Mad Cow Case Raises
Suspicions In Elderly Deaths

By Julie Davidow
Seattle Post-Intelligencerer

Rose and Jim Henry wanted a home brimming with babies and togetherness.
In nearly 50 years of marriage, the high-school sweethearts had 10 children and rarely spent time apart.
So after Jim Henry died, when the grown children stopped by their mother's Olympia home to find her sitting alone in the dark or forgetting to bathe and eat, some assumed she was simply adjusting to life alone in a big house -- the same home once filled with family.
But after watching their mother swiftly consumed by a mysterious ailment and die, Henry's children opted for an autopsy.
"I have a lot of brothers and sisters and we all wanted to know for the benefit of the family, was this Alzheimer's?" said Jeanne Giese, Henry's oldest.
The autopsy was conducted shortly after her death in 1997 at age 74. But because of bureaucratic bunglings yet to be explained, the family didn't get the results until early this year -- just a few weeks after the nation's first case of mad cow disease was discovered near Yakima.
The conclusion: Rose Henry suffered from Creutzfeldt-Jakob disease.
Now, largely because of that mad cow case, more people are wondering whether their elderly relative might have suffered from CJD -- a degenerative disease of the nervous system -- or its variant, the human form of mad cow disease.
And with so little known about the deadly diseases, some are asking if any of those cases might be traced back to bad beef.
Not likely, say most scientists.
But at the same time, doctors are being urged to more carefully diagnose dementia and pursue autopsies for suspect cases -- only an autopsy can determine whether CJD is present and in what form.
Many elderly people with dementia or other symptoms of Alzheimer's probably are never seen by a neurologist. And although Alzheimer's, CJD and human mad cow disease look quite different to a specialist, there are common symptoms that many doctors might not recognize.
"I think it's a rare enough condition that most neurologists have not seen very many cases, either," said Dr. Eric Larson, director of the Center for Health Studies at Group Health Cooperative and head of a research study on Alzheimer's and aging.
Unlike the Henrys, most families with a loved one with dementia do not opt for an autopsy.
Jeanne Hall went to her grave in January at age 83 without an autopsy.
Now her son isn't sure that was the right decision.
When Hall started losing her balance about a year ago, Steve Hall took her to the doctor. She'd been treated for vertigo. She thought she had wax in her ears.
"She was a tough old lady," said Hall, a Tri-Cities contractor. "She was trying to fix herself."
The first doctor said she probably had a stroke. The second thought she might be suffering complications from a brain tumor.
Meanwhile, Jeanne Hall continued to deteriorate. Her speech slurred. And she had trouble directing her legs, said Hall, who watched her stand up from the couch, take several steps to one side and spin around to face him.
"She got to her feet and did a cross step like Charlie Chaplin," Hall said.
Her movements reminded him of the cows, described in news reports on mad cow disease, that couldn't walk straight or stand up.
Doctors assured him his mother did not die of human mad cow disease, but Hall wonders why they never suggested an autopsy.
"Older people tend to be put on a shelf," Hall said. "Why wouldn't you have an interest in following up with people like my mom who die if not mysteriously, then certainly undiagnosed?"
Closer watch
The aftermath of Rose Henry's death illustrates why the system doesn't always work. After her death in August 1997, her family waited for the final word. The children paid for an autopsy.
More than six years later, her records surfaced.
Prompted by a Seattle Post-Intelligencer reporter's request, Dr. Tom Montine, head of neuropathology at the University of Washington, dug up Henry's slides.
He studied the brain samples himself and confirmed the preliminary diagnosis of traditional CJD, for which there is no known cause.
"Obviously with Rose Henry there was a breakdown in communication," said Montine, who arrived at the UW in 2002. "We can't let cases fall through the cracks and have families wondering what really happened."
A new statewide surveillance system is meant to close the loopholes that could be letting other cases such as Henry's go undiagnosed or unrecorded.
Otherwise, "We're never going to know anything about these diseases," said Jo Hoffman, state epidemiologist. "We're never going to know what the risk factors are and we're never going to know how people are exposed."
About one in a million people come down with traditional CJD each year. Since 1997, 35 people in Washington state have died of traditional CJD, according to state health department records. Less than half of those cases were diagnosed with an autopsy.
Scientists believe people contract variant CJD by eating beef from cows infected with bovine spongiform encephalopathy, or mad cow disease.
The mutant proteins believed to cause these brain-wasting diseases are called prions.
There are about 150 known cases of human mad cow disease in England and Europe. Only one U.S. resident has been diagnosed with mad cow disease -- a Florida woman who grew up in England.
Closer surveillance is unlikely to unearth a hidden epidemic but it's possible some CJD cases are being missed, say doctors and researchers.
"Not everybody's going to have the stereotypical pattern (of symptoms)," said Montine. "Do they have CJD? Are they basically flying under the radar?"
Next month the state Department of Health will send out hundreds of letters to neurologists and other doctors urging them to report all suspected cases of human prion disease to the state and talk to families about having autopsies performed.
Tissue samples would then be sent to the National Prion Disease Pathology Surveillance Center in Cleveland. Opened in 1997, the center is responsible for tracking prion diseases and spotting new strains.
Biochemical tests can easily distinguish between CJD and other varieties of dementia, but in a doctor's office, it's often difficult to pinpoint the source of a patient's symptoms.
There are some key differences, however.
CJD hits quickly, for example, with symptoms that advance rapidly for one to two years before death. Alzheimer's takes years to progress.
CJD and its variant form are also distinct.
The average age of traditional CJD victims is 60. In England, British scientists and doctors suspected they were dealing with a new strain when younger people started dying of a brain-wasting disease that looked like CJD, Montine said.
Human mad cow disease also often starts with psychological, rather than physical, symptoms.
"If you weren't tuned in, you might miss it, at least initially," said Dr. Christina Marra, a UW neurologist.
Depression and personality changes are the first signs, followed by pain, tingling and other sensory problems.
"Almost everyone (diagnosed with human mad cow disease) with rare exception was first seen by a psychiatrist," Marra said.
Some research suggests the source of infection for the two forms of CJD could be related.
When injected with misfolded proteins believed to cause mad cow disease, mice genetically engineered to have human traits came down with a disease that resembles sporadic CJD, according to a 2002 study in England.
And, in a study published last month, Italian researchers said they found a second strain of mad cow disease that looks a lot like traditional CJD.
Labeling traditional CJD "sporadic" is a way of getting around the fact that no one knows what causes it, said Dr. Laura Manuelidis, a neuropathologist at Yale University. Parting with the prevailing theory, she believes the culprit is more likely some type of infectious agent, not misfolded proteins. "Spontaneous CJD just means we don't know where the infection is coming from," she said.
There's also evidence that some cases of CJD could be mistakenly tagged Alzheimer's or some other type of dementia.
Swiss officials found a CJD rate of three cases per million after improving their tracking system -- triple the previously accepted rate.
In a small study at Yale led by Manuelidis in 1989, researchers discovered that 13 percent of patients diagnosed with Alzheimer's had CJD.
"The conclusion people might draw is that CJD is underdiagnosed and clinically can't be discriminated that easily from Alzheimer's disease," Manuelidis said.
Still, only three cases of prion disease have surfaced among the hundreds of autopsies performed as part of a 20-year research study on Alzheimer's at the UW, according to Gerard Schellenberg, a genetic researcher at the Veterans Affairs Puget Sound Health Care System. [Note: Schellenberg's first name has been corrected since this was originally published.]
"Those brains are looked at extreme carefully and you just don't turn up random CJD cases," Schellenberg said.
For the victims and their families, of course, statistics are not the point.
Losing their mother
"Look at that woman over there. She looks so weird!" Rose Henry shouted one Sunday morning at Mass. The mother of 10 children, Henry had strict rules about how to conduct yourself in public. The girls should act like ladies, the boys like gentleman. No arguing with strangers. No rude outbursts.
"I grabbed her elbow and I said, 'Mom, be quiet!' It was like she didn't know she was being inappropriate," Giese said.
Other changes followed.
Henry began reacting to noise like someone burst a balloon near her ear. A slammed kitchen cabinet door or a car backfiring caused her to leap up and gasp.
"The boys were in denial," remembers Patty Anderson, one of the couple's six daughters. "They said she's just trying to get used to Dad being gone."
Those who lived closer knew better. They just didn't know why or what was gnawing away their mother's mind.
An internist told the family she had dementia. Giese's brothers and sisters prepared themselves for their mother's long slide into oblivion.
A trained nurse, Giese didn't buy it.
"When the others would say she obviously has Alzheimer's, I would say, 'No, it doesn't fit. She's going too fast.' "
Henry eventually lost her balance, hung Army blankets on the windows to keep out light and tried to use the TV remote to make phone calls.
At times, says her family, it seemed Henry knew she could no longer rely on her mind.
"She would pace around and wring her hands and she'd say 'Stay with me, stay with me, I'm afraid.' It was so sad," Giese recalls.
When Henry started having seizures, a neurologist spotted signs of CJD on a brain scan.
She died four months later, blind, unable to talk or walk.
Unfamiliar with the two varieties of CJD until recently, the family has always referred to their mother's disease as "mad cow."
And they still wonder if the beef Jim and Rose ate on a dream trip to Ireland and England in 1978 had anything to do with their mother's death.
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