December 3, 2021

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Rare and Fatal: Understand What Creutzfeldt-Jakob Disease (CJD) Reported by Fucruz |  the health

Rare and Fatal: Understand What Creutzfeldt-Jakob Disease (CJD) Reported by Fucruz | the health

On Thursday (11) the Oswaldo Cruz Foundation (Vocruz) announced two suspected cases Sporadic Creutzfeldt-Jakob Disease (CJD) In the residents of Baixada Fluminense. Although confused with “mad cow disease,” the disease is not related to the consumption of beef.

There is no cure for this disease The average life expectancy after diagnosis is one year. they exist Four known forms of CJD: sporadic, genetic, iatrogenic and neo-variant (vCJD), all of which are fatal.

This is not the first time the disease has been reported in the country. Between 2005 and 2014, 603 suspected cases were reported According to the data of the Ministry of Health.

In this article, review the answers to the following questions:

  1. Are “mad cow disease” (bovine spongiform encephalopathy) and Creutzfeldt-Jakob disease (CJD) the same thing?
  2. What is the similarity between diseases?
  3. Are there other forms of Creutzfeldt-Jakob disease?
  4. What is the difference between Creutzfeldt-Jakob disease (CJD) and a new variant of Creutzfeldt-Jakob disease (vCJD)?
  5. Are both CJD and vCJD fatal?
  6. How is the diagnosis made?
  7. Is this the first time that cases of Creutzfeldt-Jakob disease (CJD) have been identified in Brazil?

Fucruz is investigating two suspected cases of mad cow disease

1. Are “mad cow disease” (bovine spongiform encephalopathy) and Creutzfeldt-Jakob disease (CJD) the same thing?

No. bovine spongiform encephalopathy (BSE), The official name for mad cow diseaseIt is a degenerative, chronic and fatal disease It affects the central nervous system of cattle and buffaloes.

The confusion between the two diseases arises due to a different type of Creutzfeldt-Jakob disease (vCJD), which affects people who have eaten meat or meat products contaminated with BSE.

2. What are the similarities between diseases?

“Both prion diseases are caused by a change in a protein called a prion. There are many diseases caused by prions and they are all rare, the most common of which is sporadic Creutzfeldt-Jakob disease,” explains Wagner Syd Palmira Cavalcanti, a doctoral student in neurology at the College of Medicine. University of Sao Paulo (FMUSP).

Prions are found in the brain of many mammals, including humans – however, they can become pathogenic by adopting an abnormal shape and reproducing excessively.

When this happens, the prion kills the neurons and instead White holes remain in the brain, hence the name “spongy” disease., where the holes are similar to a sponge.

3. Are there other forms of Creutzfeldt-Jakob disease?

Sim. According to the ‘Protocol for Notification and Investigation of Creutzfeldt-Jakob Disease’, issued by the Ministry of Health in 2018, There are four known forms of Creutzfeldt-Jakob disease: sporadic, hereditary, iatrogenic and neo-variant (vCJD).

Approximately 84% of cases occur sporadically and without a recognizable pattern of transmissibility. So far, Vucruz suspects this is the form of the disease that has occurred in the cases identified in Rio de Janeiro.

Sporadic CJD, the most common form of the disease, appears spontaneously. There is no identifiable cause. The person has the disease out of nowhere, unfortunately, unfortunately‘,” says neurologist Fabio Porto, scientific director of the Brazilian Alzheimer’s Association (ABRAz).

In addition to iatrogenic form, It is very rare (representing less than 6% of cases) and It results from accidental transmission through contaminated surgical equipment or through corneal or meningeal transplants (dura mater) or by administering growth hormones extracted from the pituitary gland of corpses.

4. What is the difference between Creutzfeldt-Jakob disease (CJD) and a new variant of Creutzfeldt-Jakob disease (vCJD)?

Be careful not to confuse the two diseases. while the first, Creutzfeldt-Jakob disease is spontaneous, and Creutzfeldt-Jakob disease is associated with consumption of contaminated beef or meat products. By bovine spongiform encephalopathy (BSE).

In addition, Creutzfeldt-Jakob disease affects people between the ages of 60 and 80 while the variant usually occurs in younger people under the age of 30.

Mad cows – Photo: Art Editoria / G1

5. Are both CJD and vCJD fatal?

Sim. According to Porto, these Diseases are characterized by the rapid development of dementia.. Life expectancy after diagnosis is very short: six months to a year.

Since there is no cure for the disease, the patient is given palliative treatment aimed at providing relief and pain relief as the disease progresses.

6. How is the diagnosis made?

The clinical examination should be carried out by a medical professional (preferably a specialist – a neurologist, an infectious doctor or a psychiatrist) and the clinical picture should be checked to identify typical signs and symptoms of the disease, as well as to make a diagnosis and differential bases for elimination of other diseases with dementia, such as encephalitis Chronic meningitis.

Certain imaging tests indicate suspected Creutzfeldt-Jakob disease (EEG, cranial magnetic resonance tomography, computed tomography) and help with clinical examination to classify conditions.

7. Is this the first time that cases of Creutzfeldt-Jakob disease (CJD) have been identified in Brazil?

No. In Brazil, according to an official document issued by the Ministry of Health in 2018, 603 suspected cases of Creutzfeldt-Jakob disease were reported in the country between 2005 and 2014.

  • 55 confirmed;
  • 52 were eliminated;
  • 92 was unspecified
  • 404 Final rating ignored or missing;

Since the start of surveillance for Creutzfeldt-Jakob disease (CJD) in Brazil in 2005, the infection has become part of the list of mandatory reporting diseases. As of 2018, the document’s release date, no case of the vCJD form has been confirmed in the country.