ALS is a rapidly progressive, fatal neuromuscular disease which typically attacks people in their 50s and 60s. Hunter was diagnosed with it at age 52.
The disease starts with an unexplained weakness and eventually ends with total paralysis.
There is usually no sensation or pain. "They can also have spasticity or stiffness of the muscles," says Dr. Richard Tim, director ofDuke'sMDA Clinic.
The disease can progress rapidly or slowly. Hunter died about a year after he was diagnosed.
The primary treatment is a drug called Ryllotec. On average, it only prolongs life for three to nine months.
"From the time of diagnosis, on average, half of them live more than three years, half of them live less than three years," says Tim.
Researchers do not really know how one gets ALS, but they are starting to get some ideas.
"There may be a hereditary component to all of ALS or some of it, and it requires an environmental trigger. It's not known," says Tim.
While the body deteriorates, the mind remains sharp. Tim says that is both good and bad.
"I think it's good in that they're not becoming senile or demented. But it's bad because of their awareness. They're aware of all the failings of their body," he says.
Tim says when celebrities get a disease it usually helps generate funds for research and awareness.
Physicist Stephen Hawking has ALS. Soap opera star Michael Zaslow died of the disease earlier this year.
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