The diagnosis of Creutzfeldt-Jakob disease (CJD) can be challenging as findings are non-specific and there is low awareness of the disease. We present a case of an 83-yearold man with a two months history of rapidly progressive dementia. After a series of extensive diagnostic examinations, he was diagnosed with probable sporadic CJD with key findings of rapidly progressive dementia, myoclonus, pyramidal signs, abnormal hyperintensity signals on diffusion-weighted magnetic resonance imaging (DW-MRI) and typical electroencephalograph (EEG). His symptoms progressively worsened and he died four months after the onset of symptoms.
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.