Electroretinograms in three cases of Creutzfeldt-Jakob disease with visual disturbances

Abstract

To report three cases of idiopathic Creutzfeldt-Jakob disease (CJD) with visual symptoms. Patient 1 initially visited our ophthalmology department complaining of blurred vision. Electro-retinography (ERG) showed a selective reduction of the b wave before she was diagnosed with CJD. Patient 2 had normal ERG results even with severely advanced CJD. However, a reduction of the b wave on ERG was observed 1 month before the patient died. Patient 3 had visual disturbances at the initial visit, but the ERG result was normal even after the CJD had advanced. Atrophy of the visual cortex was later observed. CJD can alter the vision in various ways. The selective reduction of the b wave on ERG indicates the retinal involvement. However, visual disturbances may occur without the retinal implications. Ophthalmologists should be aware that the visual impairment can be the first symptom of CJD.