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KMID : 1036520210110020040
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Clinical Neuro-Ophthalmology
2021 Volume.11 No. 2 p.40 ~ p.43
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Creutzfeldt-Jakob Disease Who Presented with Visual Field Defect
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Kim Hyun-Sung
Oh Eun-Hye
Choi Jae-Hwan
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Abstract
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A subclass of patients with Creutzfeldt-Jakob disease (CJD) present with isolated visual symptoms which persist without any cognitive decline for several weeks that is called the Heidenhain variant of CJD. A 66-year-old healthy man presented with blurred vision and dizziness 10 days ago. Examinations raised the suspicion of a right inferior quadrantanopsia, which was confirmed by Humphrey visual field test. The other examination including cognition were normal. The diffusion weighted magnetic resonance imaging of the brain exhibited increased signal intensity in the left parieto-temporo-occipital cortex. Electroencephalography disclosed continuous slow waves on the left hemisphere without sharp waves. In cerebrospinal fluid analyses, the 14-3-3 protein was positive, and total tau protein was elevated by 3,185.8 pg/mL. A real-time quaking-induced conversion assay identified pathological protein aggregation of PrPSc, confirming CJD. During the follow-up period, the patient gradually developed global bradykinesia and cognitive impairment. The Heidenhain variant of CJD should be considered a differential diagnosis in patients with visual field defect of unknown origin.
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KEYWORD
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Creutzfeldt-Jakob disease, Heidenhain variant, Visual field defect
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