A 74-year old woman with progressive amnesia

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A 74-year old woman with progressive amnesia Teaching NeuroImages Neurology Resident and Fellow Section © 2018 American Academy of Neurology

Vignette A 74-year-old female developed amnesia that rapidly progressed over 6 months. She exhibited no apparent neurological abnormalities, except for cognitive decline. Mini-mental state examination was 20/30, mainly involving orientation and recent memory. Electroencephalogram revealed no periodic synchronous discharge. Cerebrospinal fluid analysis was negative for 14-3-3 protein. Sugiyama, et al © 2018 American Academy of Neurology

Imaging Brain MRI at 6 months after onset revealed increased signal intensity in the cerebral cortices with swelling on diffusion- (A and C) and T2-weighted images (B and D) (white arrows). Sugiyama, et al © 2018 American Academy of Neurology

Cerebral cortex swelling in Creutzfeldt-Jakob disease with V180I mutation Premortem diagnosis of Creutzfeldt–Jakob disease with V180I mutation, especially in early stage, merely based on clinical features is difficult.1, 2 MRI revealed cortical swelling that is associated with V180I mutation and prompts genetic testing.1, 2 1. Jin K, Shiga Y, Shibuya S, et al. Clinical features of Creutzfeldt-Jakob disease with V180I mutation. Neurology 2004;62:502-505. 2. Iwasaki Y, Mori K, Ito M, et al. An autopsy case of Creutzfeldt-Jakob disease with a prion protein gene codon 180 mutation presenting with pathological laughing and an exaggerated startle reaction. Neuropathology 2017;37:575-581. Sugiyama, et al © 2018 American Academy of Neurology