Another American CJD Victim
By Misti Crane
Columbus Ohio Dispatch Medical Reporter
A rare affliction similar to mad cow disease has killed a 68-year-old North Side man.
Kurt Weiner, who owned Kurt & Son Ski Haus in Westerville, died yesterday afternoon, just weeks after a brain biopsy showed he had Creutzfeldt-Jakob disease.
"He was a wonderful man,'' said his sister, Erika Bell of Virginia, who witnessed her brother's quick deterioration.
Last week, Weiner's family moved him from Ohio State University Medical Center to a Westerville nursing home, knowing he would live out his final days there. There is no treatment for CJD, a brain disorder that causes a rapid, progressive dementia and usually kills its victims within a year.
Weiner's son, Kurt Weiner II, 32, will take over the business, Bell said. The elder Weiner had no other children.
According to the state Health Department, the disease is diagnosed in 10 Ohioans a year.
A different form of CJD has been linked with eating meat from cows with mad cow disease -- more properly known as bovine spongiform encephalopathy. No such cases have been documented here, according to the U.S. Centers for Disease Control and Prevention in Atlanta.
Creutzfeldt-Jakob disease is diagnosed in about one in a million people nationwide each year. It affects both men and women of diverse ethnic backgrounds.
Most cases -- about 85 percent to 90 percent -- are considered sporadic, meaning there is no known source and no evidence that it is inherited, according to the CJD Foundation, based in North Miami, Fla.
About 10 percent of cases are inherited, with genetic factors connected to elevated numbers of CJD cases in some communities in the former Czechoslovakia and Chile, as well as among Libyan-born Jews.
A very small percentage of the illness is thought to have been passed along through a medical procedure, including cornea transplants and implants of electrodes in the brain; contaminated surgical instruments; and the injection of natural human- growth hormone derived from cadavers.
Weiner had a cornea transplant 12 years ago, which might be to blame for his disease, Bell said.
CJD symptoms can include insomnia, depression, confusion, personality and behavioral changes, strange physical sensations and problems with memory, coordination and sight.
Until a couple of weeks ago, Weiner, a vibrant, active Austrian native, could still recognize his older sister, she said. Minor symptoms began after a carbon-monoxide scare in July, prompting doctors to speculate his difficulties might be related to that incident, Bell said.
But dizziness quickly gave way to more serious problems, prompting Bell, who is 75, to come here in September to be with her brother.
"He and I were extremely close,'' she said, adding that "half of Columbus probably will miss him.''
Weiner first moved to Columbus from Austria in the 1950s. He took his first job in the Lazarus sporting- goods department, Bell said. He was skiing as recently as this spring and was known for his ski instruction in Cincinnati and Mansfield, she said.
Scientists originally thought CJD was caused by a slow-acting virus, because the incubation period between the time of exposure and the onset of symptoms can be decades. Further research has led to the theory that it is carried by an infectious protein, or prion.
Prions also are connected with other rare, fatal brain diseases in humans and animals, including mad cow disease.
Federal agriculture officials say extreme vigilance and restrictions on what farmers can feed cattle has so far kept mad cow disease out of the United States.
Most of the 178,000 cases of the disease diagnosed worldwide have occurred in Great Britain, where it was first reported in 1986.
The U.S. Agriculture Department banned the import of British cattle in 1989, and extended the ban to other European countries in 1997.
Also in 1997, the U.S. Food and Drug Administration banned the feeding of animal protein, such as meat and bone meal, to cattle and sheep. Scientists think mad cow disease is transmitted to cattle when they eat feed containing meal made from infected animals.
Business reporter Paul Souhrada contributed to this report.

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