Probable sCJD found in 64-year-old woman: SSM

2024-01-30 03:26
BY Yuki Lei

The Health Bureau (SSM) said in a statement yesterday that it received a report about a probable sporadic Creutzfeldt-Jakob disease (sCJD) case from the public Conde de São Januário Hospital Centre, stressing that the case has not been linked to eating beef.

The statement noted that sporadic CJD is not transmitted through ordinary day-to-day contact with those affected.

According to the statement, the patient is a local woman aged 64. She had sought treatment at a hospital in the mainland and a medical institution in Macau, with progressive memory loss and inability to answer questions which had developed since August last year.

The victim was hospitalised in the public hospital after her symptoms were progressively aggravated with psychomotor agitation, the statement said, adding that cerebrospinal fluid (CSF) samples of the patient tested positive for prion proteins.

The patient was discharged from hospital earlier this month, the statement noted, which added that she was in a fair condition.

The statement quoted the victim’s family members as saying that they did not develop similar symptoms as the patient’s, adding that the patient, who had lived in Taiwan for about eight years a decade ago, has never received blood transfusions or undergone surgery.

No surgery was performed during the patient’s hospitalisation, the statement underlined, which noted that all the prion-contaminated medical instruments used for the patient were disposed of as infectious waste by having been burnt at high temperatures.

A prion is a self-propagating misfolded conformer of a protein that is responsible for a number of diseases that affect the brain and other neural tissue.

The statement pointed out that CJD, the commonest human prion disease, is classified as a transmissible spongiform encephalopathy (TSE) along with other prion diseases, divided into two categories – variant and classic. The variant is associated with the consumption of beef or beef products from cattle with bovine spongiform encephalopathy (BSE – Mad Cow Disease), while the classic has nothing to do with the consumption of beef or beef products with BSE, and can be divided into sporadic, familial and iatrogenic types.

Iatrogenesis is the causation of a disease, a harmful complication, or other ill effect by any medical activity, including diagnosis, intervention, error, or negligence.

The incidence of sporadic CJD around the world stood at about one individual per million each year, with only a total of three sCJD cases reported in Macau in 2012, 2017 and 2019.

CJD is characterised by progressive dementia, ataxia (a term for a group of disorders that affect coordination, balance and speech), muscle stiffness and twitching, and blurred vision, the statement noted.

According to Britain’s NHS website, CJD appears to be caused by an abnormal infectious protein called a prion. These prions accumulate at high levels in the brain and cause irreversible damage to nerve cells.

There’s still no proven cure for Creutzfeldt-Jakob disease (CJD), but clinical studies are under way to investigate possible treatments. At present, treatment involves trying to keep the person as comfortable as possible and reducing symptoms with medicines. 

This image taken from Wikipedia last night shows the magnetic resonance image of sporadic Creutzfeldt–Jakob disease (sCJD).


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