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MEXICO IS UNDER or MIS DIAGNOSING CREUTZFELDT JAKOB DISEASE AND OTHER PRION DISEASE SOME WITH POSSIBLE nvCJD

Posted Jun 13 2012 1:32pm
MEXICO IS UNDER or MIS DIAGNOSING CREUTZFELDT JAKOB DISEASE AND OTHER PRION DISEASE SOME WITH POSSIBLE nvCJD



Can prion disease suspicion be supported earlier?


Clinical, radiological and laboratory findings in a series of cases


Alejandra González-Duarte,1,* Zaira Medina,1 Rainier Rodriguez Balaguer1 and Jesus Higuera Calleja2 1Department of Neurology and 2Neuroradiology; The Insituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán; Mexico City, Mexico Key words: Creutzfeldt-Jacob disease, prions



The subacute spongiform encephalopathies are prion diseases characterized by acute and rapid neurodegeneration that lead to the death of the patient within months to a few years. The epidemiology of CJD is complicated and the frequency in Mexico is unknown. We aim to describe the cases of prion disease in Mexico. Consecutive patients who met the diagnostic criteria by the WHO were enrolled. We describe 26 patients with clinical manifestations, imaging and laboratory studies compatible with prion disease. The mean age at onset was 52 years old. The main clinical manifestations were cognitive alterations (69%) followed by extrapyramidal movements (50%), abnormal cerebellar function (46%), behavioral alterations (46%), myoclonus (46%) and mood depression (23%), among other features. Half of the patients progressed rapidly to a state of akinetic mutism (53%). Only 2 (7.6%) patients had a family history of a similar disease. Time interval between onset and diagnosis varied between 71 days to 24 months, with a median of 6 months. The classical bilateral basal ganglia hyperintensities were present in the very early stage of the disease. Protein 14-3-3 immuneassay in the CSF was positive in all measured cases. Bilateral basal ganglia hyperintensities was the most important early finding, while protein 14-3-3 was a late finding and the results were usually obtained after the patient was discharged. Around 1.5 cases of CJD cases per year are reported in our country. When suspected, MRI can support the diagnosis earlier than other studies.


*Correspondence to: Alejandra González-Duarte; Email: gonzalezduarte@aol.com Submitted: 04/04/11; Accepted: 07/24/11 DOI: 10.4161/pri.5.3.16187



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The sporadic CJD (sCJD) form is responsible for 85–90% of CJD cases. Several hypothetical mechanism for the origin and spread of sporadic CJD have been proposed, including exposure to infected meat, or the appearance of spontaneous somatic mutations to yield an infectious protein agent de novo. On the other hand, the familial form features a dominant inheritance pattern.1,3 This form is caused by somatic mutations that occur in the prionic gene (PRNP) located on the short arm of chromosome 20 at codon 102.1 Lastly, iatrogenic transmission has also been described.





The epidemiology of CJD is very complicated. It has been recognized worldwide, at rates of 0.25 to 2 cases per million per year. In Mexico, there are only three previous reports of CJD cases3-5 among other reasons, due to limited knowledge concerning this disease on the part of the medical staff, which causes a lack of notification of cases, and an under-registration of the disease.1 On the other hand, there are no centers or laboratories of microbiology and genetics where tests to support the diagnosis of the disease can be conducted.1 This is the case of most developing countries. However, the following cases demonstrate that the clinical course is surprisingly characteristic, and the findings in the MRI and EEG can accurately support the diagnosis if the clinical suspicion is strong. We aim to provide evidence that prion disease is more common than supposed. With this, we hope to increase the awareness of the clinical features of CJD.



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Results



Seven cases of suspected CJD cases were admitted to our Institution from 1999 to 2011. Demographic and clinical characteristics are individually described and summarized on Table 1. Figure 1 shows the MRI images and Figure 2 the characteristic EEGs of these cases. In addition, we included two videos of cases 1 and 7. Table 2 summarizes our findings with other the three reports of Mexican CJD cases found in the literature. Searches were performed through Pubmed using the terms “Creutzfeld- Jakob disease,” “prion disease,” “Protein 14-3-3,” “Mexico,” “Mexican.” To our knowledge, only 26 cases of CJFD have been reported in our country.



Case 1. A 59-year old right-handed woman developed a rapidly progressive mental disease and involuntary movements. Her family history was relevant for an uncle with an unknown rapidly progressive mental disease. She was born in Pachuca, Estado de México and lived in Mexico City. She did not eat red meat regularly, nor did travel frequently, and was not exposed animals or toxins. She had been diagnosed with type-2 diabetes mellitus 8 years earlier and developed peripheral neuropathy and retinopathy. She was admitted for a 2 month history of feeling anxious, irritable and forgetful, with loss of the circadian rhythm of sleep. One month later she developed involuntary myoclonic movements of the upper extremities. Her gait became unstable and her speech dysarthric. She lost 37 pounds (17 kilos). Bilateral tremor in upper extremities emerged along with severe incoordination. She began to tumble and crash with objects. One week before her admission she had a severe fall and she was brought to the emergency department. At her arrival she was sleepy. When woken-up, her language was non-fluent and very soft. Her facial expression was lost. She was able to understand and follow orders, to name objects and repeat sentences, but she was easily distracted and her thought was slow. Her left arm was clumsy. At that time her MMSE was 26 points. Cranial nerve examination showed slow eye movements. Muscle strength was normal. Tone was generalized increased. Deep tendon reflexes were 2+ in the left side and 3+ in the right side. Plantar reflexes were normal. She had bilateral tremor that was worse in the left side. The finger-to-nose test was abnormal, as were the other coordination movements. Gait was ataxic. Frontal release signs were present. Five days after her hospitalization her MMSE declined to 11 points. Blood tests were normal, including thyroid function tests, VDRL and serology for EBV. CSF analysis showed glucose of 74 mg/dL, proteins of 52 mg/dL and no cells. PCR for herpes simplex virus, varicella zoster virus and JC virus were negative. CSF gram stain, Ziehl-Nilsen stain and the cryptococcal antigen were negative. Antineoplasic antibodies (anti-Ri and anti-Yo) in CSF were also negative. The brain MRI showed bilateral hiperintesities in caudate and putamen nucleus (Fig. 1A). Protein 14-3-3 was positive (4,200 UI) in CSF. She died one month after being discharged.



Case 2. A 60-year old man developed dementia rapidly. His mother died of a similar disease at an unknown age. He was born and lived in Poza Rica, Veracruz. He had finished elementary school and was a businessman. He denied toxic exposures or drug use. He was admitted to the hospital for a 3 month history of altered mental status, dizziness and weakness in the lower extremities. One month later he presented spontaneous involuntary movements during sleep. He was prescribed carbamazepine without improvement. Two weeks later he developed aggressive behavior that alternated with a state of perplexity and indifference to the surroundings. He also developed speech alterations and visual hallucinations. There was an involuntary weight loss of 20 kg. He was admitted to our hospital for further study. Upon admission he was awake, but he did not follow simple commands. He was disoriented in time and place. His language was soft, non-fluent and dysarthric. MMS was of 14 points. His muscle strength was decreased, worse in lower than in the upper extremities. He had a left Babinski sign. The cerebellar function was severely impaired, he had altered finger-to-nose test and impaired coordinating movements worse in the left side. He showed normal liver function tests, normal thyroid tests and negative antithyroid antibodies. Vitamin B12 levels (1,297 pg/ml) and serum calcium (9.7 mg/dL) were within normal range. HIV was negative. A brain CT only showed cortical atrophy. The EEG showed generalized spikes (Fig. 2A). In the CSF analysis cell count was negative as were the cultures and stains, but there was a marked increase in the 14-3-3 protein. He was discharged and did not have further follow-up.



Case 3. A 66-years old woman developed a rapidly dementing syndrome. She was a widower and housekeeper. She lived in Michoacán. She was exposed to wood smoke for over 20 years. She denied other toxic exposures or drug use. She had contact with hens and dogs. Her left kidney was removed for unknown reasons 20 years before, and two years ago she was diganosed with type-2 diabetes. She was admitted for headache, dizziness, slowness in thinking and speech that began one year earlier and worsened three months before addmission. At her arrival she was alert but her attention span was impaired, and she was disoriented. Her speech was fluent but incongruent. The cranial nerve examination, muscle strength and reflexes were normal. Babinski signs were absent. The sensory examination was normal. There were no abnormal movements. She had frontal release signs. The finger-nose test was abnormal in both sides, as were the rapid alternating movements. She had severe ataxia and her gait was unstable. One month later she had difficulty for making eye contact, her speech became nonfluent and dysarthric. She had eye wondering and was unable to follow simple commands. VDRL was negative, vitamin B12 serum levels were inbeteween ranges and she had normal liver and thyroid function tests. Anti-Hu antibodies were indetermiated, but anti-Yo antibodies were negative. CSF analysis showed a leucocyte count of 2 cells/mm3, and PCR for tuberculosis was negative. Her MRI showed hyperintensity of basal ganglia suggestive of CJD. Her EEG showed diffuse slow activity with periodics sharp waves. She worsened in weeks, as she was unable to follow commands and was mute and acinetic. She was discharged home.



Case 4. A male of 57 years of age came for altered mental function and involuntary movements. He lived in Morelos, finished elementary school and worked as a gardener. He had a cousin that died of a neurological disease that consisted of encephalopathy and seizures. He smoked tobacco occasionally, but had no other toxic exposures or drug use. He began 3 months before his admission with involuntary movements on his left upper arm and slow speech. He had a normal CT scan. One month later he was unable to communicate or walk. He had trouble swallowing, generalized rigidity and slowness in his movements. His coordination was worse on the left side. His laboratory studies were normal. CSF analysis was normal. The MRI showed bilateral hyperintensities in the basal ganglia (Fig. 1B). His EEG showed slow activity with triphasic waves in the right midtemporal region. He was discharged and no further follow-up.



Case 5. A 60-years old male had rapid dementia. He was married, completed elementary school and worked as a policeman. He was from Coacalco, Estado de México and lived in Mexico City. He smoked (Tobacco index of 21) and drank alcohol heavily every other week since age 22. He began two months before admission with dizziness, sleepiness and severe headache that woke him at night. He was irritable, very emotional and complained of auditory hallucinations. One month later, involuntary myoclonic movements developed on the right arm, as well as tremor in his left arm. At his arrival he showed impaired concentration and difficulties in performing mental tasks, and he had impaired short span memory. His thought was slow and his speech was dysarthric. He had severe difficulties in finding objects in front of him. His cranial nerve examination was relevant for vertical nistagmus and bilateral sensoryneural hypoacusia. Mucular tone was increased in the left side. He had spontaneous myclonic movements in the upper and lower extremities. He had ataxia and altered rapid alternating movements. While walking he tended to fall to the left side. His laboratory studies showed marked elevation of TSH and he was prescribed with levothyroxine. Liver function tests and electrolytes were normal. Vitamin B12 levels were normal (>15,000). Nor VRDL or anti-thyroid antibodies were done. He had a first EEG that showed diffuse slow activity and a second EEG performed one week later showed periodic sharp waves (Fig. 2C). His CSF analysis showed proteins of 60 mg/dL, and severe elevation of 14-3-3 protein. He died one month later.



Case 6. A 50 year-old woman developed dementia rapidly. She was divorced and worked as a secretary. She lived in Mexico City. She smoked and drank alcohol occasionally. She began one year before with muscular weakness, muscular atrophy and involuntary movements in the upper extremities. She began falling to the right side and experiencing dizziness and upper extremity rigidity. At her arrival she was inattentive, disoriented and unable to follow simple commands. Her speech was non-fluent. Cranial nerve examination was relevant for vertical nistagmus and left XI nerve paralysis. Muscle strength was 3/5 in upper extremities and 0/5 in the lower extremities. Deep tendon reflexes were 3+ in the upper and lower extremities. She had a fixed flexed posture on both arms above the torax and of both legs above the hip. There was severe spasticity. There were muscle fasciculations in the upper extremities. She was unable to perform the finger-nose test and her movements were uncoordinated on both sides. Frontal release reflexes were present. Cognitive functions declined within three months. The patient was mute and did not make eye contact with other persons. Liver function tests were normal, seric sodium was 128 mEq/L, VDRL, HIV and PPD were negative. Thyroid function was normal, as it was the vitamin B12 levels, and the antithyroid, anti-Yo and anti-borrellia antibodies. Seric heavy metal levels were normal. Her CSF showed increased proteins of 64 mg/dL with normal glucose and no cells. VHS PCR, cryptococcus antigen and black ink stain were negative. Her MRI showed hyperintensity in the basal ganglia, and the EEG showed triphasic waves (Fig. 2B). The 14-3-3 protein on the CSF was positive. She died two months later.



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Case 7. A 38-year old woman born in Guerrero came because a rapidly developing psychiatric illness associated with involuntary movements. She began 11 months earlier with abnormal behavior, easy crying and somnolence. She was very irritable and fought constantly with her husband. She was diagnosed with mood depression and started on SSRIs. She began having episodes of unexplained aggression and haloperidol and quetiapine were prescribed. Five months later she developed visual hallucination and insomnia. She presented rapid and non-rhythmic coarse involuntary movements of the lower and upper extremities. She began walking on her toes and her balance was impaired. She stumbled and fell frequently. She began presenting continuous involuntary movements that were also present at night. Soon after she was unable to walk. Ten months later she was admitted to the hospital. Her MMSS was 12/30 and her speech was dysarthric. Muscle strength was 4/5, but deep tendon reflexes and muscle tone were normal. Hoffmann and Tromner signs were present, but she did not show Babinski signs. She had severe ataxia and severe alterations on the finger-to-nose test and of the rapid alternating movements. She had myoclonus of the upper and lower limbs. Frontal release signs were not present. She had normal laboratory results including thyroid function tests and antinuclear antibodies. HIV and VDRL were negative. Heavy metal screening for aluminum, copper, arsenic, nickel, zinc and lead were negative. The CSF examination showed glucose of 58 mg/dL, proteins of 38 mg/dL and cero cells. Oligoclonal bands were negative. The MRI showed bilateral hypeintense images in the basal ganglia (Fig. 1C) with enlarged ventricles. The EEG showed slow background activity, poor gradient, poor reactivity to stimuli and sparse triphasic waves on the left frontotemporal region. Protein 14-3-3 was positive in the CSF. Akinetic mutism developed and she was discharged.



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Discussion



The occurrence and significance of spongiform encephalopathies in Mexico is unknown. Four cases were reported from 1996– 2001,4,5 and 18 more cases were detected between 2000–2005.3 We are adding seven new cases identified in our Institution from January 1999 to February 2010, counting for a total of 26 patients (Table 2). According to the World Health Organization criteria,1 six (23%) were classified as definite, ten (38%) as probable and ten (38%) possible. The overall incidence is relatively low when compared to the reports from other countries.



The mean age at onset was 52 years old, slightly younger than what it is reported in the literature,1,3,4 where mean age for sporadic CJD is 60 to 69 years old. The main clinical manifestations were cognitive alterations (69%), followed by extrapyramidal movements (50%), abnormal cerebellar function (46%), behavioral alterations (46%), myoclonus (46%) and mood depression (23%), among other features (Table 2). Half of the patients progressed rapidly to a state of akinetic mutism (53%). Only two (7.6%) patients had a family history of a similar disease. More than one third (39%) of the patients were born and lived in Mexico City, and three of the four centers that reported the cases were also in this location.



Time interval between onset and diagnosis varied between 71 days to 24 months, with a median of 6 months. According to the Slow Virus Infection Research Committee stages of the disease6 most of the patients were seen on stages I (nervousness, insomnia, headache, dizziness, mood depression or anxiety) before admission to our facilities, 19 (65%) were seen at stage II (occurrence of a distinct neurological syndrome with a prominent involvement of cortical functions and hallucinations) and only ten (38%) arrived at a later stage (severe reduced movement or complete akinetic mutism).



CSF findings were irrelevant in most cases, as only mild proteinorraquia was present in some. However, for the past decade, new diagnostic tests have shown to be extremely helpful. Albeit virtually all patients with CJD have abnormal EEG findings, most of them are non-specific at the early stages.2,11 However, the importance of performing an EEG relies on the fact that abnormalities only rarely occur in patients with rapidly progressive dementia of other etiologies such as Alzheimer disease, vascular dementia or Lewy body disease.2,11



Increased signal intensity in caudate nucleus, putamen and parietal or occipital cortical areas in fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted (DW) MRI sequences were seen in all of the cases who had an MRI (Fig. 1) and in almost half of the patients of the other series. This finding was the earliest and most consistent positive result. We think that the distinguishing features of the MRI, along with a typical clinical course, can be used as the basis of the diagnosis when 14-3-3 protein in the CSF is not available. As described before, DWI abnormalities have a higher sensitivity (92%) and specificity (93–98%), and may be detected as early as 3 weeks of symptom duration.7-9 Detection of protein 14-3-3 in the CSF has been the most promising surrogate marker for prion disease in the last years, for which a specificity of 84% and a sensitivity of 94% have been reported in reference 5, 10 and 11. Although CSF protein 14-3-3 was positive in most cases, the result often came back after the patient was discharged. Brain biopsy may be diagnostic of CJD, however, this procedure can be recommended only if some other potentially treatable disease remains to be excluded.



The patient of case 7 has several interesting findings. First, she was relatively young for sporadic (sCJD), as she was 38 years old at admission. The median age at death for sCJD is 68 years old, and for vCJD is 28 years old. She also displayed prominent psychiatric and behavioral symptoms at onset, and the overall duration of illness was 11 months. Although these features are more consistent with the variant form of the disease (vCJD) than with sCJD, in which duration of the disease is usually shorter, between 4 and 5 months and dementia and neurologic signs are present earlier or at the same time of the psychiatric manifestations, against vCJD is the presence of periodic sharp waves on the electroencephalogram and no evidence for suspecting iatrogenic or acquired disease.



No occupational activities were more prevalent and the origin of the sporadic disease could not be eluded from this study. We found two patients who had a family history of a similar disease. It is generally considered that between 10 and 15% of CJD cases have a family history of the disease.3



Our study has several deficiencies. First, we do not perform biopsies or autopsies in all of our patients. Second, PrP genotyping is available in other countries and is useful for genotyping the patients, unfortunately it is not performed in our country or similar developing countries. Even when CSF 14-3-3 protein determination may be carried out in some medical centers as special studies, it was not possible to perform it in all the patients. Lastly, we do not have the time of the duration of the disease in many cases, mostly because it was considered that a close followup in a specialized center far from their local environment would not change the clinical course and would only add the inconvenience of the trip.



Non-the less, we consider that this study reveal important information about CJD in our country. There is a tendency to underestimate the real frequency of the disease, and it is possible that it is more common than it appears, but less diagnosed due to technical and clinical deficiencies.



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Conclusions CJD is rarely recognized, however, the rapid development of signs and symptoms in addition to abnormalities in the MRI and EEG will aid the diagnosis in many cases despite not having timely access to CSF protein 14-3-3. Specially, the early identification of new onset of depression, agitation, irritability or memory loss should not be overlooked in middle age patients who do not have a previous history of a psychiatric or neurologic illness. Unfortunately, there is no treatment available, however, the recognition of more cases will allow us to understand the epidemiology and pathophysiology of this rare and devastating disease.



Disclosure of Potential Conflicts of Interest



No potential conflicts of interest were disclosed.





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Monday, May 21, 2012



Creutzfeldt-Jakob disease cluster in the health area of Meixoeiro Hospital




Conclusions The incidence of CJD in the health area of Meixoeiro Hospital is three times higher than expected. The hypothesis that at least some cases of sCJD are apparently because of covert transmission or zoonosis events should not be formally refuted and might explain the high rate found.




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IMPORTATION OF LIVE CATTLE FROM MEXICO INTO THE UNITED STATES FOR IMMEDIATE SLAUGHTER






Bovine Spongiform Encephalopathy never mentioned, and Mexico has NO idea what their BSE risk status is.



Scientific Report of the European Food Safety Authority on the Assessment of the Geographical BSE-Risk (GBR) of MEXICO



Question N° EFSA-Q-2003-083



Adopted July 2004



Summary



The European Food Safety Authority and its Scientific Expert Working Group on the Assessment of the Geographical Bovine Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission (EC) to provide an up-to-date scientific report on the GBR in Mexico, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in Mexico. This scientific report addresses the GBR of Mexico as assessed in 2004 based on data covering the period 1980-2003.



The BSE agent was probably imported into Mexico and could have reached domestic cattle. These cattle imported could have been rendered and therefore led to an internal challenge in the mid to late 1990’s. It is possible that imported meat and bone meal (MBM) into Mexico reached domestic cattle and leads to an internal challenge around 1993.



It is likely that BSE infectivity entered processing at the time of imported ‘at - risk’ MBM (1993) and at the time of slaughter of imported live ‘at - risk’ cattle (mid to late 1990s). The high level of external challenge is maintained throughout the reference period, and the system has not been made stable. Thus it is likely that BSE infectivity was recycled and propagated from approximately 1993. The risk has since grown consistently due to a maintained internal and external challenge and lack of a stable system.



EFSA concludes that the current geographical BSE risk (GBR) level is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSEagent. The GBR is likely to increase due to continued internal and external challenge, coupled with a very unstable system.







Key words: BSE, geographical risk assessment, GBR, Mexico, third countries




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Annex to the EFSA Scientific Report (2004) 4, 1-13 on the Assessment of the Geographical BSE Risk of Mexico



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2.3 Overall assessment of the external challenge


The level of the external challenge that has to be met by the BSE/cattle system is estimated according to the guidance given by the SSC in its final opinion on the GBR of July 2000 (as updated in January 2002).


Live cattle imports:


According to the CD the country imported in total over the period 1980 to 2003, approximately 3.2 million live cattle from BSE - risk countries, of which conclusively none came from the UK. The numbers shown in table 1 are the raw import figures and are not reflecting the adjusted imports for the assessment of the external challenge. Broken down to 5 - years periods the resulting external challenge is as given in table 3. This assessment takes into account the evidence that certain imported cattle did not enter the domestic BSE/cattle system, i.e. were not rendered into feed. In the case of Mexico, it is assumed that “cattle still alive” (imports from Spain) did not enter the rendering system.


MBM imports:


According to the CD the country imported in total over the period 1980 - 2003 approximately 826,000 tons MBM from BSE - risk countries (according to “other data”: ~ 919,000 tons), of which none came from the UK. The numbers shown in table 2 are the raw import figures and are not reflecting the adjusted imports for the assessment of the external challenge. Broken down to 5 - years periods the resulting external challenge is as given in table 3. This assessment takes into account the evidence that certain imported MBM did not enter the domestic BSE/cattle system or did not represent an external challenge for other reasons. However, in the case of Mexico, there was not sufficient evidence to remove any quantities of MBM from the external challenge.




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Annex to the EFSA Scientific Report (2004) 4, 1-13 on the Assessment of the Geographical BSE Risk of Mexico


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would harbour, while being pre - clinical, as much infectivity as a clinical BSE case. Hence cattle imports could have led to an internal challenge about 3 years after the import of breeding cattle (that are normally imported at 20 - 24 months of age) that could have been infected prior to import. In case of Mexico this implies that an internal challenge caused by live cattle imports (predominantly from USA or Canada) first occurred in the mid to late 1990’s and continued to the present.


On the other hand imports of contaminated MBM would lead to an internal challenge in the year of import, if fed to cattle. The feeding system is of utmost importance in this context. If it could be excluded that imported, potentially contaminated feed stuffs reached cattle, such imports might not lead to an internal challenge at all. In case of Mexico this implies that an internal challenge caused by MBM imports (predominantly from USA or Canada) first occurred around 1993 and continued to the present.


In view of the above - described consideration the combination of the very / extremely high external challenges with a very unstable system makes the occurrence of an internal challenge likely in Mexico from approximately 1993 onwards.


4.2 Risk that BSE infectivity entered processing


It is likely that BSE infectivity entered processing at the time of imported ‘at - risk’ MBM (1993) and at the time of slaughter of imported live ‘at - risk’ cattle (mid to late 1990’s). The high level of external challenge is maintained throughout the reference period, and the system has not been made stable, leading to increased internal challenge.


4.3 Risk that BSE infectivity was recycled and propagated


It is likely that BSE infectivity was recycled and propagated from approximately 1993. The risk has since grown consistently due to a maintained internal and external challenge and lack of a stable system.


5. CONCLUSION ON THE GEOGRAPHICAL BSE – RISK


5.1 The current GBR as function of the past stability and challenge


The current geographical BSE risk (GBR) level is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent.


5.2 The expected development of the GBR as a function of the past and present stability and challenge


• The GBR is likely to increase due to continued internal and external challenge, coupled with a very unstable system.



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The most recent assessments (and reassessments) were published in June 2005 (Table I; 18), and included the categorisation of Canada, the USA, and Mexico as GBR III. Although only Canada and the USA have reported cases, the historically open system of trade in North America suggests that it is likely that BSE is present also in Mexico.







Subject: Mexico SAGARPA Assessment of BSE VS EFSA Scientific Report on the Assessment of the Geographical BSE-Risk (GBR) of Mexico


Date: February 5, 2007 at 1:11 pm PST


Empresa solicitante: SAGARPA


Tipo del análisis efectuado: Cuantitativo


Temática: “Análisis de riesgo sobre la ocurrencia de la encefalopatía espongiforme bovina en México”


INTRODUCTION:


The bovine spongiform encephalopathy (BSE), it is a neurological disease, invariably fatal and with long period of incubation, that affects cattle. Its etiologic agent is the prion. General consensus exists with respect to that the feeding of contaminated meat and bone flours, it is the most significant source in the dissemination and transmission of this etiologic agent. At this time there is no exist evidence that BSE is transmitted by means of embryos, their semen and in case of existing maternal transmission, if this could happened it would be in a so extremely low rate that it could not be considered like a trigger or leading factor of an epidemic. Controversy in respect to other probable ways of transmission remains. The BSE was diagnosed for the first time in 1986 in the United Kingdom. At this time it exists in 26 countries, including a Canada and the United States of North America (USA).


This document summarizes the analyzed elements and the results of the study of the evaluation of the risk factors, of the epidemiology surveillance and related activities, as well as the quantitative estimation of the risk with respect to the probability of introduction of the disease to the Mexican herd.


EVALUATED ELEMENTS:


Demography and characteristics of the Mexican cattle industry: Cattle is one of the main activities in the Mexican farming sector, due to its contribution in the supply of meat (beef) products, dairy, among others; as well as its participation in the international trade on cattle exports, mainly to the United States of North America.


According to data of the 2001, cattle population is of 30.620.933 of heads, of which 28.480.803 are beef cattle and 2.140.130 dairy cattle. The main cattle production states are located in the center-north, where its operation is intensive and its feeding is based on grains; as well as in the coast of the Gulf of Mexico and the south-southeastern, with intensive programs and feeding is based mainly on the pasturing (grass). The national dairy herd, is calculated as specialized or technified that represent 17,44% of the herd, semi-specialized 14,90% of the herd, double-purpose herd (beef and dairy) 59,68% and the small family-run herd or the referred as “backyard” (traspatio) 7,98%.Previous numbers are to be considered as an estimation of the dairy livestock inventory by production units. Nevertheless, it is necessary to consider that all races of pure breed can be found in anyone of those groups.


Legal grounds: Mexico counts on a normative frame that covers (deals with) the relevant aspects of the Epidemiology Surveillance of the BSE, like the Federal Law of Animal Health, the Federal Law of Metrology and Regulation, the General Law of Health and several Mexican Official Norms (NOM-009-Z00-1994, Sanitary process of the meat, NOM-030-ZOO-1995, Specifications and procedures for the import of beef, carcasses, viscera and offal at zoo-sanitary inspection points, NOM-061-ZOO-1999, Zoo-sanitary specifications of nutritional products destined for animal feed and NOM-060-ZOO-1999, Zoo-sanitary specifications for the transformation of animals offal and its use in animal feeding). Wider and extended covertures of these regulations were evaluated.


Veterinary infrastructure: The veterinary services in the country are structural and normative organized by the Mexican State through the Secretariat of Agriculture, Livestock, Rural Affairs, Fishery and Alimentary (SAGARPA) Federally Empower, that is to say, that has the capacity and authority to negotiate an to come to agreement with the States Governments that integrate the Republic; to coordinate itself with the other Secretariats of State; to deal with organizations of the Private and Social sector as well as with the rest of the Civil Society as a whole.


The National Service of Health, Food Safety and Ag-alimentary Quality (SENASICA), it is an organism of this Secretariat, which has attributions in the matter of vegetable health, animal health and ag-alimentary safety and is conformed by the following main directorates: Sanidad Vegetal, Salud Animal, Inocuidad Agroalimentaria, Acuícola y Pesquera, Inspección Fitozoosanitaria, (equivalent to U.S. APHIS, FSIS and VS –Veterinary Services) Jurídica, Administración e Informática. In accordance with the assigned attributions, it corresponds to central offices the substantive part and the operative part, to the personnel assigned to the State Delegations of the SAGARPA and other instances of the SENASICA.


Consequently, the four main areas are assigned to the Main directorate of Salud Animal-Animal Health (DGSA) and to the Main directorate of Inspección Fotozoosanitaria-Plant Inspection (DGIF) and Veterinary Services (SV) in Mexico are in charge of: surveillance, epidemiology, animal movement, zoo-sanitary campaigns and emergencies.


Imports This is perhaps one of the medullar points, in the sense that it represents the information of the imports made during the risk periods and therefore, it provides the fundamental information for the risk assessment. In 1991, Mexico implemented measures to avoid the appearance of BSE, as the disease had become a serious worldwide problem, reason why, live bovines imports were prohibited, beef, beef products and by-products and in 1994 flour of meat and bone from countries affected by this disease was also prohibited and in the 2000 MBM feeding ban was imposed. In order to mitigate the risk of transmission of the EEB, a revision of the established requirements for import for ruminants’ products began.


Cattle imports and its products and by-products, as well as specific risk materials played a very important role in this study, where considerable amounts of cattle imports from countries now affected by BSE were identified, countries that at the time of the import they remained clean and therefore just some preventive risk measures were in place.


Slaughter, Cattle disposition and Offal.- Different cattle slaughter schemes were analyzed as well as the processes in use, finding some significant differences among them, being the most important the sanitary jurisdiction of the organizations that regulate us.


In Mexico, the slaughter is divided in three different systems, Federal Inspection Type Plants (TIF), which has been increased in the past years; in 1992 they participated with the 13,5%, in 1997 with the 19,40% and in 2002 with the 26,60% of the national total. In the case of the municipal slaughterhouses from 1992 to 1997, their slaughtered animals corresponded to the 49,5% and for 2002 it was increased to 73,4%, whereas the slaughter in private plants decreased of 37,10% in 1992 to 31,10%, in 1997 and from 1998 to date, we have no information.


The procedures to be followed by the establishments in the animal slaughter and those that industrialize, process, packing, chilled/froze beef products or by-products for human consumption, in order to obtain products of optimal hygienic quality, are written in the NOM-009-ZOO-1994 “sanitary Process of Beef”.


The direct consumption of beef can be stratified in three great destinies, differentiated by the market that are destined to, the rural one, the one of small centers of population, (and) the one of the big cities, characterized each one of them by its consumption and the partial or integral industrialization by direct consumer and by means of commercialization or points of sale, as well as for the origin of the own supplier.(?)


Rendering of Cattle Products.


The processes applied by the rendering plants for obtaining the protein from inedible offal, were evaluated.


Food elaboration and its use for animal feeding.- This analysis was focused in the processes of food elaboration for animal consumption.


In Mexico, the control in the production of food from animal origin, as much as the elaboration of the meat flour as that of the balanced food manufacture it is regulated by the Mexican Official Norm NOM-061-ZOO-1999, “Zoo-sanitary Specifications of nutritional products for animal consumption”, which bans the use of MBM flours of ruminant origin or any mixture that contains it for the elaboration of balanced meals for ruminants, and the Mexican Official Norm NOM-060-ZOO-1999, “Zoo-sanitary Specifications for the transformation of animal offal and its use in the animal feeding”.


In accordance with the Section of Manufacturers of Balanced Food for Animals of the National Camera of the Industry of Transformation (CANACINTRA), there are 396 balanced food plants registered, same that have the capacity to produce more than 20 million tons a year, according to the numbers registered during 1999-2002. 63% of such plants are integrated and produce 64% of the animal feed produced nationwide, the rest corresponds to commercial plants.


The animal feed produced by the integrated plants, that is the most significant part, during the 2002 it produced the 58,7% of the products destined for raising of poultry, the 16,5% for swine, the 14,3% for dairy and 9,2% for feedlots (cattle) and 1,3% for other species.


As far as the composition of the main raw materials to produce balanced foods, these mainly correspond in 45% to domestic sorghum and 55% sorghum concentrated; 16% to domestic yellow maize and 84% imported; 91% domestic protein pastes and 9% imported; 80% of other domestic forage grains (broken maize, wheat, barley, oats, etc.) and 20% imported and other ingredients (wheat by-products, maize, vitamins, minerals, oils, etc.).


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Neuropathies in Mexico, Epidemiology Surveillance Program.- For this analysis, the legal elements related to the notification of the BSE were taken into account, in Mexico, as well as the activities made by the Commission Mexico - United States for the Prevention of the Aftosa Fever and Other Exotic Diseases of Animals (CPA), official entity in charge of carrying out this activity and other connected activities as training, taking of samples and the diagnosis of laboratory.


BSE Diagnosis.


Veterinary Services diagnoses capacity was evaluated as well as its adherence to the international standards, according to what is indicated by the International Organization of Animal Health (OIE), as well as the processes of taking and shipping of samples.


For the diagnosis of the BSE, the OIE recommends five laboratory tests: Histopathology (HP), Immunohistochemistry (IHQ), Western blot (immunotransferency), ELISA (enzimoinmunoassay) and Bio-assay in mouse. At this time Mexico counts with two laboratories of diagnosis for this disease: the National Center of Services of Diagnosis in Animal Health (CENASA) and the Laboratory of high security of the CPA. The CENASA performs the histopathology test and at the CPA the Immunohistochemistry test is carried out.


The reception of samples at CPA, it depends to a great extent on the economic resources which are accounted for this activity, expense that is approximately of $400,00 ($ 36.50 USD.) per sample received (includes the material for conservation, packing and shipping), reason for what, have to wait for the collection of several to be sent at the same time and in order to reduce costs, but delaying the result. As the CPA does not have a certified pathologist to carry-out the HP test technique, these samples are sent to the CENASA for their diagnosis; this implies that such samples are stored by approximately one week, since it doesn’t have the human resources for its transfer.


The main problem at CENASA, for the right operation of the diagnosis of the BSE, it is the lack of coordination on shipping and receiving of samples, which is not done accordingly to the calendar of the laboratory and the operative area, because in a short period of time the expected/projected number of samples is exceeded, resulting in delays in accomplishment of the tests and the disposition in excess of material and human resources.


At this moment, the techniques are being standardized, Immunohistochemistry at the CENASA and the western blot (immunmotransferency) at the CPA, which will allow us to have more tools for the diagnose in Mexico; in addition, the WB allow us to count on another technique of the higher sensitivity and specificity, that guarantees optimal result in less time (approximately 8 hours).


It must be mentioned that, we have had contemplated the formation of a network of laboratories of diagnosis of TSE´s to specialized on the HP technique, where we will have 6 regional laboratories and 4 universities involved, this will in the future allow the processing and diagnosis of the sample from its place of origin and only its confirmation by other techniques at central level. For this, we already count with the procedure for the authorization and verification of a laboratory of histopathology for the diagnosis of the BSE.


Monetary Compensation to cattle dealers: Because the BSE is considered as an exotic disease, a contingency fund that could be put to work in case the disease appears, does not exist at this time.


In the case of the contingency funds, the national campaign for diseases relies on a section on this subject. Nevertheless, for the exotic diseases official norms do not exist and article 36 of the Federal Law of Animal Health only establishes that will be due to create, but it does not explain the mechanism to be considered for its creation.


The pre-established form to compensate the possible producers that are themselves affected by the presence of BSE in their cattle, it will be from the federal budget that is agreed upon the program Alliance for the Country for the corresponding fiscal year.


In this sense, it is necessary to pinpoint that the minimum amount to consider for this budget will be a 4% of the total assigned to the Fito-zoo-sanitary Contingencies Plan on behalf of the Federal Government.


This will have a distribution by federal entity, which a specific amount will be able to be assigned to joint, if necessary, to the DINESA against the BSE. Also, the State Governments will proportionally contribute an equal amount to the federal to be incorporated to the compensation funds of the Device of Emergency. As for the cattlemen, they will have to come-up with resources equivalent to the third part of the total amount assigned by the Federal and State governments.


Animal identification and traceability of cattle products.- Different elements were considered with which Mexico counts on to carry out the traceability of animals and its products upon a sanitary problem, including the animal identification and the organizations related to this activity.


Actually, the identification system of the cattle in Mexico is organized in two forms, one State-ID with aims of demonstration of property and control of cattle rustling and another Federal-ID, with aims of identification for the development of the zoo-sanitary campaigns against the bovine tuberculosis and brucellosis, first it is based on the registry and recognition of the Hot-Brands of each producer, and the second in addition to the previous system, one is based on a metallic earring of blue color with a number of identification, which is described in the NOM-031-ZOO-1995, Campaña Nacional Contra la Tuberculosis Bovina (Mycobacterium bovis), National Campaign Against Bovine Tuberculosis.


This procedure assigns a number to an animal, which is used during zoo-sanitary surveillance campaigns, these activities are registered along with an identification number, in a document called test-opinion, in which it is written down, in addition to the test results applied to the animals, the identification and data of the cattle herd and ranch of origin of the animal for its later traceability. This test-opinion is along with the certificate of “Herd free of bovine tuberculosis” as described in the same Mexican Official Norm.


According to the procedure previously described, in Mexico, there were around 3.291 registered herds with 282.932 heads of bovines identified in 2003, that represents 0,94% of the total population in this country.


Nevertheless, in the same NOM, it’s expressed in point 11 referring to mobilization that the animals coming from disease-free herds, they will be able to be mobilized to any destination within the national territory with no need to be tested for tuberculosis before its mobilization, if the following requirements are met: obtain a zoo-sanitary certificate, and for the zoo-sanitary certificate to be issued, certification that they come from a disease-free herd and that the animals must have a disease-free herd identification.


Considering that in order to mobilize the animals it is necessary to have a valid disease-free zoo-sanitary certificate, we can estimate that there are more than 3,431,022 identified animals, according to the information obtained from the Statistical Report of the Cattle Mobilization of FY2000, with information captured up to the 24 of August of 2001 by the National Organism of Herd Certification, A.C., that represents the 11,4% of the bovine total population on which we can observe that more than 50% of these mobilizations are directed to slaughterhouses, 17% to feedlots, 15% for export and 11% for pasturing.


Based on the above, experience of a suitable animal traceability is shown specially in the case of the animals destined to be exported, where the USDA when finding a positive animal reactor to the tests of tuberculosis in the United States, it has been possible to trace it back to its the original herd; on the other hand, the identification system used on dairy cattle, which counts on a homogenous system of identification for production and genetic improvement control, nevertheless, this mechanism although is available for the federal government, it would make use of, only in the presence of a serious epidemiology event.


Educational Programs, Awareness and Training.- The CPA, one of its activities, is to maintain a permanent program of training courses on exotic diseases of the animals, on a national context. In 1994, BSE awareness programs were incorporated , with the diffusion of information, talks and courses on the following areas: disease history, economic consequences, etiology, transmission mechanisms, clinical signage, histopathology injuries, differential diagnosis, measures of prevention and activities of epidemiologist surveillance, supported by audio-visual means, these programs are taken to a diverse audience, including the students of the last semesters of Veterinary Medicine, to the personnel that conforms the Quarantine National System, as well as Veterinary Doctors, government, private and to other specialists.


ESTIMATION OF RISK (Risk Assessment)


According to the qualitative estimation in this assessment, it was determined that the risk of occurrence of the disease in the bovine population, is low.


The quantitative estimation index was located at 5.268908E 08 of the risk of disease exposure of the national herd, number that represents numerically like a low probability of occurrence of the problem in Mexico.


CONCLUSIONS AND RECOMMENDATIONS


Conclusions


The BSE is a disease that was described for the first time in 1986, nevertheless, today, epidemiologists have many unanswered questions on how is transmitted.


The introduction of the BSE in Mexico would cause a serious socioeconomic impact, commercial, political and probably of public health concern, because the presence of the disease would restrict sanitarily and commercially, disrupting the actual distribution of meat products at national level and to other countries, independently of the impact in the consumption of the inhabitants with respect to the beef consumption and products of bovine origin.


Considering the way of transmission, in case of a breakout, the native animals that are at greater risk of being infected in Mexico, those are the dairy cattle in specialized systems and the bovines at feedlots in the arid and tropical regions.


In Mexico, we got Laws, Mexican Official Norms and Agreements, that cover relevant aspects of the epidemiology surveillance of the BSE, same that must be fortified in its operative phase, mainly in its application and enforcement.


The Mexican Official Norm NOM-030-ZOO-1995, Specifications and procedures for the import of beef, carcasses, viscera and offal at zoo-sanitary inspection points, prohibits the import of cattle products, however, fresh beef has been imported, chilled, frozen and beef preparations, as long as it comes from animals smaller of thirty months of age, which diminishes the risk but does not exclude it.


The evaluation showed that the four great areas of concern are assigned to the Main Directorate of Animal Health (DGSA) and to the Main Directorate of Fito-zoo-sanitary Inspection (DGIF); responsibility of the Veterinary Services in Mexico, in relation to the BSE are: epidemiology surveillance, animal movement control, zoo-sanitary campaigns and emergencies; functionality and capability of communicating among them was evaluated as we as the capacity of response before a sanitary emergency caused by the BSE.


It is necessary to increase and to better coordination of the surveillance activities, particularity between the areas of diagnoses and operational, for the correct execution of the surveillance activities in the field.


The imported bovines (1996-2003) have been slaughtered and those destined to improve genetics, once they conclude their productive life and are discarded, will also be slaughtered.


The actions of detection of downer-cows need to be reinforced for its processing at TIF plants, till now a deficient activity, where the majority of the animals with such clinical characteristics, regularly are not taken this plants but rather are slaughtered at same ranch/location and consumed regionally or they are taken to slaughterhouses without supervision and sanitary inspection.


Ante mortem inspections need to be reinforced at Federal Inspection Type Plants, municipal and private slaughterhouses, mainly in these two last ones, with the purpose of detecting bovines clinically affected by BSE.


There is commercial interest to incorporate flours of meat and bone of ruminants in the rations destined to the feeding of the bovines, like an alternative source of protein matter, reason why official mechanisms must be reinforced in preventing this type of illegal practices.


One of the tools in preventing the BSE is to avoid the exposure of the native bovines to the consumption of presumably contaminated feed with the pathological agent or unless is processed by means of a thermal process that guarantees its inactivation. However, the heat treatment that the flours of meat and bone are put under in XXXXXX XXXXX XXXXX XXXXXX XXXXXX XXXXXX XXXXXX XXXXX XXXXX XXXX XXXXX XXXXXX XXXXX XXXXX XXXXXX XXXXX XXXXX XXXX during 20 minutes), even though this one, it does not guarantee the destruction of the prion either, but it reduces its infectivity significantly.


The Country counts on regulations, in respect to the transformation of offal (NOM-061-ZOO-1999), there still are deficiencies as to the number and qualification of the personnel responsible in supervising their fulfillment through inspection and verification.


Deficiencies in the availability of technical information at official and private levels were detected, crucial information necessary for the elaboration of the present assessment, such as the case of the information on product imports and on rendering plants, were not available for the study.


Blood was not considered as a potential source of transmission of the BSE, by-product in form of flour (dry blood), that is also produced by the rendering plants and is used in animals feeds.


The BSE epidemiology surveillance program in Mexico must be reinforced by focusing on a target animal study (bovine suspected of BSE and with suggestive clinical signs of the disease). On the other hand, as a result from this study, we found that a percentage of the obtained samples for BSE testing have been inadequately collected and among other causes were: absence of cerebral stem, incomplete cerebral stem, over-manipulated samples, advanced changes (decompose) postmortem, not enough tissue to work on, low concentration of conservative (solution) or samples taken from inadequate age of animal (too young); showing all of these, a necessity to review these procedures.


It was also detected the fact that, as a routine practice the samples sent for the diagnosis of bovine rabies, whenever they come out positive to this disease, they are no longer processed for the BSE testing, discarding with this, the possibility of finding both diseases in a same animal, rabies virus and the BSE prion. It is also concluded that with the loss of diagnosis material, it prevented us from obtaining valuable epidemiology information useful in restructuring our surveillance program.


The identification of the cattle, as well as the traceability of its products and by-products, presents serious deficiencies at national level, which is important in case the BSE is detected in the Country, given its importance like a primordial component to trace, to prevent and to eradicate this and other animal diseases, turning out to be an additional vital tool to determine the dissemination degree in case of break-out in the country, that would immediately allow us to be able to establish its origin (native or imported) and to take the appropriate counter-epidemic measures.


From 1994, the Commission Mexico - United States for the Prevention of Foot-and-Mouth Disease (FMD) and other Exotic Diseases (CPA), it has carried out activities of awareness and training on BSE, however, this has been centered to certain zones of the country, leaving some other zones, particularly the rural zones without cover, same that can provide with valuable epidemiology information and some cases for diagnosis of neuropathy in ruminants.


According to the analysis made on the risk assessment in its qualitative modality, it is considered like low-risk, the risk of introduction of the BSE to the national herd, whereas the quantitative study locates it in values of 5.268908E-08.


Recommendations:


We ought:


to reinforce the inspection and supervision activities by the sanitary authority of the SAGARPA over all of those involved in the cattle production chain, in respect to the fulfillment and application of the established technical regulations expressed on the official norms on the monitoring of BSE, specially the NOM-060-ZOO-1999, Zoo-sanitary specifications for the transformation of animals offal and its use in animal feeding and the NOM-061-ZOO-1999, Zoo-sanitary specifications of nutritional products for animal consumption;


to increase the number of inspectors (Vet Doctors) as much as governmental as private, with a vision of having a better supervision of the rendering plants and feed factories. It is recommendable that such inspectors have a veterinary doctor’s degree.


to reinforce the active epidemiology surveillance subsystem, having special attention to aim at target animals and the size of the statistical test, as well as its stratification at national level;


to review, to update and to homologate the criteria and definitions of the Mexican official norms related to the monitoring of the BSE and the requirements of import, according to norms NOM-008, NOM-030 and NOM-060;


to provide technical and legal elements in the official norms, that may allow to optimize the use of financial and human resources (federal, state and private), with the purpose of that the material and human infrastructure, the installed diagnoses and the potential, can be used with greater efficiency, in the prevention activities, diagnosis and surveillance of the BSE in Mexico;


to homologate the mechanism of training in the obtaining of the samples for the BSE, using the technique of the teaspoon, by means of a national program;


to have a certified pathologist for the high security laboratory of the CPA, because this situation of not having one, causes the delay in the processing of samples, as well as the loss of economic resources by requiring the support of the CENASA;


to plan the taking of samples at a national level and to coordinate its shipment to the CPA for its processing in the laboratory of high security or its re-expedition to the CENASA, with the purpose of optimizing the diagnosis;


to obtain funds and allocate them at each state, in order to compensate cattle dealers affected by the animal culling at risk by BSE, in case of BSE showing up in Mexico, the same or similar mechanism are to established for the handling of monetary compensation, like the one used on the Alliance for the Country or to extend the already existing state government faculties, by means of an exclusive and specific account for the implementation of BSE comp payment.


to implement a national animal identification and traceability system, its products and by-products, that it may allow us to apply prevention measures and control of diseases, as it would be the case of the BSE.


With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked:


Justification of the blockade (p. 6, paragraph 1):


Gallinaza and pollinaza- feather meal (hay bed or substrate on which birds grow up, constituted by rice husk, straw or another type of hay, agriculturist, that at the end of the raising cycle of young hens or chicken, contains the feces of the animals that were bred on it, as well as rest of non-consumed food by the birds), it has been considered in multiple occasions, like an element of potential risk in the transmission of the bovine spongiform encephalopathy (BSE), when it is used to feed ruminants. The risk is generated, as it is common, the bird feed, contains flours of meat and bone of ruminant like source of protein. In this way, in theory, if some of the bovines with which the meat and bone flour was prepared as bird feed were infected with the BSE prion and given the high resistance of the agent (prion) to high temperatures, in the industrial process as the making of the flour, like the making of the nutritional concentrated feed for birds, and even the passage by digestive-tract of the bird, it would not guarantee the destruction of the BSE prion, reason why the possibility would exist, when gallinaza or pollinaza is used in the feeding of ruminants, this could infect susceptible ruminants.


With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked:


Justification of the blockade (p. 6, paragraph 2):


As much gallinaza as pollinaza, they can contain up to a 3% of wasted food, independently of bird feces that could also contain the prion, all implying that the flours of meat and bone of bovine origin, can be consumed by other bovines and by doing this, constituting a possible situation of BSE risk.


Norma NOM-060-ZOO-1999 Zoo-sanitary specifications for the transformation of animal’s offal and its use in animal feeding and the NOM-061-ZOO-1999 Zoo-sanitary Specifications of nutritional products for animal consumption, they clearly indicate the prohibition to feed ruminants with flours of meat and bone of ruminant origin, however, the prohibition to feed ruminants with gallinaza or pollinaza, is not contemplated in these norms.


With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked:


Justification of the blockade (p. 6, paragraph 3):


Other elements to consider are the production cycles of the farms of birds in Mexico, a common practice is that when a cycle is reached (ended) “all inside, all outside”, and the pollinaza and gallinaza are destined to feed the cattle. Depending on the type and the characteristics of the bed, it is possible to calculate an approximated weight of 13,9 kg. by square meter of bird farm surface.


With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked:


Justification of the blockade (p. 6, paragraph 4):


In the Mexican market, two types of products are accepted: pollinaza and gallinaza, which has been consolidated as a production system, considering that near 90% of the feces are used as ruminant’s feed, with prices reaching near those of cereal grains, the rest is used in agriculture.


With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked:


Justification of the blockade (p. 6, paragraph 5):


The use of the animal feces like source of high nutrients supply, it obeys mainly to its high content of mineral matter and non-protein nitrogen. In general, nitrogen is concentrated in greater amount in bird feces. What is doubtless, it is that the feces are raw material available all the year long for animal feeding, especially bovines.


The FAO (1980) made a description of the physical composition of pollinaza as it is detailed next:


Feces 62%


Bed 31%


Wasted Feed 3%


Feathers 2%


Unknown ingredients related to fresh matter 2%


Source: The FAO. Feed from Wastes Animal: State of knowledge, Production animal and Health, to paper 18. Rome, Italy 1980.


Conclusion:


Making public the information that has been eliminated of the report, it would open the door for those in the grain business to use it for their benefit and by pressing the government/the authority to establish a NOM banning such products as ruminant feed. This would bring/cause an important alteration in the commercialization of these products nationwide, which in turn would remarkably increase the production costs of the cattle in feed lots. Today, we foresee escalating grains prices at medium term, originated by its use in the ethanol production; this would aggravate the situation and force a NOM as described before, which in addition, if our sanitary status with respect to the BSE is considered low, it would be obviously excessive cost and highly harmful for the producer of birds and cattle. It is why, that it was decided to block the reference information.


With foundation in Article 14, fraction VI, of the Federal Law of Transparency and Access to the Governmental Public Information, the following paragraphs have been blocked:


Justification of the blockade (p. 12, paragraph 3):


During the period between 1996 to 2003 years in which, considering the long period of incubation of the BSE, the disease was already present in the United States of America and Canada, Mexico as usual, imported considerable amounts of calves destined for dairy production. In the same term “bullfight” bulls from Spain were imported once Europe reached a free status from FMD, same that allowed the import of some cattle for reproduction from other European countries, with exception of the United Kingdom and Ireland, countries in which BSE already existed.


In all the cases these imports were immediately stopped even before the confirmation of BSE in those countries, nevertheless, as already indicated, the ample period of incubation of the disease, those imports are looked as of certain risk, even though in that moment they were not.


The nonexistence of a national animal identification and traceability system at that time made it impossible to establish the destiny of most of those animals and to even know if they have been eliminated at the end of its productive life. It is possible to indicate that the recent imports of heifers coming from the United States of America and in the near future from Canada, new requirements and actions that guarantee their traceability and other measures to mitigate the BSE risk, are in place.


Even though during the administration of the Lic. Vicente Fox, the SAGARPA made a concerted effort to establish the National System of Individual Cattle Identification (SINIIGA), the magnitude, cost and coverage of the project, its conclusion in the short and medium term are way far distant, what implies that it will be long time before Mexico can count on a suitable (working) national system of identification and traceability of animals and products of origin animal.


The blockade of the above paragraph obeys to the convenience of not exposing to the Federal Government to unnecessarily critics that even though funded, it would not contribute to the solution of a problem that, although is of urgent attention, by its magnitude and cost, it exceeds in much, the present capacities as much of the Government, like of the National Cattlemen Sector. The critic would sustain in that what it is said in such paragraph is purely speculative, without possibility of corroborating it documentarily.


END...



Hola Amigo Terry,


Finally, here is a translation - if you can call it that - i'm not happy with it but guess that some paragraphs are very literally translated (poorest job i have ever done translating a document), please read it and if something is not clear enough or not right just let me know it and i'll correct it...


If you don't find anything of importance; if it is to vague and shows that they have done nothing about it; if somehow it gives you the impresion that they don't know a thing and are trying to cover their butts in a very stupid way;...yea! you got the right impression!!


All they are saying it's a "mea culpa" and we ought to do this and that; we don't know how they came in or where they are; we are looking into it; we screwed up all the BSE testing and we don't know how to do it right; it is too costly and we don't have the money; we didn't do it, past administration did it; we are trying to fix it; etc.,


All of the above and more, but we are following OIE rules, we have NO BSE anywhere and risk is extremely low or null, but CATTLEMEN WIL BE COMPENSATED!!


Conclusion- they are a bunch of murderers and me a national security threat for having them to admit it!! .....Oh my Lord!


snip...end (tss)


Have a wonderful weekend and our best regards,


xxxxxxxxxxxxxxxxxxxxxxx



=======================================





BSE MAD COW IN MEXICO ??








El proyecto más grande de la Fundación CJD es su proyecto continuo con su Línea de Ayuda gratuita (1800-659-1991). La línea de ayuda está disponible para familias que están lidiando con diagnosis sospechado de CJD de sus personas amadas y necesitan apoyo e información, y para personas que tienen preguntas referentes a la enfermedad de Prion como son: médicos, profesionales, directores de funerales, embalsamadotes, público en general, prensa y medios de comunicación.



En la parte posterior, podrá encontrar las estadísticas de la Línea de Ayuda de Enero 1, 2010 – Febrero 28, 2010.



La Fundación de CJD no es una agencia de reporte y no se requiere que las familias nos reporten la enfermedad o muerte de sus seres queridos La intención de estas estadísticas no es de naturaleza científica, sino por lo contrario, son para ayudar a validar el trabajo que realizamos diariamente.



2010 NUEVOS CASOS REPORTADOS TOTAL DE MUERTOS REPORTADOS EN EUA


LINE DE AYUDA


(LLAMADAS DE FAMILIA)


LINE DE AYUDA


(E-MAILS DE FAMILIA)


SITIOWEB VISITANTES


UNICOS


Enero 28 19 62 52 3,996


Febrero 23 20 75 29 4,313


Total = 51 39 137 81 8,309




Nota: No todos los nuevos casos y muertes reportados son confirmados por autopsia.




Ganadores de Fondos de la Fundación CJD 2009









ATTEMPTED CJD SURVEILLANCE MEXICO


105 MILLION


4 BASIC


WORKER


NON WORKER


GOV


PRIVATE


PROB


PATIENT SURVEILLANCE AND HEALTH INSTITUTIONS TO COOPERATE.


VERY DIFFICULT TO GO INTO NEUROLOGY


PRIONS IN MEXICO LAST YEAR


REVIEW DEATH CERTIFICATES GUADALOHARO NO MENTION OF CJD IN THE LAST 12 YEARS...2 CASES...SEARCH THROUGH DEATH CERTIFICATES AT HIS HOSPITAL. DATA NOT COMPLETE TO CALCULATE PREVALENCE. MOST PATIENT BY INTERNET, FEW BY NEUROLOGIST. OFFERS TO NEUROLOGIST TO GET INFORMATION FROM USA PRION UNIT.






no prion studies by this institution or surveillance being done by this institution.











BASICALLY, Mexico has no CJD surveillance program, as with the BSE surveillance policy. ...








Geographical BSE risk assessment and its impact on disease detection and dissemination


Original Research Article


Preventive Veterinary Medicine, Available online 1 February 2012,


Mo Salman, Vittorio Silano, Dagmar Heim, Joachim Kreysa


Preventive Veterinary Medicine


1 February 2012


Geographical BSE risk assessment and its impact on disease detection and dissemination


Salman M, Silano V, Heim D, Kreysa J.


Source


Campus Stop 1644, Animal Population Health Institute, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO 80523-1644, USA.


Abstract


Bovine Spongiform Encephalopathy (BSE) rapidly evolved into an issue of major public concern particularly when, in 1996, evidence was provided that this disease had crossed the species barrier and infected humans in the UK with what has become known as "variant Creutzfeldt Jakob Disease" (vCJD). The aim of this paper is to describe the European Geographical BSE risk assessment (GBR) that was successfully used for assessing the qualitative likelihood that BSE could be present in a country where it was not yet officially recognized. It also discusses how this can lead to risk-based and therefore preventive management of BSE at national and international levels. The basic assumption of the GBR method is that the BSE agent is initially introduced into a country's domestic cattle production system through the importation of contaminated feedstuffs or live cattle. This is referred to as an "external challenge". The ability of the system to cope with such a challenge is, in turn, referred to as its "stability": a stable system will not allow the BSE agent to propagate and amplify following its introduction, while an unstable system will. The BSE-status of a country assessed by this system was used by the European Commission as the basis for trade legislation rules for cattle and their products. The GBR was an invaluable tool in evaluating the potential global spread of BSE as it demonstrated how a disease could be transferred through international trade. This was shown to be a critical factor to address in reducing the spread and amplification of BSE throughout the world. Furthermore, GBR resulted in the implementation of additional measures and management activities both to improve surveillance and to prevent transmission within the cattle population.


Copyright © 2012 Elsevier B.V. All rights reserved.






see more here ;






IN SHORT, AND IN A NUT SHELL ;


(Adopted by the International Committee of the OIE on 23 May 2006)


11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to the Central Bureau,






PO-028: Oral transmission of L-type bovine spongiform encephalopathy (L-BSE) in primate model Microcebus murinus


Nadine Mestre-Frances,1 Simon Nicot,2 Sylvie Rouland,1 Anne-Gaëlle Biacabe,2 Isabelle Quadrio,3 Armand Perret-Liaudet,3 Thierry Baron,2 Jean-Michel Verdier1 1IN SER M UM2; Montpellier, France; 2Anses; Lyon, France; 3Hopitaux Civils de Lyon; Lyon, France


An atypical form of bovine spongiform encephalopathy has been identified in cattle in Europe, North America and Japan and was designed as L-type BSE (L-BSE) due to the lower apparent molecular mass of the unglycosylated, protease-resistant prion protein (PrPres) detected by western blot compared with classical BSE. Experimental evidences from studies in transgenic mice expressing human PrP and in primate models suggest a higher risk of transmission to humans of the L-BSE form than for classical BSE agent. However, a major unresolved issue concerns the potential transmissibility of the L-BSE agent by oral route. To address this question, we infected mouse lemurs (Microcebus murinus), a non-human primate model, with L-BSE by intracerebral or oral route.


Four adult lemurs were intracerebrally (IC) inoculated with 5mg of L-BSE infected brain homogenate of an atypical French BSE case (02-2528). Four young and four adult animals were fed with 5 mg or 50 mg of infected brain. After sacrifice, the brain tissues were biochemically and immunocytochemically investigated for PrPres.


The 4 animals IC inoculated died at 19 and 22 months postinoculation (mpi). They developed blindness, tremor, abnormal posture, incoordinated movements, balance loss. Symptoms get worse according to the disease progression, until severe ataxia. Severe spongiosis was evidenced into the thalamus, the striatum, the mesencephalon, and the brainstem, whereas into the cortex the vacuolisation was weaker. Strong deposits of PrPres were detected into the thalamus, the striatum, and the hippocampus whereas in the cerebral cortex, PrPres was prominently accumulated in plaques.


The orally inoculated animals showed similar clinical symptoms occurring between 27 and 34 mpi. Disease was characterized by progressive prostration, loss of appetite and poor appearance of the fur. Only one adult animal showed disequilibrium. PrPres was strongly accumulated only in the striatum and thalamus and weakly into the cortex. No plaques were evidenced. Two animals that were orally challenged at the age of two years are still alive and healthy 34 months after inoculation. The western blot analysis showed uniform molecular profiles, irrespective of the route or dose of infection, and included notably a PrPres form with low apparent molecular mass (~19 kDa) similar to the PrPres in the original cattle brain. However, the PrPres profile in lemurs was characterized by a higher proportion of di- and mono-glycosylated species (up to 95% of the total signal) than in the bovine L-BSE inoculum (~80%). In addition, small amounts of PrPres were detected by western blotting in the spleen of three animals (one intra-cerebrally inoculated and two fed with 5 mg of cattle brain).


Here, we demonstrate that the L-BSE agent can be transmitted by oral route from cattle to young and adult mouse lemurs. In comparison to IC inoculated animals, orally challenged lemurs were characterized by longer survival periods as expected with this route of infection.








Friday, May 25, 2012


R-CALF USDA’s New BSE Rule Eliminates Important Protections Needed to Prevent BSE Spread






Saturday, May 26, 2012


Are USDA assurances on mad cow case 'gross oversimplification'?


SNIP...


What irks many scientists is the USDA’s April 25 statement that the rare disease is “not generally associated with an animal consuming infected feed.”


The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul Brown, one of the world’s experts on this type of disease who retired recently from the National Institutes of Health. "(The agency) has no foundation on which to base that statement.”


“We can’t say it’s not feed related,” agreed Dr. Linda Detwiler, an official with the USDA during the Clinton Administration now at Mississippi State.


In the May 1 email to me, USDA’s Cole backed off a bit. “No one knows the origins of atypical cases of BSE,” she said


The argument about feed is critical because if feed is the cause, not a spontaneous mutation, the California cow could be part of a larger outbreak.


SNIP...






Sunday, May 27, 2012


CANADA PLANS TO IMPRISON ANYONE SPEAKING ABOUT MAD COW or ANY OTHER DISEASE OUTBREAK


CENSORSHIP IS A TERRIBLE THING






Sunday, May 27, 2012


GAIN REPORT BSE Case in United States Will Not Affect Trade, States Canadian Food Inspection Agency







Subject: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products APHIS-2008-0010-0008 RIN:0579-AC68


Comment from Terry Singeltary Document ID: APHIS-2008-0010-0008 Document Type: Public Submission This is comment on Proposed Rule: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products Docket ID: APHIS-2008-0010 RIN:0579-AC68


Topics: No Topics associated with this document View Document: More Document Subtype: Public Comment Status: Posted Received Date: March 22 2012, at 12:00 AM Eastern Daylight Time Date Posted: March 22 2012, at 12:00 AM Eastern Daylight Time Comment Start Date: March 16 2012, at 12:00 AM Eastern Daylight Time Comment Due Date: May 15 2012, at 11:59 PM Eastern Daylight Time Tracking Number: 80fdd617 First Name: Terry Middle Name: S. Last Name: Singeltary City: Bacliff Country: United States State or Province: TX Organization Name: CJD TSE PRION Submitter's Representative: CONSUMERS


Comment: comment submission Document ID APHIS-2008-0010-0001


Greetings USDA,


OIE et al, what a difference it makes with science, from one day to the next. i.e. that mad cow gold card the USA once held. up until that fateful day in December of 2003, the science of BSE was NO IMPORTS TO USA FROM BSE COUNTRY. what a difference a day makes$ now that the shoe is on the other foot, the USDA via the OIE, wants to change science again, just for trade $ I implore the OIE decision and policy makers, for the sake of the world, to refuse any status quo of the USA BSE risk assessment. if at al, the USA BSE GBR should be raise to BSE GBR IV, for the following reasons. North America is awash with many different TSE Prion strains, in many different species, and they are mutating and spreading. IF the OIE, and whatever policy makers, do anything but raise the risk factor for BSE in North America, they I would regard that to be highly suspicious. IN fact, it would be criminal in my opinion, because the OIE knows this, and to knowingly expose the rest of the world to this dangerous pathogen, would be ‘knowingly’ and ‘willfully’, just for the almighty dollar, once again. I warned the OIE about all this, including the risk factors for CWD, and the fact that the zoonosis potential was great, way back in 2002. THE OIE in collaboration with the USDA, made the legal trading of the atypical Nor-98 Scrapie a legal global commodity. yes, thanks to the OIE and the USDA et al, it’s now legal to trade the atypical Nor-98 Scrapie strain all around the globe. IF you let them, they will do the same thing with atypical BSE and CWD (both strains to date). This with science showing that indeed these TSE prion strains are transmissible. I strenuously urge the OIE et al to refuse any weakening to the USA trade protocols for the BSE TSE prion disease (all strains), and urge them to reclassify the USA with BSE GBR IV risk factor.


SEE REFERENCE SOURCES IN ATTACHMENTS


PLEASE SEE Terry S. Singeltary Sr. _Attachment_ WORD FILE ;











***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.


Second threat


snip...






MAD COW USDA ATYPICAL L-TYPE BASE BSE, the rest of the story...


***Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model






***Infectivity in skeletal muscle of BASE-infected cattle






***feedstuffs- It also suggests a similar cause or source for atypical BSE in these countries.






***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans.





The present study demonstrated successful intraspecies transmission of H-type BSE to cattle and the distribution and immunolabeling patterns of PrPSc in the brain of the H-type BSE-challenged cattle. TSE agent virulence can be minimally defined by oral transmission of different TSE agents (C-type, L-type, and H-type BSE agents) [59]. Oral transmission studies with H-type BSEinfected cattle have been initiated and are underway to provide information regarding the extent of similarity in the immunohistochemical and molecular features before and after transmission.


In addition, the present data will support risk assessments in some peripheral tissues derived from cattle affected with H-type BSE.






in the url that follows, I have posted



SRM breaches first, as late as 2011.


then


MAD COW FEED BAN BREACHES AND TONNAGES OF MAD COW FEED IN COMMERCE up until 2007, when they ceased posting them.


then,


MAD COW SURVEILLANCE BREACHES.


Friday, May 18, 2012


Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States Friday May 18, 2012






TSS



Wednesday, May 30, 2012


PO-028: Oral transmission of L-type bovine spongiform encephalopathy (L-BSE) in primate model Microcebus murinus





Tuesday, June 05, 2012


Captive Deer Breeding Legislation Overwhelmingly Defeated During 2012 Legislative Session






Saturday, June 09, 2012


USDA Establishes a Herd Certification Program for Chronic Wasting Disease in the United States





Monday, June 11, 2012


another atypical Nor-98 Scrapie case documented in Canada for 2012






too bad Canada’s policy on BSE aka mad cow type disease, and the reporting there from of completed cases, have ceased to exist on the CFIA site for the public to follow.



you have to request a copy. CFIA ceased giving those copies out to me. ...


•Request a copy of a completed BSE investigation report for a case after January 2009





Sunday, May 27, 2012


CANADA PLANS TO IMPRISON ANYONE SPEAKING ABOUT MAD COW or ANY OTHER DISEASE OUTBREAK, CENSORSHIP IS A TERRIBLE THING





Thursday, March 29, 2012


atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012


NIAA Annual Conference April 11-14, 2011San Antonio, Texas





Monday, November 30, 2009


USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE





price of prion poker goes up again $$$



Monday, June 11, 2012


Guidance for Industry Draft Guidance for Industry: Amendment to “Guidance for Industry: Revised Preventive Measures to Reduce the Possible Risk of Transmission of Creutzfeldt-Jakob Disease and Variant Creutzfeldt-Jakob Disease by Blood and Blood Products”






layperson


kind regards, terry

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