LPHIA - Doctors have known for years that some people with Lou Gehrig's disease also suffer from a type of dementia. And some with that dementia also develop crippling symptoms like Gehrig's, gradually losing control of their muscles.

Today, a team led by University of Pennsylvania scientists reports the discovery of a likely culprit in both. The two distinct diseases are marked by an abnormal accumulation of the same protein a startling, two-for-one discovery described in the journal Science.

"It's huge," said Mike Hutton, a neuroscientist at the Mayo Clinic College of Medicine in Jacksonville, Fla., who was not involved with the study. advertisement OAS_AD('ArticleFlex_1')


"It completely changes the way in which I think ALS research will focus," he added, using the abbreviation for amyotrophic lateral sclerosis, the disease that felled Gehrig.

With this find, science has now identified at least one faulty protein associated with each of the major neurodegenerative diseases, Alzheimer's, Parkinson's, and Huntingdon's, among others.

Cures remain years away, but pinpointing the apparent culprit is a major step toward fighting it.

The dementia in the new research is a type of frontotemporal dementia, marked by toxic clumps of proteins in the brain's frontal and temporal lobes, which control judgment and behavior.

It is less common than Alzheimer's and tends to strike younger people.

ALS is diagnosed in 5,600 people in the U.S. every year, and up to 30,000 have it at any given time, according to the nonprofit ALS Association. It is almost always fatal.

Scientists aren't certain whether the protein accumulations described in Science directly cause either disease or merely play a supporting role.

The culprit: a previously known protein called TDP-43.

TDP-43 is normally present in the nuclei of healthy brain cells, and is believed to play a role in transcribing the genetic code.
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