Stephen Hawking, the world's oldest ALS survivor: Here's what we know thus far

Hawking died with amyotrophic lateral sclerosis, or ALS, a neurological illness that causes nerve and muscle function to deteriorate over time. He was diagnosed with ALS at the age of 21 and given only a few years to live, making his long, brilliant career as much of a scientific wonder as the ideas and discoveries it produced. Here's everything you should know about ALS and how Hawking managed to stay alive for so long.

What exactly is ALS?

According to the ALS Association, ALS, also called as Lou Gehrig's illness, is the progressive neurodegenerative disease which get into the motor neurons — the spinal cord and the cell in the brain that controls the muscle function — until walking, talking, speaking, swallowing, and breathing becomes difficult or impossible. Slurred speech, muscular weakness, and twitching are common ALS symptoms, according to the Mayo Clinic, and they become worse with time. The rate at which a person's health deteriorates varies greatly, although the typical survival period following diagnosis is three years, according to the ALS Association. Although ALS has no cure, it can be controlled with medication, physical therapy, occupational therapy, and speech therapy. Some people rely on ventilators to help them breathe.

Who is affected by ALS?

According to the Mayo Clinic, doctors aren't clear what causes ALS in most cases however it appears to have a hereditary component in certain people. Men appear to be more prone than women to get the illness, at least before the age of 65, according to the Mayo Clinic. It's generally diagnosed in adults between the ages of 40 and 60.

Hawking's lifespan is quite unusual.

It's quite uncommon. According to the ALS Association, just 5% of ALS patients live longer than 20 years, and living for 50 years or more is practically unheard of — however North America's longest-lived ALS patient, a Canadian called Steven Wells, has had the disease for approximately 40 years.

“This is fairly unusual,” says Lucie Bruijn, the ALS Association's head scientist, who adds that she is unaware of anybody who has lived longer with ALS than Hawking. Hawking was also fortunate to avoid the dementia that other patients with ALS endure as the disease progressed according to her.

How did Hawking manage to survive with ALS for so long?

According to Bruijn, researchers are unsure. “ALS is a complicated disease with a wide range of symptoms,” she explains. “We're collaborating with teams all around the world on these massive data projects, trying to understand each person's clinical path, their genetics, and what they've been exposed to.” They're "trying to figure out the jigsaw" from it, she says.

Bruijn believes the sample size is too small to draw solid conclusions about the variables that allow people like Hawking and Wells to live so long, but it's likely a combination of genes, environment, and clinical treatment. According to Bruijn, the kind of motor neurons afflicted by a person's illness may also important, as motor neurons that regulate eye movement frequently outlast those in the brain and spinal cord. She believes that learning how such cells resist death would help scientists better comprehend long-term survival. “How are those motor neurons distinct from those in the spinal cord and brain?” Bruijn inquires. “We don't have all of the answers yet, but I believe they are the types of things that may lead us in the right direction.”

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