Imaging and EEG Findings of Rare Heidenhain Variant of Creutzfeldt-Jakob Disease

Background: Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative spongiform encephalopathy. Findings are usually consistent with basal ganglia and frontal lobe destruction. However in certain variants, the clinical and diagnostic findings can differ. This case illustrates the occipital findings of the rare Heidenhain variant of CJD.

Methods: We describe the clinical course and radiologic findings of a 65-year-old with the Heidenhain variant of Creutzfeldt-Jakob disease. Diagnostic studies included lumbar puncture, EEG and MRI scans of the brain.

Results: This case illustrates the findings of the rare Heidenhain variant of CJD, including prominent changes in MRI and EEG in the occipital region. This was further supported by the positive enolase and 14-3-3 in cerebral spinal fluid.

Conclusions: In patients presenting with clinical symptoms of visual disorders of unclear origin presenting with or shortly after psychosis or dementia, this variant of CJD must be considered in the differential diagnosis.