title: The first Japanese patient with variant Creutzfeldt-Jakob disease (vCJD).
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.
authors.affiliation: Department of Neurology, Kansai Medical University, Moriguchi, Japan.
authors.forename: Akiyo
authors.initials: A
authors.lastname: Shinde
authors.forename: Takenobu
authors.initials: T
authors.lastname: Kunieda
authors.forename: Yoshimi
authors.initials: Y
authors.lastname: Kinoshita
authors.forename: Reika
authors.initials: R
authors.lastname: Wate
authors.forename: Satoshi
authors.initials: S
authors.lastname: Nakano
authors.forename: Hidefumi
authors.initials: H
authors.lastname: Ito
authors.forename: Masahito
authors.initials: M
authors.lastname: Yamada
authors.forename: Tetsuyuki
authors.initials: T
authors.lastname: Kitamoto
authors.forename: Yosikazu
authors.initials: Y
authors.lastname: Nakamura
authors.forename: Sadayuki
authors.initials: S
authors.lastname: Matsumoto
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Kusaka
mesh_terms: D017668:Age of Onset; D044466:Asian People; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000378:metabolism / Q000473:pathology*; D018450:Disease Progression; D006801:Humans; D008297:Male; D008875:Middle Aged; D018620:PrPSc Proteins / Q000378:metabolism*
chemical_list: D018620:PrPSc Proteins