Thursday, January 06, 2011

Italy: 'Mad cow disease' claims second Italian victim

Italy: 'Mad cow disease' claims second Italian victim

Livorno, 6 Jan. (AKI) - The human variant of the brain-wasting 'mad cow' disease claimed a second victim in Italy after a 44-year-old woman died in hospital in the northwestern port city of Livorno, the local health authority reported on Thursday.

The health authority said the 44-year-old Livorno woman died on Wednesday and was admitted to a city hospice in July in the final stages of her battle against variant Creutzfeldt-Jakob disease (vCJD), commonly known as 'mad cow' disease.

There is there is no known cure for vCJD.

Italy's first case, a Sicilian woman, died in 2002, a year after she contracted the disease.

VCJD is the human form of the fatal brain-wasting illness first identified in cattle - BSE or bovine spongiform encephalopathy. A total of 275 people have been infected with the disease over the past decade, 170 in Britain, the worst-affected country, 56 in Israel, and 25 in France.

Nearly 200,000 cattle have died from BSE over the same period. The human form of BSE is believed to be caused by eating meat infected with the bovine disease.

EU restrictions on the sale of T-bone beef steaks and beef offal were lifted in 2006 when officials declared the 'mad cow' outbreak was over.
A massive cull in Britain prevented the spread of the disease but isolated cases are still reported.

Almost 190,000 cattle have died from BSE over the past ten years, the vast majority in the UK.

Italy's health ministry and various local health authorities have always denied vCJD patients can infect their family members and carers with the disease.


Eurosurveillance, Volume 6, Issue 6, 07 February 2002 Articles S Salmaso1


Citation style for this article: Salmaso S.

First case of vCJD reported in Italy.

Euro Surveill. 2002;6(6):pii=2022.

Available online:

Date of submission:



First case of vCJD reported in Italy

The first case of variant Creutzfeld-Jakob Disease (vCJD) has been reported in Italy (1). The case from Sicily was diagnosed in Italy and Great Britain on the basis of clinical and instrumental tests and tonsillar biopsy. The Istituto Superiore di Sanità (ISS) has classified the case as probable, but since the patient is still alive, the ISS has not released any additional information. The surveillance and reporting of CJD has been mandatory in Italy since the beginning of 2002. A ban on feeding mammalian protein to ruminants was approved on 28 July 1984, and enforced from 15 September that year. The prevalence of indigenous cases of bovine spongiform encephalopathy per 10 000 tests was 1.03 for the year 2001 (2).

References :

1.Ministero della Salute. Segnalato caso variante malattia Creutzfeldt-Jakob. Press release 05/02/2002 N° 40, 5 February 2002.


2.Centro per lo Studio e le Ricerche sulle Encefalopatie Animali e Neuropatologie Comparate. Focolai BSE in Italia: 2001/2002.


Reported by Stefania Salmaso

(, Istituto Superiore di Sanità, Italy.

Eurosurveillance, Volume 6, Issue 40, 03 October 2002


A M Molesworth1


Citation style for this article: Molesworth AM. First case of variant Creutzfeldt-Jakob disease reported in Italy - update. Euro Surveill. 2002;6(40):pii=1891. Available online: Date of submission:



First case of variant Creutzfeldt-Jakob disease reported in Italy - update

A paper published in the Lancet this week describes the first case of variant Creutzfeldt-Jakob disease (vCJD) in Italy (1). The patient, a 25 year old Sicilian woman, developed clinical symptoms in 2001 and was first reported in February this year (2). Although definitive diagnosis of vCJD requires neuropathological confirmation, the woman’s illness fulfils the diagnostic criteria for probable vCJD (3), a brain scan was characteristic of vCJD and the abnormal protein associated with vCJD has been detected in a tonsil biopsy.

The patient had never undergone neurosurgery, or received a blood transfusion or any other treatment that might be associated with transmission of a prion disease. Scientific evidence suggests that vCJD is, however, caused by transmission of the bovine spongiform encephalopathy agent (BSE) to humans. The woman is likely to have acquired her infection within Italy, having never travelled to the United Kingdom (UK) or any other country with reported BSE. Cases of indigenous BSE were first reported in Italy following the implementation, in 2001, of mandatory testing of cattle older than 30 months destined for slaughter (4). By the end of September 2002 70 indigenous cases and two British imported cases had been identified (5).

To date, in addition to the Italian case, single cases of definite or probable vCJD have been diagnosed with onset in each of the Republic of Ireland, Canada and the United States, a further six in France and 127 in the UK (see EUROCJD, The occurrence of cases both within and outside the UK remains a concern, supporting the need for continuous surveillance programmes of transmissible spongiform encephalopathies in humans and animals in the UK and other countries.

References :

La Bella V, Collinge J, Pocchiari M, Piccoli F. Variant Creutzfeldt-Jakob disease in an Italian woman. Lancet 2002; 360: 997-8. (

Salmaso S. First case of vCJD reported in Italy. Eurosurveillance Weekly 2002; 6: 020207. (

Will RG, Zeidler M, Stewart GE, Macleod MA, Ironside JW, Cousens SN, et al. Diagnosis of new variant Creutzfeldt-Jakob disease. Ann Neurol 2000;47: 575-82.

European Commission. Regulation (EC) No 999/2001 of the European Parliament and of the Council of 22 May 2001 (

Centro per lo Studio e le Ricerche sulle Encefalopatie Animali e Neuropatologie Comparate. Focolai BSE in Italia [BSE Outbreaks in Italy]: 2001/2002 (

Reported by Anna Molesworth ( and Peter Horby, Public Health Laboratory Service Communicable Disease Surveillance Centre, London, England.



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