001 CREUTZFELDT-JAKOB DISEASE (CJD)

R. G. Will. CJD Surveillance Unit, Western General Hospital, Edinburgh, UK

CJD is one of the transmissible spongiform encephalopathies or prion diseases. There are a number of subtypes of human prion disease, including sporadic CJD, which occurs worldwide with an annual mortality rate of about 1 case/million population, hereditary forms associated with mutations of the human prion protein gene, and iatrogenic cases related to accidental transmission of infection from person to person; for example, through human pituitary hormones and human dura mater grafts.

This group of diseases has become the subject of widespread public concern following the identification of variant CJD as a zoonotic disease, caused by transmission of the agent of bovine spongiform encephalopathy (BSE) from cattle to humans. 151 cases of variant CJD have been identified in the UK, eight in France and single cases in Italy, Ireland, Canada, and the USA. The number of annual deaths from variant CJD in the UK appears to be in decline, but uncertainty remains about the future course of the epidemic.

Public health concerns have increased following the recent identification of a possible case of transfusion-transmitted variant CJD and a second “pre-clinical” case also linked to blood transfusion.

002 CLINICAL PATHOLOGICAL FEATURES OF MULTIPLE SCLEROSIS AND NEUROMYELITIS OPTICA IN ASIA

C. Lu¹, Z. Li¹, Y. Wang¹, Y. Hashizume². ¹Department of Neurology, Huashan Hospital, 32 Wulumuqi Road, 200040 Shanghai, China; ²Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute-cho, Aichi-gun, 480–1195, Japan

Early clinical and epidemiological cohorts have shown the distinguished features between oriental multiple sclerosis (MS) and western MS in terms of clinical manifestation, the process of disease, the severity, and the response for therapy. To evaluate the spectrum of MS and neuromyelitis optica (NMO), including demographic data, clinical characteristics, clinical courses, and to evaluate CSF, and serologic studies, …