• Vol. 27 No. 6, 854–856
  • 15 November 1998

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 synchronous 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 occipital parasagittal area, left more than right. 14-3-3 protein was detected in the cerebrospinal fluid. The patient died within 5 months of presentation.

The electroencephalographic pattern is distinctive in many but not in all patients with Creutzfeldt-Jakob disease (CJD). Often, it is one of diffuse and non-specific slowing in the background with stereotypical generalised periodic high-voltage slow and sharp wave complexes.

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