Rare Brain Ailment Tough to Spot in US

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http://www.philly.com/mld/philly/living/health/3260917.htm

Tue, May. 14, 2002

Rare Brain Ailment Tough to Spot in US

BY MEG BRYANT

WASHINGTON - (Reuters Health) - All too often, patients in the US with a

rare brain-wasting illness similar to the one caused by " mad cow " disease

may end up with no one catching on to the cause of their unusual symptoms,

researchers reported at the American Geriatrics Society meeting here.

The condition is called sporadic Creutzfeldt-Jakob disease (CJD) and the

number of people who die of the disease in the US is low--less than one per

million people per year, according to the US Centers for Disease Control and

Prevention. However, researchers at the University of Louisville in Kentucky

think it may be more common, yet unrecognized. They reported the case of a

68-year-old woman with the ailment who was initially misdiagnosed.

Sporadic CJD occurs at random and is not linked to the consumption of meat

contaminated by bovine spongiform encephalitis, commonly known as mad cow

disease. However, it does share some similarities with new variant

Creutzfeldt-Jakob disease (vCJD), the mad cow-related ailment that has

killed more than 100 people in Europe, most of them in Great Britain.

Although similar diseases, sporadic CJD most often strikes at an average age

of 65, while the average age for vCJD is 29. Both forms of the disease are

fatal, eventually cause spongy holes to form in the brain, and the first

symptoms can be mistaken for other ailments.

In vCJD, severe depression followed by schizophrenia-like delusions and

hallucinations are often the first signs of the fatal brain degenerative

disorder. In sporadic CJD, the first symptoms are often memory loss and

unsteadiness, which can be confused for some other ailment, such as

Alzheimer's disease.

In the case of the 68-year-old woman, her first symptoms were agitation,

delusions and loss of coordination. First thought to be experiencing mental

illness, the woman was treated with antipsychotics and antidepressants. When

those failed to help, she underwent brain scans that indicated a

normal-appearing brain. Only an electroencephalogram (EEG)--a measure of the

brain's electrical activity--showed characteristic signs of the

brain-wasting disease. The patient eventually died.

Although only a handful of cases are reported in the US each year,

difficulty in diagnosing CJD could mean the incidence is actually higher,

lead investigator L. K. Tanwani, of the University of Louisville, told

Reuters Health. " One of the reasons we're not seeing more cases is that

we're not looking for it, " he said.

The diagnosis needs to be confirmed with an autopsy, but the researchers

found resistance on the part of the patient's family and concerns by

neurosurgeons and pathologists that an autopsy would contaminate the

operating room and surgical instruments. In fact, the hospital where the

patient died initially refused to perform an autopsy. Even though sporadic

CJD occurs at random--and is not thought to be caused by an infectious

agent--the brain tissue of people with the disease may pose a small danger

to others.

There have been a few cases in which neurosurgical instruments used on

patients or neurological tissue taken from CJD patients have spread the

disease to others during operations. However, no such cases have been

reported after 1976, when sterilization procedures changed, according to the

Centers for Disease Control and Prevention. The World Health Organization

issued a set of guidelines in 1999 for the proper handling of CJD patients

after death.

To improve diagnosis and combat fear and misunderstanding about the disease,

the researchers recommend better education of healthcare professionals,

patients' families, and funeral homes about the proper handling of CJD

patients.

According to Tanwani, countries like Great Britain, where vCJD cases make

the disease in general more common, have begun to develop protocols and

in-service training programs for professionals handling CJD patients, and

these could become a model for hospitals and funeral homes in the US. " There

is a definite need for it (here), at least in the big geriatric centers, " he

said.