Variant Creutzfeldt-Jakob disease in Hong Kong

R Kay, WY Lau, HK Ng, YL Chan, DJ Lyon, CA van Hasselt
Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong

A 34-year-old Chinese woman who had lived in the United Kingdom in the 1980s was admitted to hospital in Hong Kong because of a 7-month history of progressive neurological deterioration. Initially, she complained of heartburn and paraesthesia of the hands and feet. She then developed slowness of speech and gait, and was noted to be forgetful and irritable. In January 2001, she was brought back to Hong Kong for treatment. On admission in May she was dysarthric, ataxic, and dystonic. Magnetic resonance imaging showed high signals in both thalami suggestive of variant Creutzfeldt-Jakob disease. Other investigations, including electroencephalogram and lumbar puncture, were unremarkable. A tonsil biopsy showed the presence of prions. This patientÕs presentation is typical of the variant Creutzfeldt-Jakob disease cases that have been reported since 1996. Because of her residential history, we conclude that this is an imported case from the United Kingdom.

Hong Kong Med J 2001;7:296-8

Key words: Creutzfeldt-Jakob syndrome; Encephalopathy, bovine spongiform; Great Britain; Hong Kong; Prions