Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008
Posted Apr 09 2009 7:13pm
Eurosurveillance, Volume 13, Issue 15, 10 April 2008 Rapid communications Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008 J de Pedro Cuesta ()1 Instituto de Salud Carlos III, Department of Applied Epidemiology, National Centre of Epidemiology, Madrid, Spain
Citation style for this article: de Pedro Cuesta J. Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008. Euro Surveill. 2008;13(15):pii=18831. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=18831
In 2005, the first case of variant Creutzfeldt-Jakob disease (vCJD) was reported in Spain, in a woman born in 1978 with clinical onset of symptoms in 2004 . She subsequently died in 2005.
Recently, two more laboratory-confirmed vCJD cases were reported to the Spanish CJD state registry. In February 2006, a woman born in 1957 developed progressive cognitive deterioration, and died in December 2007 with suspected sporadic CJD (typical EEG in October 2007) MM at codon 129 and no mutations in PRPN gene. A man born in 1967 had onset in May 2007 with psychiatric symptoms, and after several months developed progressive cognitive decline with dementia, typical MRI, MM at codon 129, no mutations in PRPN gene. He died in February 2008. Post-mortem, neuropathology with histochemistry confirmed vCJD in both cases. No clear specific dietary habits, blood donations or reception were recorded. Neither case appears to have visited the United Kingdom before 2004.
The latest two cases were resident in the same region of the country, Castilla y Leon, but no link between them was established.
Spain Awaits Lab Result to Confirm Fourth Mad Cow Disease Case
Spanish health authorities said they were looking into the possibility that a woman who died last week at a hospital in Leon suffered from the human variant of mad cow disease.
If laboratory tests confirm the 64-year-old had Creutzfeldt-Jakob Disease (CJD), the human variant of bovine spongiform encephalopathy (BSE) or mad cow disease, it will be the fourth death from the disease in Spain.
"We are currently carrying out tests at a laboratory in Alcorcon to determine if the disease is Creutzfeldt-Jakob or not," a health ministry spokesman told AFP.
Regional newspaper Diario de Leon said the woman was the mother of the last person to die in Spain from the brain-wasting disease, a 41-year-old man who died in February 2008.
The health ministry and regional authorities could not confirm the parental link between the two.
Spain recorded its first human death from mad cow disease in June 2005 when a 26-year-old woman succumbed to it in Madrid.
More than 200 people around the world are suspected to have died, most of them in Britain, from the human variant of the disease, which was first described in 1996.
Scientists believe the disease was caused by using infected parts of cattle to make feed for other cattle.
Authorities believe eating meat from infected animals can trigger the human variant of the fatal brain-wasting disease.
The 27-member EU, of which Spain is part, has banned high-risk materials such as spinal cord from use in feed and stricter labelling was also introduced.
Two people die from vCJD in Spain Two people have died in Spain after contracting the human form of "mad cow disease", variant Creutzfeldt-Jakob Disease (vCJD) that is is thought to be transmitted in infected meat and bone. Since 2000, more than 800 Spanish have suffered from different types of vCJD.
Variant Creutzfeldt-Jakob Disease (vCJD) was first found in humans in 1995 and is thought to be transmitted in infected meat and bone. Since 2000, more than 800 Spanish have suffered from different types of vCJD but the only case of a death in the country was in July 2005.
Recently, however, two people have died in Spain after contracting this human form of "mad cow disease". The woman, 50 years old, died in December 2007 while the man, 41 years old, died in February 2008.
Elena Espinosa, from the Spanish Ministry of Health, has asked the consumers and the farmer sector to "be calmed". Javier Castrodeza, Public Health General Director of the Regional Government of Castilla Leon, where the two people died, declared that this facts don’t have any "relevant health consequences" and "don’t involve a public health problem" at all.
For further information
Creutzfeldt-Jakob Disease (vCJD) Spanish Ministry of Health. Regional Government of Castilla Leon El Pais. "Dos muertes por vacas locas"(Spanish). El Mundo. "Los dos casos de ’vacas locas’ traen a la memoria la crisis ganadera de 2001"(Spanish)
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Citation style for this article: Centro Nacional de Epidemiología, Instituto de Salud Carlos III. First case of vCJD reported in Spain. Euro Surveill. 2005;10(31):pii=2764. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=2764 Date of submission: --------------------------------------------------------------------------------
Centro Nacional de Epidemiología, Instituto de Salud Carlos III, Madrid, Spain (http://cne.isciii.es/)
The Spanish Ministry of Health has reported the detection of a case of variant Creutzfeldt-Jakob disease (vCJD) in Spain . The patient was a 26 year old woman in Madrid who died on 10 July 2005. She had no specific risk factors for vCJD (no prior blood transfusions or visits to the United Kingdom).
The patient’s symptoms began in November 2004, with rapid progression to dementia, normal MRI and EEG, and 14-3-3 protein found in cerebral spinal fluid. Other neurological manifestations including ataxia, dysarthria, apraxia and myoclonus appeared in early 2005. The first abnormal MRI was observed in April 2005. The patient was methionine homozygous at codon 129 of the prion protein gene (PRPN). There were no identified PRPN mutations or family history of CJD.
The case was notified to the Spanish CJD state registry in May 2005 and initially fitted the criteria for both probable sporadic CJD (sCJD) and possible vCJD; it was registered as probable sCJD. Brain neuropathology results of tests conducted at Fundación Hospital de Alcorcón at Madrid subsequently confirmed vCJD. Materials have been sent to the European reference centre at the United Kingdom’s National CJD Surveillance Unit in Edinburgh for further study, and results are expected later in August.
References: Ministerio de Sanidad y Consumo. La Comunidad de Madrid comunica al Ministerio de Sanidad la probable detección del primer caso de la variante de la enfermedad de Creutzfeldt-Jakob en España. Press release 29 July 2005. (http://www.msc.es/gabinetePrensa/notaPrensa/desarrolloNotaPrensa.jsp?id=385)
[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al  have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.
Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.