Stephen Hawking’s long life with ALS reminds us how little we know about the disease

Stephen Hawking, who passed away at the age of 76 last week, was a world-renowned physicist. He will forever live inside our history books, synonymous with theories that helped us understand the universe’s beginnings and how black holes behave.

But Hawking is not only noteworthy for his prolific physics work: For over 50 years he lived with amyotrophic lateral sclerosis, or ALS. The disease (which in the U.K. is called motor neuron disease, and in the U.S. commonly referred to as Lou Gehrig’s disease) typically runs its fatal course more swiftly.

The condition makes its mark on the body by affecting the neurons that control muscles. The brain and spinal column contain cells known as motor neurons—upper motor neurons in the brain and lower motor neurons in the spine. To move a muscle, the brain sends messages from the upper motor neurons through to the lower ones, and then to the muscles we want to move. In patients with ALS, motor neurons degenerate and become weaker over time.

Because we use our muscles for walking, eating, speaking, and of course breathing, people with the disease often come to rely on wheelchairs, feeding tubes, and ventilators as their condition progresses. On average, a person newly diagnosed with ALS lives just two to three years.

But Stephen Hawking lived for 55 years following his diagnosis. What made him such an outlier?

Read More at:
https://www.popsci.com/stephen-hawking-als-motor-neuron-disease#page-2

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