Amyotrophic Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) It is a progressive and degenerative disease of unknown cause characterized by the involvement of the neurons that control the motor pathway, more specifically, voluntary muscle movements.

There is damage at the level of the first motor neuron, responsible for transmitting the electrical impulse from the cerebral cortex to the second motor neuron, and of the latter, located in the spinal cord and responsible for transmitting the impulse to the muscles for mobility, being in both cases a voluntary impulse.

It predominantly affects males, with an age of onset between 40 and 70 years.

How does amyotrophic lateral sclerosis occur?

Although the cause is unknown, different agents have been implicated, from immunological, viral, environmental, genetic to food, with no clear correlation with the disease.

There is a sporadic form (the most frequent: 90-95% of cases) and a familial form of autosomal dominant inheritance that affects younger patients.

Symptoms

The fundamental symptoms of amyotrophic lateral sclerosis (ALS) They are derived from the presence of progressive muscle weakness, starting in the upper extremities, with involvement of the distal muscles and asymmetrically. As the disease progresses, the involvement is usually bilateral and also affects the proximal musculature.

The involvement of the muscles responsible for swallowing and language articulation produces paralysis of the same with the appearance of dysphagia, dysarthria and lingual atrophy. The change of the voice is characteristic at this time, with a nasal-type voice. There is no affectation of the sensitivity or of the ocular musculature. There is also no associated.

When the disease is in an advanced stage, the respiratory muscles are affected, causing in most cases the death of the patient.

Fasciculations, very sharp reflexes (hyperreflexia), or spasticity are also seen.

Diagnosis

It is a clinical diagnosis based on the results obtained after a complete medical history that includes a thorough neurological physical examination.

Neuroimaging tests are useful to rule out other diseases since no alterations in these tests are observed in the Amyotrophic Lateral Sclerosis.

He may show suggestive alterations of ALS that can support the diagnosis.

Treatment of amyotrophic lateral sclerosis

There is no curative treatment for ALS. The only treatment currently available is riluzole, which can slow the progression of the disease somewhat without slowing it down.

Survival is usually 2-3 years after diagnosis. Death is usually caused by respiratory (aspiration) infections or respiratory failure. However, up to 10% of cases can survive for more than 10 years.