How is ALS treated? Are there new developments? Comprehensive care is important

The management of ALS patients is based on the collaboration of several medical disciplines and especially on the support of palliative care.

Although the disease is incurable and has been progressing steadily since its onset, some of its symptoms can be medically suppressed.

The aim of such symptomatic and palliative treatment is to maintain the best possible quality of life and self-sufficiency of patients for as long as possible.

So far, the only drug that can slow the progression of the disease is riluzole.

This drug limits the release of toxic glutamate from nerve endings.

It is administered twice daily in 50mg doses. Some studies have shown that regular use can extend patients' lives by 3-6 months.

Ongoing studies on the effectiveness of other therapeutic methods have unfortunately not yielded the desired success.

Great hope was placed on the effect of growth factors, but in the field of motoneurons their bioavailability is too low.

Gene therapy that would work with the SOD1 gene is still in clinical trials.

Stem cell transplantation therapies, especially embryonic, neuronal and bone marrow stem cell therapies, are still being tested in laboratory mouse models.

So far, none of these experimental therapies has been successfully applied to an ALS patient.

However, adequate nutrition is proving to be a very important factor in improving patient prognosis. Patients with swallowing disorders and atrophic tongues find it difficult to maintain the desired body weight, especially when muscle atrophy and catabolism are present.

A supply of good quality protein, given in liquid form, is considered most essential.

With the progression of the disease and the inability to swallow, it is necessary to perform a percutaneous endoscopic gastrostomy. Through this conduit, nutrition is fed directly into the patient's stomach. The oral cavity and oesophagus are bypassed, but the stomach and intestines themselves retain their function.

This is the most effective way of preventing malnutrition and malnutrition, which can accelerate the progression of the disease.

Symptomatic therapy targets several systems:

• facilitating breathing by administering mucolytics to improve expectoration
• treatment of constipation
• relieving fasciculation with antiepileptic drugs
• reducing painful muscle spasms by myorelaxation
• management of pain with analgesics, non-steroidal antiphlogistics or opioids
• patients with breathing difficulties are helped with home positive pressure ventilation
• psychological support for the patient and family