The first case of variant Creutzfeldt-Jakob disease (vCJD)
in a Japanese patient was announced by the Japanese Ministry of Health, Labour
and Welfare on 4 February 2005 [1]. The patient, who first experienced neurological
symptoms in December 2001, and died in December 2004, was male and in his
40s.
The patient is reported to have spent approximately one month in the United
Kingdom in 1988, although the exact length of his stay has not yet been
confirmed. The possibility of exposure to the vCJD infective agent while
the patient was in the UK is widely accepted at present.
On 16 September 2004, the Japanese CJD surveillance panel, in collaboration
with the United Kingdom National CJD surveillance unit (http://www.cjd.ed.ac.uk/)
diagnosed an isolated form of CJD, and confirmed the need to further observe
the developing symptoms.
On 3 February 2005, the CJD sub-committee reported that the characteristic
findings of the post-mortem pathological investigation, which included a
western blot, strongly indicated vCJD. A genetic mutation of the prion protein
has not been detected.
There are no records of the patient ever having received a blood transfusion.
Records of the Japanese Red Cross Society are still being checked to determine
whether he ever donated blood.
There is ongoing further investigation into the cause of this case and
the possibility of secondary transmission. The public is being provided
with accurate information regarding vCJD.
Following the announcement of this case, the Japanese Pharmaceutical and
Food Safety Bureau’s Blood and Blood Products Steering Committee have maintained
restrictions on blood donors who have lived in the UK, but these will be
regularly reviewed. The Japanese Red Cross Society has been instructed to
refuse donations from people who have lived in the UK for longer than one
month.
Japan first reported cases of bovine spongiform encephalopathy (BSE) in
farmed cows in 2001 and 14 cases of BSE were reported between 2001 and 2004
[3,4]. After the first case, the import, production and use of meat and
bone meal was banned, and Japan implemented a programme of testing every
carcass for BSE, and removing specified risk materials. It also suspended
beef imports from countries with BSE.
Acknowledgements:
Thanks to Kate Manvatkar for translation of the press releases from Japanese
and to Neil Barron for checking the translation.