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PURPOSE: Although in the early stage of Creutzfeldt-Jakob disease most patients have obvious dementia, we found that the disease can be diagnosed in patients solely on the basis of a visual system disorder. METHODS: We examined three patients who initially complained of a nonspecific, insidious visual disturbance. RESULTS: The three patients were found to have Creutzfeldt-Jakob disease, confirmed by histopathologic analysis. Each patient had a homonymous hemianopsia on the first neuro-ophthalmologic examination. The initial neurologic, neuroimaging, and electrophysiologic examinations were not conclusive. The subsequent rapid deterioration in the neurologic status, including dementia and typical electroencephalographic changes, was suggestive of Creutzfeldt-Jakob disease. CONCLUSION: At onset of Creutzfeldt-Jakob disease, patients may have a homonymous hemianopsia despite normal results of magnetic resonance imaging of the brain and neurologic examination