A 72-year old Englishman was admitted with rapid deterioration in cognitive function, gait disturbance, and cerebellar signs and lapsed into a coma within one week of admission to the hospital. He had long-standing hypertension and hypercholesterolaemia, for which he was on regular medication. He had suffered recurrent episodes of stroke between September 1997 and May 2001. Three months prior to presentation, he became forgetful and generally mentally slow, affecting his daily activities. He was also noted to have fluctuations in his conscious level, associated with myoclonic jerks of the limbs. The brain MRI revealed hyperintense lesions on T2- weighted images in the periventricular region, left corona radiata, centrum semiovale, pons, midbrain and right thalamus. The electroencephalograph revealed periodic sharp wave complexes, strongly suggestive of Creutzfeldt-Jakob disease. However, we were not able to get a tissue diagnosis or send the cerebrospinal fluid for protein 14-3-3.