Hospital Tests Man for Brain Illness: ; CAMC Surgeon Did Biopsy to See If Patient; Has Human Form of Mad Cow Disease
DAILY MAIL STAFF
Physicians at Charleston Area Medical Center recently treated a patient who initially tested positive for a rare, fatal disorder that is the human equivalent of mad cow disease.
Creutzfeldt-Jakob disease (CJD) prompts rapid, progressive dementia and death.
But health officials said this elderly man’s illness has not been linked to eating tainted beef or deer. Privacy laws prohibit naming the man.
“This is a different form with a different cause,” said Dr. Julie Sinclair, the Epidemic Intelligence Officer assigned to the state Bureau of Public Health by the Centers for Disease Control and Prevention. “There is an undetermined cause in this guy. They are doing further investigation.”
Dr. John Schmidt, who performed a brain biopsy and another procedure on the patient, said this would be his first CJD case in 25 years of doing neurosurgery.
“This is a big deal,” Schmidt said. “You have a diagnosis of CJD. It’s really weird – not from a virus, not a bacterium and uniformly fatal. It takes a long time to develop.”
Hospital safety officers had to take special precautions for the surgery, from removing all extraneous equipment from the operating room to quarantining the surgical instruments. If specialized lab work confirms a definitive diagnosis of CJD, then the instruments would have to be destroyed, Schmidt said.
And all medical waste collected had to be immediately incinerated, said CAMC spokesman Dale Witte.
Though no cases of iatrogenic (caused by medical treatment) CJD have been reported since current sterilization procedures were adopted in 1976, transmission of CJD occurred in at least 250 patients worldwide, according to the CDC. These were linked to contaminated human growth hormone, corneal grafts or surgical equipment.
Schmidt said he knows of one case of a brain surgeon infected with CJD after he operated on a patient with it.
Still, CJD is extremely rare, happening in only one in a million people, Sinclair said. Most cases are confirmed through a post- mortem exam.
Since 1979, 31 deaths have been attributed to CJD in West Virginia, Sinclair said. All the victims were older than 40 and most were over 60.
Most cases occur sporadically. That means no recognizable pattern of transmission exists.
Meanwhile, the Centers for Disease Control is quietly reviewing several mysterious deaths of young Americans from CJD to discern if they may have eaten cows with the disease. Deaths have occurred over the last five years in several states, including New Jersey, Texas, Michigan and Wisconsin, according to the Associated Press.
A small percentage of patients develop CJD because they inherited mutations of a protein gene called a prion.
But whatever the cause, people with the disease usually die within a year from what is classified as transmissible spongiform encephalopathy. Medical officials declined to comment on the prognosis of the man treated at CAMC.
While the disease is limited among animals, it is not uncommon, said state Agriculture Commissioner Gus Douglass.
“Mad cow disease, scrapie in sheep and chronic wasting in deer are all from the same family,” he said. “We know these are transmittable to humans. Others, medical science has not proven they are transmittable. I’m not surprised we might find CJD in West Virginia.”
However, the state has never had a case of mad cow disease, Douglass said.
Indeed, it has never been determined that a mad cow case has originated in the United States, he said.
Sinclair said the bureau received the report of the CJD case from a physician treating the elderly patient.
“This is a very good physician, on the ball,” Sinclair said, noting that because the condition is so rare, it would be difficult to diagnose.
Sinclair also said it has not been shown that CJD could be transmitted casually from person to person.
“Family members don’t have to be concerned,” Sinclair said.
Contact Therese Smith Cox at therese@dailymail.com or 348-4874.