal cord. The degeneration of these neurons decreases the brain’s ability to initiate and control muscle movement causing progressive paralysis in the affected person.
Some 4,000 people a year suffer from ALS in Spain, and about 900 new cases are diagnosed each year, according to figures from the Spanish Society of Neurology (SEN). The disease most often appears between the ages of 55 and 65, although it can also affect younger or older people. Men have a slightly higher incidence than women.
What are the symptoms of ALS?
ALS “usually appears between the ages of 55 and 65, but it can also affect younger or older people,” explains Dr. Mónica Povedano. Men have a slightly higher incidence than women.
The initial symptoms of ALS can be very varied, depending on the patient, and can even be confused with those of other diseases. For this reason, in many cases there is a delay of several months in diagnosing the disease. In this regard, the SEN points out that, in Spain, the diagnostic delay of this disease is about 12 months and diagnostic errors are an important source of this delay.
Often, the first symptoms of ALS begin in the hands, feet or limbs, and then spread to other parts of the body. As the disease progresses, more muscle groups develop problems, causing, for example, swallowing, speech and breathing problems, muscle twitching (fasciculations), as well as inappropriate crying, laughing or yawning.
What is the life expectancy of an ALS patient?
According to the Spanish Society of Neurology, 50% of ALS patients die before 3 years of diagnosis. Hence, early diagnosis is essential to try to increase the patient’s life expectancy.
“We are dealing with a disease that expresses itself in the form of weakness and motor disorders, but does not cause pain and, therefore, does not frighten the patient. As there is no pain, they do not attach importance to the appearance of these first signs of loss of mobility, and do not consult a doctor,” says Dr. Mónica Povedano.
For this reason, she advises that “when these small symptoms appear, it is necessary to go to the doctor. Most of the time it will be a functional impotence that will not be of any importance, but a professional will rule it out. However, if it is a disease such as ALS, it will be detected early”.
How is ALS treated today?
The treatment of people affected by ALS is focused on alleviating the symptoms of the disease. They cannot reverse the damage, but they can delay the progression of symptoms, and improve their quality of life and that of the people around them. It is very important that the patient is placed in the hands of “multidisciplinary management units, because that is what will ensure that the symptoms are treated correctly and that survival increases,” remarks the iMove specialist.
Physiotherapy and rehabilitation play a very important role in the treatment of ALS to address muscle weakness. “It will not be possible to recover the strength that the patient has lost. The work will focus on maintaining muscle tone and mitigating pain due to the lack of mobilization,” explains Dr. Povedano of iMove’s Neuromuscular Diseases Unit. Likewise, “there are studies that show that the patient’s survival increases with good physiotherapy work, not only of the extremities, but also of respiratory physiotherapy to maintain mobility and elasticity of the thoracic cage. These exercises will be applied and adapted in a personalized way to each patient depending on the phase or stage of the disease.
What are the latest advances in research?
In recent years there have been important advances in the knowledge of Amyotrophic Lateral Sclerosis, and there has been an increase in the number of clinical trials aimed at finding new drugs and effective treatments. One of the most recent, “focuses on those types of the disease that are genetically determined, i.e. we know the linked gene. For this type, clinical trials are already being carried out with forms of action directed directly against this gene, which opens up a new horizon of possibilities for sick people,” concludes Dr. Povedano.