Definition
Amyotrophic lateral sclerosis is a serious neurodegenerative disease, the target of which is made up of motor neurons located in the central nervous system (brain and spinal cord).
Probably, this condition is better known by the acronym "ALS", but it is also known as Gehrig's disease or motor neuron disease.
Generally, ALS occurs in patients over 50 years of age.
Causes
As mentioned, amyotrophic lateral sclerosis is caused by a progressive degeneration of motor neurons. The exact cause of this phenomenon, however, has not yet been fully identified. However, there are many hypotheses about it.
Among the possible causes that are hypothesized to favor the development of neuronal deterioration, we recall: the presence of a family history of ALS, an imbalance in the levels of glutamate in the cerebrospinal fluid (which can cause toxicity towards motor neurons), the formation of protein aggregates within motor neurons, the accumulation of toxic waste substances within motor nerve cells, mitochondrial dysfunction, lack of neurotrophic growth factors within motor neurons and a malfunction of glial cells.
Additionally, individuals with a smoking habit and individuals exposed to chemicals or lead and other metals appear to be at greater risk of developing ALS.
Symptoms
The symptoms of ALS usually begin to manifest from the upper and lower limbs, hands and feet and then spread to all the muscles of the body, including the respiratory ones.
Patients with ALS, therefore, may experience symptoms such as difficulty walking, weakness in the legs and arms, tremors, speech and swallowing difficulties, muscle cramps, inability to maintain correct posture, joint stiffness, hoarseness, dyspnea and breathlessness. short.
These symptoms appear gradually and tend to get worse over time.
In the final stage of the disease, patients are paralyzed, therefore, they are forced to use the wheelchair, are no longer able to speak and feed.
The disease also affects the respiratory muscles, therefore, patients require mechanical ventilation to be able to breathe.
The information on ALS - Drugs to Treat Amyotrophic Lateral Sclerosis is not intended to replace the direct relationship between health professional and patient. Always consult your doctor and / or specialist before taking ALS - Drugs to Treat Amyotrophic Lateral Sclerosis.
Medicines
Unfortunately, there are currently no medications to treat ALS. The implications of this disease are always tragic. Generally, death occurs due to severe respiratory failure, approximately 3 to 5 years after the onset of the disease.
However, there is a drug that is used in the treatment of ALS: riluzole. This medicine appears to be able to slow the course of the disease, but not stop it. Furthermore, the response to therapy varies from patient to patient and the results are not always as expected.
Alongside an "eventual riluzole-based therapy, therefore, a symptomatic treatment is carried out, aimed at reducing - as far as possible - the symptoms induced by the disease.
ALS patients can be helped through physiotherapy, speech therapy, and occupational therapy. In the final stages of the disease, however, mechanical ventilation and artificial feeding are used.
Finally, it is not uncommon for ALS patients to experience depression; therefore, in these cases also psychotherapy can be of help.
The following is the only drug approved in the therapy against ALS and some examples of pharmacological specialties; it is up to the doctor to choose the most suitable active ingredient and dosage for the patient, based on the severity of the disease and the state of health of the patient. and his response to treatment.
Riluzole
As mentioned, riluzole (Rilutek ®, Riluzole Zentiva ®) is the only drug currently approved for the treatment of ALS. This active ingredient works by reducing the release of glutamate in the brain and spinal cord. chemical messenger may be useful in preventing the degeneration of motor neurons that characterizes amyotrophic lateral sclerosis.
Riluzole is available for oral administration in the form of tablets or an oral suspension.
The dose of drug usually used is 50 mg, to be taken twice a day, with an interval of 12 hours between one administration and the other. It is very important that riluzole is always taken at the same times of the day (for example, at morning and evening).
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