Severe cortical involvement in MV2 Creutzfeldt-Jakob disease: An autopsy case report

Kenji Ishihara, Masayuki Sugie, Jun Ichi Shiota, Mitsuru Kawamura, Tetsuyuki Kitamoto, Imaharu Nakano

Research output: Contribution to journalArticlepeer-review

5 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)433-437
Number of pages5
Issue number5
Publication statusPublished - 2006 Oct


  • Creutzfeldt-Jakob disease
  • MRI
  • MV2
  • Pathology

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology

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