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Concern over high incidence of mad cow disease

Three patients suffering from Creutzfeldt–Jakob Disease (CJD), a rare and fatal degenerative brain disorder, have been admitted to the Department of Neurology in the Government General Hospital here.

The high incidence of the CJD, termed as Mad Cow Disease, has caused a great deal of concern. At least six cases have been reported at the GGH in the last one year.

The disease, characterised by rapidly progressive dementia, with an incidence rate of 1 in 1.5 million, was first discovered by Prusiner, who later won a Nobel Prize.

A 30-year-old woman, Ratna Kumari, from Chirala in Prakasam district, has been admitted with symptoms ranging from depression, anxiety, incoherent talk and unwieldy gait, known in medical parlance as myoclonic jerks.

“We initially believed it to be a psychiatric condition. An MRI scan, however, revealed classical CJD Cortical Ribbon Sign (CRS). Later, an EEG test confirmed our findings. Unfortunately, the CJD cannot be cured and the prognosis is rather painful,” professor and Head of Department of Neurology N.V Sundarachary told The Hindu on Wednesday.

Rattaiah of Krishna district was the third person admitted to hospital.

Sporadic disease

Occurrences of the CJD could be sporadic, inherited, or infectious.

The alarming trend is that there is an increase in the sporadic disease. It can also be transmitted from person to person like the HIV.

The first suspected human transmission of the disease was reported in 1974 when the rapidly progressive disease was diagnosed in a woman 18 months after she received a corneal transplant.

The donor had died of undiagnosed CJD.

Risk group

Variant CJD (vCJD) is caused by eating contaminated beef products.

Last month, a 60-year-old woman, Samrajyam, who was diagnosed with CJD, died here. She was referred to NIMHANS in Bengaluru. But since there was no possibility of any curative treatment, she was brought back to Guntur.

The reason behind the spread of CJD also dispelled many myths, including that of viral disease.

“The disease is caused by infectious protein particles called prions, which mainly attack the neurons of the brain. The onset of CJD begins with the Protenicuous Particular Cellular (Prpc), with a soluble nature, turning into an insoluble nature (PrPsc), crystallising, and later degenerating the cells. People consuming contaminated meat are at risk of getting infected with CJD,” said Prof. Sundarachary.

Eighty per cent of sporadic cases of CJD are diagnosed in persons between 50 and 70 years. About one-third of patients initially express vague feelings of fatigue, disordered sleep, or decreased appetite, he said.

Some have neurological symptoms, such as memory loss, confusion, or uncharacteristic behaviour.

At least six cases have been reported in the GGH in Guntur in the

last one year

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