NR AVPA
AU Ishihara,K.; Sugie,M.; Shiota,J.; Kawamura,M.; Kitamoto,T.; Nakano,I.
TI Severe cortical involvement in MV2 Creutzfeldt-Jakob disease: an autopsy case report.
QU Neuropathology 2006 Oct; 26(5): 433-7
PT case reports; journal article; research support, non-u.s. gov't
AB 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.
MH Aged; Autopsy; Blotting, Western; Cerebellum/metabolism/pathology; Cerebral Cortex/metabolism/*pathology; Creutzfeldt-Jakob Syndrome/metabolism/*pathology/physiopathology; Female; Humans; Magnetic Resonance Imaging; Prions/metabolism
AD Department of Neurology, Showa University School of Medicine, Hatanodai 1-5-8, Shinagawa-ku, Tokyo 142-8666, Japan. k-ishihara@mvj.biglobe.ne.jp
SP englisch
PO Australien