Just muscle weakness can be life-threatening!

Amyotrophic Lateral Sclerosis (ALS) is a disease caused by the degeneration of nerve cells that control the brain and spinal cord. It is a rare disease, occurring at a rate of about 1.25-2.5 cases per 100,000 population per year. This disease is slightly more common in men than in women and typically begins to show symptoms at an average age of 60 years. The exact cause is mostly unknown. Most patients do not have a family history of muscle weakness, and surprisingly, about 50% of patients on average die within approximately 2 years after symptom onset.

What symptoms indicate ALS?

Symptoms of muscle atrophy and weakness manifest as difficulty lifting the arm, wrist or ankle drop, frequent stumbling and falling, as well as muscle twitching in some areas. These symptoms usually start in one arm or leg and then spread to nearby areas until the patient experiences symptoms throughout the body, affecting swallowing, frequent choking, stiff tongue, tongue atrophy, speech problems, and eventually spreading to the respiratory system.

Diagnosis of ALS

To diagnose this disease, doctors need to rule out other diseases with similar symptoms, such as vitamin B12 deficiency and cervical spinal cord diseases, by performing MRI scans, nerve and muscle tests using electromyography (EMG). Once a definitive diagnosis is made, patients are promptly started on treatment.

How to treat ALS

The medication used to treat this disease is called riluzole, which helps slow disease progression by inhibiting glutamate, a neurotransmitter that causes cell death. Therefore, treatment is mostly supportive to prevent complications and improve the patient’s quality of life, such as exercise, physical therapy to prevent joint stiffness, supporting adequate nutrition and rest. If the patient begins to experience fatigue and difficulty breathing, non-invasive ventilatory support may be considered to reduce respiratory effort.

However, although treatment options are still quite limited, close care by specialists can effectively reduce the risk of complications and help patients live as close to normal life as possible.