Two neurodegenerative infections that Fiocruz is investigating in Rio de Janeiro are suspected of Creutzfeldt-Jakob Disease (CJD), says the Ministry of Agriculture, Livestock and Supply (Mapa).
At first, the Oswaldo Cruz Foundation (Fiocruz) had informed that they were suspected of Bovine Spongiform Encephalopathy, popularly known as mad cow disease. However, considering clinical and radiological aspects, the foundation explained in a second moment that suspicions are focused on CJD.
About CJD, Mapa says that “the disease occurs, in most cases, sporadically and has an unknown infectious cause and source.” The folder also says that, “in this way, the suspected cases are not related to the consumption of beef or by-products contaminated with Bovine Spongiform Encephalitis (BSE), known as the ‘Mad Cow’ disease”.
Fiocruz says “that the two patients hospitalized for investigation of Bovine Spongiform Encephalopathy (‘mad cow disease’) are suspected of having the sporadic form of Creutzfeldt-Jakob Disease (CJD), considering the clinical and radiological aspects”. The institution also says that “this sporadic form is not related to meat consumption. We reiterate that the patients are admitted to the Hospital Center for the Covid-19 Pandemic of the INI and that both cases do not have diagnostic confirmation”.
According to the Ministry, “according to information available on the Ministry of Health website, between 2005 and 2014, 603 suspected cases of CJD were reported in Brazil. Since CJD surveillance was instituted in Brazil, no case of the vCJD form has been confirmed. vCJD is a variant of CJD associated with beef consumption.”
Understand Creutzfeldt-Jakob Disease (CJD)
A Creutzfeldt-Jakob disease, a fatal neurodegenerative disease that affects the central nervous system, caused by infectious agents called prions. Patients may have reasoning disorders, memory loss, speech impairment, lack of coordination of muscle movements and tremors.
Prions are particles made up of proteins found in cells of the nervous system of humans and other mammals. Under normal conditions, the cellular prion protein (PrPc) does not harm the body. It has several physiological functions, such as protecting neurons against cell death.
However, for reasons that are not fully understood, these proteins can show errors during their formation. These errors lead to changes in the protein’s structure, which becomes infectious and toxic.
“Under disease conditions, the protein starts to form aggregates that are infectious. They can then jump from one neuron to another, causing them to die. When the neuron dies, there is a loss of brain mass”, explained Sérgio Ferreira, a professor at the Institutes of Biochemistry and Biophysics at the Federal University of Rio de Janeiro (UFRJ).
About 85% of cases are sporadic, usually affecting people between 55 and 70 years old, with an incidence of 1 or 2 cases for every 1 million inhabitants. Between 10 and 15% are due to an inherited mutation in the prion. Also rare occurrences were recorded as a result of surgical procedures and through the use of contaminated instruments.
Reference: CNN Brasil
