![]() So the common way people die is of respiratory failure. Life expectancy turns on two things: the motor neurons running the diaphragm-the breathing muscles. But that means that half the people live longer, and there are people who live for a long, long time. On average people live two to three years after diagnosis. One thing that is highlighted by this man's course is that this is an incredibly variable disorder in many ways. What has Stephen Hawking's case shown about the disease? One of the misconceptions about ALS is that it's only a motor-neuron disease, and that's not true. So some of these patients can actually develop dementia, called frontal-temporal lobe dementia. It's now well recognized that 10 percent of these patients can develop degeneration in another part of the brain, such as other parts of the frontal lobe that don't contain the motor neurons or the temporal lobe. And it was thought for quite some time that these disorders were limited to motor neurons. Those are the four classic motor-neuron disorders that have been described. ![]() But it pretty much always spreads to limb muscles. The other classic syndrome is called progressive baldor palsy-or progressive supranuclear palsy-which is weakening of cranial muscles, like the tongue, face and swallowing muscles. However, pathologically they also have lower motor-neuron disorder. There is also primary lateral sclerosis-PLS-and clinically it looks like an isolated upper motor-neuron disorder. However, pathologically, if you do an autopsy of a patient, they will have evidence of deterioration of upper motor neurons. It appears to be an isolated illness of the lower motor neurons. One is referred to as progressive muscular atrophy, or PMA. It's been known for quite some time that there are variants of ALS. The disease causes weakness of either upper motor neurons or lower motor neurons or both. The guys in the brain are called the upper motor neurons, and the guys in the spine are called the lower motor neurons. These are controlled electrically and are synaptically connected to motor neurons that reside lower down in the brain-as well as motor neurons that reside in the spinal cord. Each muscle is controlled by motor neurons that reside in the brain in the frontal lobe. What is ALS-and is there more than one form of it?ĪLS, which is also known as a motor-neuron disease-and colloquially as Lou Gehrig's disease in the U.S.-is a neurodegenerative disease. Why has Hawking lived so long with this malady when so many other people die so soon after diagnosis? We spoke with Leo McCluskey, an associate professor of neurology and medical director of the ALS Center at the University of Pennsylvania, to find out more about the disease and why it has spared Hawking and his amazing brain. Hawking's condition was first diagnosed when he was 21, and he was not expected to see his 25th birthday. Most patients with ALS-also known as Lou Gehrig's disease, for the famous baseball player who succumbed to the disease-are diagnosed after the age of 50 and die within five years of their diagnosis. And he is currently the director of research at the school's Center for Theoretical Cosmology.īut like his mind, Hawking's illness seems to be singular. Hawking spent 30 years as a full professor of mathematics at the University of Cambridge. And since 1985 he has had to speak through his trademark computer system-which he operates with his cheek-and have around-the-clock care.īut his disease seems hardly to have slowed him down. ![]() For much of his time in the public eye, though, he has been confined to a wheelchair by a form of the motor-neuron disease amyotrophic lateral sclerosis (ALS). The famous theoretical physicist has helped to bring his ideas about black holes and quantum gravity to a broad public audience. Stephen Hawking turns 70 on Sunday, beating the odds of a daunting diagnosis by nearly half a century. This story, originally published on his 70th birthday on January 7, 2012, is being resurfaced to explain how he beat the odds and lived so long with the disease amyotrophic lateral sclerosis (ALS). Editor’s Note: Renowned theoretical physicist Stephen Hawking died on March 14, 2018, at age 76.
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