Creutzfeldt-Jakob Disease Presenting with Visual Blurring, Diplopia and Visual Loss: Heidenhain's Variant

Focal electroencephalographic abnormalities as described in Heidenhain’s variant of Creutzfeldt-Jakob disease are uncommon. We report a 73-year-old male presenting with visual symptoms, right hemianopia and rapidly progressive dementia. Myoclonus was synchronou s with generalised periodic epileptiform discharges on electroencephalography (EEG). In addition, there were periodic focal sharp waves at the left occipital region. Diffusion-weighted magnetic resonance brain images showed slightly increased signal intensity in the occipita l parasagittal area, left more than right. 14-3-3 protein was detected in the cerebrospinal fluid. The patient died within 5 months of present ation. Ann Acad Med Singapore 1998; 27:854-6