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

Purpose: To report three cases of idiopathic Creutzfeldt-Jakob disease (CJD) with visual symptoms. Cases: 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. Observations: 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. Conclusion: Ophthalmologists should be aware that the visual impairment can be the first symptom of CJD.