Visual symptoms in the Heidenhain variant of Creutzfeldt-Jakob Disease.

BACKGROUND AND PURPOSE: The distinguishing feature in Heidenhain variant Creutzfeldt-Jakob Disease (HvCJD) is the presence of visual symptoms preceding the appearance of other clinical manifestations. The purpose of this report is to describe the broad range of visual symptomatology in a patient with HvCJD.

METHODS

The authors describe the clinical, neuroimaging, and EEG features of a 51-year-old man with progressive visual symptoms that were most likely due to HvCJD. Migraine and epilepsy were initial diagnostic considerations.

RESULTS

Our patient presented with progressive visual decline and superimposed spells of visual dysfunction that had complex characteristics. Evolution of an abnormal signal in the parieto-occipital cortex on diffusion weighted MRI, changes on EEG, and clinical course were consistent with HvCJD.

CONCLUSIONS

HvCJD should be considered in all patients who present with unexplained visual phenomena. A remarkable spectrum of visual disturbances can be seen. Close follow-up as well as serial MRI and EEG can help clarify the underlying disease process. Diffusion-weighted and FLAIR sequences should be included in the MRI protocol.