BSE and variant Creutzfeldt-Jakob disease

Masahito Yamada^1),2)

¹⁾Department of Neurology and Neurobiology of Aging, Kanazawa University
Graduate School of Medical Science
²⁾Creutzfeldt-Jakob Disease Surveillance Committee, Japan

Evidence of the link between variant Creutzfeldt-Jakob disease (vCJD) and bovine spongiform encephalopathy (BSE) includes (1) the association of BSE and vCJD in time and place (the UK), (2) the specific pattern (Parchi type 2B/Collinge type 4) of protease-resistant PrP in Western blot analysis, and (3) the evidence in the transmission experiments with animals. vCJD has been reported with clinical features of characteristic neuropsychiatric manifestations, absence of periodic synchronous discharges (PSD) on EEG, and pulvinar sign on MRI. In February 2005, the CJD Surveillance Committee, Japan, identified the first Japanese case of vCJD showing PSD on EEG. We suggest revision of the WHO vCJD case definition (2001) to prevent missing cases of vCJD.

Address correspondence to Dr. Masahito Yamada, Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science (13-1, Takaramachi, Kanazawa 920-8640, Japan)