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Hawking’s fame has led to guest appearances on some of his favorite television shows including “The Simpsons” and “Star Trek.” His animated likeness from “The Simpsons” has even been turned into an action figure _ one of which sits proudly on his office desk. There’s also a Homer Simpson clock that Hawking is known to glare at when visitors are late for an appointment.

“He’s a big ham, he loves the spotlight,” said Kitty Ferguson, who’s written two biographies of the physicist.

She said he has a wry sense of humor and has programmed his computer to respond to random encounters with people who ask if he’s Stephen Hawking. “No, but I’m often mistaken for that man,” his voice synthesizer deadpans.

Lou Gehrig’s disease, also called amyotrophic lateral sclerosis, or ALS, attacks motor neurons, cells that control the muscles. Patients typically suffer muscle weakness and wasting, become paralyzed and have problems talking, swallowing and breathing. Only about 10 percent of patients live longer than a decade.

People who are stricken at a young age, as Hawking was, generally have a better chance of surviving longer. Most people are diagnosed between 50 and 70. Life expectancy generally ranges from two to five years after symptoms like slurred speech, difficulty swallowing and muscle weakness set in. Hawking’s personal physicians don’t discuss his condition with the press, Croasdell said.

For some reason, the disease has progressed more slowly in Hawking than in most. Al-Chalabi and colleagues are analyzing a DNA sample from Hawking, along with those of other patients, to see if there is something rare about his disease or any genetic mutations that could explain his long survival and if that information could be used to help others.

Some experts said the type of care Hawking has, including about a dozen health workers 24 hours a day, may have extended his life expectancy.

“The disease can sometimes stabilize and then the kind of care delivered may be a factor in survival,” said Virginia Lee, a brain disease expert at the University of Pennsylvania School of Medicine. “Remaining mentally alert is also extremely important and he has clearly done that.”

Hawking says he tries not to think about his limitations.

“I have had (Lou Gehrig’s disease) for practically all my adult life,” he says on his website. “Yet it has not prevented me from having a very attractive family and being successful in my work,” he writes. “I try to lead as normal a life as possible and not think about my condition or regret the things it prevents me from doing, which are not that many.”

From the office pictures documenting his achievements, that certainly seems to be the case. Framed photos show the physicist with several popes and on memorable trips to China and Easter Island.

He has even flown in a space simulator. In 2007, Hawking took a zero-gravity flight in Florida, the first time in 40 years he abandoned his wheelchair.

“That was the happiest I’ve ever seen Stephen,” said Sam Blackburn, Hawking’s graduate assistant, who accompanied him on the ride along with about a half-dozen others, including two doctors. “He just had the biggest grin on his face.”

Hawking has also been married twice and has three children and three grandchildren. With his daughter Lucy, he has written several children’s books on physics.

Al-Chalabi said most patients with Lou Gehrig’s disease succumb after their breathing muscles stop working. He had no predictions for what the biggest health risks to Hawking’s future might be.

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