Creutzfeldt-Jakob disease (CJD) is an invariably fatal neurodegenerative disease, caused by tissue deposition of a misfolded prion protein (PrP) [1]. It is categorized etiologically into sporadic, genetic, iatrogenic, and variant forms [2]. The genetic form of CJD, accounting for approximately 10%-15% of all cases, results from prion protein encoding gene PRNP mutation and is inherited with an autosomal dominant pattern [2].While CJD classically presents as rapidly progressive dementia, a small subgroup of cases present with prominent visual symptoms at
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