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Magnetic resonance imaging showed extensive cortical regions with hyperintensity in diffusion\u2010weighted images, and these regions showed swelling in T2\u2010weighted and fluid\u2010attenuated inversion recovery (FLAIR) images. The medial occipital cortex and striatum showed no apparent hyperintensity on diffusion\u2010weighted imaging (DWI). Mild myoclonus was detected, and the patient died 10 months after the onset of symptoms; she did not enter the akinetic mutism state. The patient's brain weighed 1050 g, and neuropathological examination showed extensive characteristic various\u2010sized and non\u2010confluent (VaSNoC) vacuoles in the cerebral cortex. These vacuoles were observable macroscopically by loupe on images of hematoxylin and eosin\u2010stained tissue. Gliosis, hypertrophic astrocytosis, and neuron loss were generally mild in character. Prion protein (PrP) immunostaining showed very mild diffuse\u2010synaptic\u2010type PrP deposition in the cerebral gray matter. These clinicopathological findings led us to several conclusions relative to the early disease pathology of V180I genetic Creutzfeldt\u2013Jakob disease: (i) spongiform change was not found in the medial occipital cortex, which corresponds to the results of DWI; (ii) VaSNoC\u2010type spongiform changes, extensively recognized in the cerebral cortex, corresponded to the DWI findings showing continued hyperintensity with higher brightness, and T2\u2010weighted and FLAIR images findings showing a swelling; and (iii) spongiform changes first appear in the deeper layer and subsequently in the superficial layer in the cerebral cortex.<\/jats:p>","DOI":"10.1111\/neup.12516","type":"journal-article","created":{"date-parts":[[2018,9,16]],"date-time":"2018-09-16T02:28:52Z","timestamp":1537064932000},"page":"638-645","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Autopsy case of V180I genetic Creutzfeldt\u2010Jakob disease 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