AR-News: Woman thought to have human form of mad cow disease dies

[jim robertson]

Woman thought to have human form of mad cow disease dies
Doctors believe Charlene Singh contracted vCJD in England
By Miriam Falco
CNN
Monday, June 21, 2004 Posted: 5:47 PM EDT (2147 GMT)



Charlene Singh, pictured in December 2003, died Sunday morning.


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(CNN) -- Charlene Singh, the only U.S. resident thought to have the human 
form of mad cow disease, or variant Creutzfeldt-Jakob disease (vCJD), died 
Sunday morning.

In April 2002, the Centers for Disease Control and Prevention and the 
Florida Department of Health announced "the occurrence of a likely case of 
variant Creutzfeldt-Jakob disease in a Florida resident [then] aged 22 
years."

Health officials concluded the young woman contracted the disease when she 
lived in England. She was born there in 1979 and moved to the United States 
in 1992.

There is no cure for the disease.

"It's shocking and stunning," said Singh's aunt, Sharon Singh-Passley. "Her 
body couldn't take it anymore."

Singh, 25, died in her sleep, her aunt said.

Disease details
The first case of the human form of mad cow disease was detected in April 
1996. Most cases occurred in Britain.

The British Department of Health has reported 146 definite and probable 
cases in its country.

France has reported six vCJD cases, and Ireland, Italy, Canada and the 
United States have each reported one case, said Dr. Francois Meslin of the 
World Health Organization.

A definitive diagnosis is not possible until after death. An autopsy is 
scheduled for Singh on Tuesday, her aunt said.

Variant Creutzfeldt-Jakob disease is the human form of the brain-wasting 
disease found in cows called bovine spongiform encephalopathy (BSE). It is 
believed that people who consumed meat from cows infected with BSE may 
develop vCJD.

The disease has an incubation period of 10 to 15 years, and younger people 
are more prone to develop vCJD than the traditional form of 
Creutzfeldt-Jakob disease (CJD), the CDC reports.

The average age of vCJD patients is 29 as opposed to 65 years for CJD. 
Patients with vCJD are also sick longer -- an average of 14 months, compared 
with four and a half months for classic CJD patients.

Experimental treatment
CNN first reported Singh's story in October 2002. Doctors had told her 
family she would live only a few months.

Singh had been a young, vibrant woman until November 2001, her family said. 
That's when she began to forget things and lose her temper.

Doctors prescribed antidepressants, but her condition continued to decline. 
Her father, Patrick Singh, said, "her hand began to shake pretty rapidly. We 
decided ... this can't be depression. Depression doesn't make your hand 
shake. It doesn't make you walk and stumble."

Dissatisfied with the diagnosis from American physicians, Singh's mother 
took her to England in early 2003, where she was diagnosed with probable 
vCJD.


Charlene Singh is shown in this high school photo.
Singh had "received experimental treatment with quinacrine for 3 months," 
the CDC reported, but her condition did not improve. Her family did its best 
to make her comfortable.

A neurosurgeon who saw media reports about her offered and gave Singh 
hyperbaric treatment, pumping pure oxygen into her lungs to try to improve 
brain function.

Her family and the doctor felt those treatments helped her. Last year, 
Singh's relatives said they thought she seemed a little more relaxed and 
appeared to try to respond to simple commands.


http://www.cnn.com/2004/HEALTH/06/21/madcow.patient/index.html



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