/ que estabelece regras para a prevenção, o controlo e a erradicação de determinadas encefalopatias espongiformes transmissíveis, no respeitante à . 1 jul. que regula a utilização de matérias de risco no que respeita às encefalopatias espongiformes transmissíveis e altera a Decisão 94//CE. regras para a prevenção, o controlo e a erradicação de determinadas encefalopatias espongiformes transmissíveis (Texto relevante para efeitos do EEE).

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To increase the timeliness of detection of human cases of the new variant of Creutzfeldt-Jakob disease vCJD and to reduce the risk of transmission, the Brazilian Ministry of Health has established and standardized rules and control measures. These include the definition of criteria for suspect cases, reporting, monitoring, and control measures for illness prevention and transmission. Guidelines to be used by the team of health care staff were published and distributed to health workers.

A detailed proposal for a simplified system of surveillance for prion diseases was developed and mandatory reporting introduced. Additional effort is necessary to increase vCJD case detection, thus making it necessary to establish a partnership with health care services for best identification of suspected cases and dissemination of information to all involved in the service dealing with vCJD investigation.

In view of the recent emergence of the new variant of Creutzfeldt-Jakob Disease vCJD ttransmissveis humans, along with its social and economic repercussions, the Brazilian Ministry of Health transmissvesi specific activities directed toward prevention of CJD inby commissioning a Task Force with the following purposes: Materials derived from ruminants are used as components in the production of medicines, cosmetics and other health related products. Examples of health related products made from materials of animal origin include: For import of such products, ANVISA the National Health Regulatory Agency has established several legal requirements based on the potential degree of infectivity of animal tissue encffalopatias imported and the risk attributed to the country of origin, defined by the number of infected cases identified in the region.

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Information on these measures is available at: At this time however, there was no surveillance protocol in place. The implementation of the system is currently directed towards the timely discovery of possible cases of Creutzfeldt-Jakob CJD Disease and its variant vCJD ; aiming to reduce under-reporting of prion diseases in Brazil; to better understand the epidemiological profile of this illness throughout the country; to enable epidemiological, clinical and laboratory investigation of reported cases; to adopt individual and collective protective measures upon detection of new cases of such diseases; and to deploy possible prevention and control measures in the event of cases being identified within Brazilian territory.


The system still has some limitations, mainly concerning laboratory diagnosis and the very transmidsveis of Human Prion Diseases. Despite the difficulties outlined, the state surveillance services have successfully adapted their routines during investigations of suspected cases of prion disease and have managed, with the assistance of reference laboratories and neurology services involved, to notify a number several of cases given the rareness of the disease and eslongiformes nature of the proposal.

With regard to laboratory surveillance, collaboration amongst research centers has been pursued, to devise possible, often highly complex, methodologies.

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In spite of current advances in diagnosing the disease, espongiforms is recommended that other tests available for detection should be used in conjunction. Molecular diagnosis is being carried out by the Ludwig Institute, through purification of the DNA obtained after leukocyte separation.

Polymorphism studies and mutation research in the human prion gene PRNP can be used as an aid to diagnose CJD, whereby researchers have associated the occurrence of polymorphisms at codon and protease resistant chemical-physical properties of cerebral prion protein PrP Sc with the disease. Two PrPSc strains have also been reported, namely Types 1 and 2, along with three possible genotypes at codon Methione homozygous MMvaline homozygous VV and heterozygous], responsible for 6 sub-types of the disease.

Concerning histopathological diagnoses, sending of material to Rio de Janeiro Federal University laboratory is in place, where macroscopic studies of the dspongiformes, frontal lobe fragments, basal ganglia, temporal lobe, occipital lobe, mesencephalon, bridge, bulb and cerebellum are being carried out.

The diagnosis is considered positive for CJD when the following 3 pathognomic characteristics of the disease are identified: Spongiosis, neuronal loss and gliosis.


The microscopic aspect reveals spongiosis of the cortex due to reactive astrocytic encefalopaatias, in proportion to the degree of neuronal loss. Constant improvements in surveillance activities are aimed primarily at increasing the sensitivity of the system for early detection of vCJD cases, in view of the burden these place on the Brazilian health system.

The decision to include other prion disease in the system stems from the syndromic features displayed by these pathologies, particularly in their initial stages, which make the devising of a notification form exclusively for suspected cases of the new variant unrealistic.

Thus, all prion diseases are reported. At a later stage, cases not meeting the criteria for vCJD are excluded and may or may not call for control responses, in addition to the natural interest for clinical and epidemiological research.

The epidemiological notification form and flow of encefaoopatias of samples to the laboratory are shown below. National Center for Biotechnology InformationU. Journal List Dement Neuropsychol v. Find articles by Antonio Silva Lima Neto.

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Abstract To increase the timeliness of detection of human cases of the new variant of Creutzfeldt-Jakob disease vCJD and to reduce the risk of transmission, the Brazilian Ministry of Health has established and standardized rules and control measures. Open in a separate window. Mal da vaca louca: Available at in http: Support Center Support Center. Please review our privacy policy.

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