The first Japanese patient with variant Creutzfeldt-Jakob disease (vCJD)

Akiyo Shinde, Takenobu Kunieda, Yoshimi Kinoshita, Reika Wate, Satoshi Nakano, Hidefumi Ito, Masahito Yamada, Tetsuyuki Kitamoto, Yosikazu Nakamura, Sadayuki Matsumoto, Hirofumi Kusaka

Research output: Contribution to journalArticlepeer-review

7 Citations (Scopus)

Abstract

Eleven years after a brief visit to some European countries, a 48-year-old Japanese man developed writing difficulty, irritability and general fatigue. Then he complained of dysesthetic pains in his legs, for which benzodiazepines were prescribed. However, at the time pulvinar sign was retrospectively confirmed on brain MRI. Eighteen months after the onset, his gait became ataxic with rapid deterioration of mental status over the following several months. Thirty-one months after the onset, he became akinetic and mute with periodic synchronous discharges on EEG, and died at the age of 51. The total clinical course was approximately 43 months. Pathological examination revealed the characteristic alterations of spongiform encephalopathy, severe in the thalamus, moderate but widely spread in the cerebral cortices, and moderate in the cerebellum. Abundant amyloid plaques were easily identified in the cerebral cortex and the cerebellum on HE staining. Immunohistochemistry for abnormal prion protein (PrP sc) confirmed amyloid plaques in several forms, such as florid, uni- and multi-centric plaques as well as perineuronal and periaxonal deposits in the basal ganglia and synaptic patterns in the thalami. A Western blotting study identified type 2B protease-resistant PrP. This is the first Japanese patient who was definitely diagnosed as variant Creutzfeldt-Jakob disease (vCJD). The pathological findings were similar to those of previous reports of vCJD in the UK. However, the changes were much more severe both in degree and distribution, probably due to a longer duration of the illness than those in the UK.

Original languageEnglish
Pages (from-to)713-719
Number of pages7
JournalNeuropathology
Volume29
Issue number6
DOIs
Publication statusPublished - 2009 Dec

Keywords

  • Latent period
  • Periodic synchronous discharge
  • Pulvinar sign
  • Spongiform encephalopathy
  • Variant Creutzfeldt-Jakob disease

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Clinical Neurology

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