February 2, 2004  Santa Cruz Sentinel by PEGGY TOWNSEND

Instead, it was the swiftness by which her husband, Don, turned from a vibrant man with a sharp wit to a man who couldn't remember to turn off the water after he brushed his teeth.

Within a week, Don Brose went from forgetting little things and complaining of a numb face to sometimes stumbling like a drunk.

Within a month, the man who had once built a jet airplane from the wheels up ran terrified from his house at the sound of a barking dog.

Two months after his first symptom, he was dead.

"I thought we had more time," says Judy softly.

Advertisement But Don Brose's killer, a disorder called Creutzfeldt-Jakob Disease, is a swift assassin. Within a matter of months, it turns a victim's brain into a sponge-like mass, filling the sufferer's head with nightmare-like delusions and paranoid fantasies - until it finally kills them.

It affects about 300 people a year in the United States, with no known cause for the majority of cases. A variant form of the disease made headlines recently, though. It is caused by eating beef from cattle with mad cow disease.

But no matter its cause or form, Creutzfeldt-Jakob's is always a terrible disease.

Louanne Korver, Brose's daughter, watched as her father slid into a childlike state, batting the air against invisible monsters, crying because he accidentally bit down on the spoon as she fed him.

"With Alzheimer's, you see changes every few months," Korver says, "but this was hourly."

A man's man Don Brose was 71 years old, but most people thought he was about 50.

He was trim and tough, a man's man who loved to party and could design an entire house in his head before he sat down to draw it.

He flew airplanes and once drove his motorcycle from Santa Cruz to Guatemala.

A few months before his death, he was planning a 20,000-mile motorcycle trip to Tierra del Fuego and up to Brazil for Carnaval.

But last summer, a few days before he and Judy were planning to fly to the San Juan Islands for a trip on a private yacht, Don began to have chest pains.

He underwent triple bypass surgery, says Judy, a trim, energetic woman with chin-length brown hair. Eight weeks later, he felt well enough to buy a new BMW motorcycle in preparation for his trip to South America.

But on Sept. 28, he woke up his wife.

"Judy," he said, "you have to take me to the hospital right now. My face is numb. I've had a stroke."

Doctors could find nothing wrong with Don - even though he insisted something was not right.

"I'm not a doctor," Don told them, "but I'm a plumber, and I'm telling you I'm not fine."

Still, he was sent home.

"By Oct. 1, he was out of the hospital, but he was acting weird," Judy says. "At first, he was kind of forgetful. He would leave the water on after he brushed his teeth. He left the water on after he took a shower."

At the family's regular monthly dinner, he appeared drunk, chiding his daughter, Louanne Korver, over and over for getting the wrong kind of pizza dough and talking gibberish at one point.

"I was so bummed about dad getting drunk for our family dinner," Korver says.

"It turns out he didn't have a drink at all."

"It was subtle, yet so significant," Judy says of her husband's odd behavior. "You were just puzzled by it."

Then one day, Judy pointed her finger at him to make a point during a conversation, and he drew back as if he had seen a ghost.

"I thought you had a gun," he said.

Everyone thought it was just the post-surgery heart medication he was taking.

But even though he changed medications, things were about to get worse.

Proteins gone bad At first, Creutzfeldt-Jakob Disease was thought to be caused by some kind of virus because it seemed to be transmissible.

But Dr. Stanley Prusiner, a neurologist at UC San Francisco, discovered the disease was actually caused, not by a virus, but by a rogue protein called a "prion."

Prions transform normal protein molecules into deadly ones by altering the shape of the healthy molecules.

"These prion proteins take on an abnormal shape and become a template," said Dr. Michael Geschwind, a neurologist and another leading Creutzfeldt-Jakob Disease researcher at UCSF.

"When a normal molecule comes near (the prion), it also changes shape and where you had one, you now have two templates. And then you have four, and then eight," he says.

Most of the time, our body can clear out these abnormal proteins, but with Creutzfeldt-Jakob Disease, the abnormal molecules begin to overwhelm the normal ones.

No one knows what causes the disease in 85 percent of the cases - called sporadic Creutzfeldt-Jakob Disease.

"Often we see a trigger like a trauma, or illness or a stressful event that proceeds the onset of illness," Geschwind says.

But no one has figured out why.

In 10-15 percent of cases, the disease is inherited.

In a still smaller number of cases, people may be infected by contact with the brain matter from a Creutzfeldt-Jakob's victim, or get it through a surgical procedure with tainted instruments or tissue - like a corneal transplant.

But probably one of the most frightening methods of transmission is through eating beef from cattle with bovine spongiform encephalopathy or mad cow disease, as happened in Britain over the past 10 years.

This variant form of Creutzfeldt-Jakob Disease takes longer to kill than its counterpart - about a year compared to four to six months for the classic form - and its victims are often much younger.

The typical age for classic Creutzfeldt-Jakob's is 63.

The average of those with the variant form of the disease is 28.

Yet many of the symptoms - dizziness, confusion, delusions, paranoia - are the same.

Paranoia sets in It had been a few weeks, but doctors still could find nothing wrong with Don.

Then, on Oct. 21, the delusions began.

On that day, the developer and successful businessman walked into his wife's real estate office and insisted that people were making faces at him.

"And what did you do to the lights in the room?" he demanded angrily of Judy. "Everyone looks different. There's a green light around everyone."

Everything, he told her, seemed out of proportion.

Judy rushed Don to the neurologist who had examined him before but the doctor was still not sure of what was causing the symptoms.

"This is the day I knew something was weird," says Judy. "We had passed over another boundary."

The paranoia and delusions started to come more often.

Don told Judy he felt like a human VCR. He knew she was sitting next to him, he said, but his memory had rewound so he perceived that she was actually standing at the refrigerator where she had been a few minutes before.

Then one day, while driving with Judy, he accused her of altering his beloved blue Audi so that she was driving it by remote control.

When she stopped the car, he leaped out and began yanking out the fuses and wires under the hood.

On the way back home, Judy stopped the car and began to cry.

"I don't know what to do, honey," she told Don.

When she tells the story, she begins to cry again.

A low risk Experts say the risk for being infected with the variant form of Creutzfeldt-Jakob Disease through tainted beef is low.

According to the Centers for Disease Control, an estimated 1 million head of cattle were infected with mad cow disease in the United Kingdom in the '90s - having contracted the disease from eating feed containing contaminated bone and meat.

But only 148 cases of the variant form of Creutzfeldt-Jakob Disease have been diagnosed in Great Britain so far and only one case has appeared in the United States. That victim, who lived in Florida, spent many years in Great Britain before coming to the U.S.

It also does not appear that Brose contracted Creutzfeldt Jakob's from eating tainted beef. While autopsy results are still pending, doctors believe he most likely suffered from the sporadic - or more classic - form of the disease.

Dr. Geschwind of UCSF said it appears the form of Creutzfeldt-Jakob Disease that comes from eating tainted beef affects only people with a specific genetic makeup.

"It's possible another genotype will get it in 40 to 50 years," he says. But right now, genetic factors seems to make a person more susceptible to the disease.

Still the spectre of developing Creutzfeldt-Jakob's Disease from eating tained beef is chilling and new regulations have emerged in the U.S. since a 6-year-old Holstein in Washington was discovered to have mad cow disease on Dec. 23, two weeks after it was slaughtered and released for use as human food.

The U.S. Department of Agriculture now says cattle that are not able to walk (as was the Holstein's case) will no longer be used for human consumption and slaughtered cattle will not get a "passing" status for release until after the mad cow tests are completed.

In addition, "high risk" parts - such as the brain, eyes, spine and small intestines - of older animals will not be allowed to be used for human consumption.

The remainder of the animal is believed to be safe.

But Geschwind thinks the government should go further, testing more animals for bovine spongiform encephalopathy.

"It's not clear the muscle doesn't also contain prions," Geschwind says.

Prions have been shown to occur in muscles of humans and in mice, he says. "There's no reason the same thing may not be true in other animals," he says.

In the long run, Geschwind says, increased testing would be better both economically and medically.

Since the discovery of the infected cow, Don's daughter Louanne has stopped eating beef.

So has Geschwind, although, he says, "honestly, there is no clear logic to that because I have always thought the possibility of BSE being in the U.S. was there.

"For me it was just a matter of when."

Still, Geschwind - and government officials - believe the risk of getting variant Creutzfeldt-Jakob Disease is extremely low.

A swift end By the end of October, doctors at UCSF gave Don the diagnosis of Creutzfeldt-Jakob Disease, and even though the disease is always fatal, the family was glad to finally know what was wrong.

"I'd watched this special on mad cow disease, and I knew how bad it was," Korver says. "That's when I did my biggest grieving (after the diagnosis). I knew it was fast and terrible and totally untreatable."

But the next day she woke up and thought, "OK, what can we do for dad, and how are we going to make this as good as we can?"

The family decided to bring Don home to the hilltop house in Corralitos he had built himself.

Friends gathered. Music played. Food was prepared.

It was Don's favorite thing: a party.

And in between the living nightmares - the times when he believed he was drowning or fighting off invisible monsters - there were moments of lucidity.

"There were times he would look at me and cry," Korver says. "There was a recognition in his face. He would tell you how much he loved you, and he sobbed because he knew.

"He knew what he had."

Ten days after he got home from UCSF, Don grew combative and was taken back to the hospital.

A few days later, Judy realized she didn't have the six months doctors had predicted.

She would not even have a week.

At noon on Nov. 24, Don was transferred to a nursing home where the family gathered.

As evening came, his breathing grew ragged.

"I told him how much I loved him and said 'dad, we're all OK. We're all fine,'" Korver says. "I told him 'it's OK to go.'"

At 6 p.m., Don opened his eyes, and Judy leaned over him.

"Hi honey," she said softly.

Don smiled, then took one breath and was gone, his daughter says.

It had been less than two months since the day that first symptom appeared. The day when Don Brose, the man who read "Discover" magazine from cover to cover, flew airplanes and designed and built 22 homes, began to disappear.

Now that he's gone, the family is trying to regroup, to fill the empty spot where Don used to be.

"I just miss him," Judy says.

Contact Peggy Townsend atptownsend@santacruzsentinel.com.