title: Severe cortical involvement in MV2 Creutzfeldt-Jakob disease: an autopsy case report.
abstract: MV2 type sporadic Creutzfeldt-Jakob disease (sCJD) is reported to have a long duration and marked involvement of the cerebral deep gray matter. We describe an autopsied long-surviving sCJD case of MV2. In the early stages, the patient exhibited memory impairment, attention deficit and semantic memory disorder. Diffusion-weighted MRI showed abnormal hyperintensity signals along the cerebral cortex, sparing the thalami and basal ganglia. Pathological observations included: severe spongiosis throughout the cerebral cortex, several kuru plaques and plaque-like PrP deposits in the cerebellum, with only minimal degeneration in the thalami and basal ganglia. Our case suggests that MV2 has a wide clinicopathological spectrum, which ranges from "VV2" to "MM2" type.
authors.affiliation: Department of Neurology, Showa University School of Medicine, Hatanodai 1-5-8, Shinagawa-ku, Tokyo 142-8666, Japan. k-ishihara@mvj.biglobe.ne.jp
authors.forename: Kenji
authors.initials: K
authors.lastname: Ishihara
authors.forename: Masayuki
authors.initials: M
authors.lastname: Sugie
authors.forename: Jun-Ichi
authors.initials: J
authors.lastname: Shiota
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Kawamura
authors.forename: Tetsuyuki
authors.initials: T
authors.lastname: Kitamoto
authors.forename: Imaharu
authors.initials: I
authors.lastname: Nakano
mesh_terms: D000368:Aged; D001344:Autopsy; D015153:Blotting, Western; D002531:Cerebellum / Q000378:metabolism / Q000473:pathology; D002540:Cerebral Cortex / Q000378:metabolism / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D011328:Prions / Q000378:metabolism
chemical_list: D011328:Prions