A disease that's related to the infamous Mad Cow Disease has killed 4 Utahns this year. While doctors say the number of deaths is not unusual, based on the incidence worldwide, that's little consolation to the Utah families who had to deal with it. The story from science specialist Ed Yeates.

Ellie Steiger of Taylorsville became one of those rare statistics few people hear about. She died in March from Cruetzfeldt-Jacob's Disease, a cruel illness which literally eats away the brain.

((MELVIN STEIGER/ELLIE'S HUSBAND: "IT WAS VERY DIFFICULT TO GIVE UP, THAT THERE WAS NO CURE, NO HELP."))

It's still hard for him to talk about his wife's death, but Melvin Steiger now spends a lot of time with his children and grandchildren - remembering the good times with Ellie.

Unlike its variant cousin - the so-called Mad Cow disease - CJD occurs sporadically worldwide in only one to one-point-five cases per million per year.

But the symptoms are the same. Within six months, Ellie Steiger couldn't walk or talk or remember.

((MELVIN STEIGER: "SHE DIDN'T KNOW HOW MANY KIDS SHE HAD. SHE DIDN'T KNOW MY NAME."))

((ED YEATES: "CJD IS CAUSED BY A PROTEIN CALLED A PREON. WE ALL HAVE NORMAL PREONS IN OUR BRAIN. BUT WHEN ONE WITH AN ABNORMAL SHAPE GETS IN THERE, IT POISONS EVERYTHING - CHANGING THE OTHERS SO THEY BECOME ABNORMAL TOO."))

The disease is NOT highly contagious, but injecting just a few molecules of a mutated preon into a normal brain starts the deterioration. That's why pathologists take special precautions handling brain and tissue fluid from a CJD victim.

((MELVIN STEIGER: "WE GOT TALKING TO MORTICIANS AND FOUND OUT THE DAY SHE DIED THAT THEY WOULD NOT PROCESS THE BODY."))

In other isolated examples: A patient picked up CJD from a corneal transplant taken from a contaminated cadaver. Some children treated with a growth hormone from infected pituitaries got CJD. And some seizure patients in Switzerland got it from old electrodes once implanted in the brains of CJD victims.

Doctors call Ellie's case a sporadic incident - NO known cause. At death, her body was taken in a closed casket directly to this cemetary for burial.

((MELVIN STEIGER: "YOU ALWAYS PREFER TO MAKE IT AS NICE AS YOU COULD. AND THAT DIDN'T WORK. ON THE OTHER HAND WE TOOK NO UNNECESSARY RISKS TO EXPOSE ANYBODY ELSE."))

Though Utah's yearly cases of CJD compare with the national average, Melvin Steiger and other families have launched a campaign - hoping to make it a mandatory "reportable" disease. Donald Doerr of Ogden, West valley councilman Leland DeLange, and Jill Cunliffe of Layton are the other Utahns to die of CJD this year.

Doctors also remind us that these CJD deaths are not related to beef contamination in Great Britain.