All you need to know about amyotrophic lateral sclerosis

a The British scientist Stephen Hawking was one of the most famous figures to suffer from amyotrophic lateral sclerosis (ALS). He was diagnosed at age 21 and spent most of his life in a wheelchair. According to the Portuguese Amyotrophic Lateral Sclerosis Association (APELA), the hope for surviving the disease is short, about three to five years. “In certain cases of very slow progression, patients’ survival time can exceed 10 to 15 years, from the onset of the first symptoms,” he says.

In Portugal, it is estimated that there are between 900 and 1,200 people diagnosed with amyotrophic lateral sclerosisIt is a slow and progressive neurodegenerative disease, the cause of which is unknown, and is more common in men. As APELA explains in a statement, the pathology results from the gradual deterioration of neurons, that is, those that control the body’s motor activities.

In ALS, these neurons that transmit information from the brain to the muscles die prematurely. As such, the muscles that allow us to move (skeletal striated muscles) become weaker, causing progressive muscular atrophy and fatigue, accompanied by functional limitations in everyday life.

Diagnosis is usually made one year after the first symptoms appear. People often report small, involuntary muscle movements (fasciculations), fatigue and cramps. Given that we have muscles to move our legs and arms, to talk, to chew and swallow, to breathe and to cough, there may be complaints about any of them. Respiratory failure is the main cause of death in this pathology.

The survival of these patients can be prolonged using tube feeding and non-surgical ventilator support to assist with breathing.

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