title: Graft-related disease progression in dura mater graft-associated Creutzfeldt-Jakob disease: a cross-sectional study.
abstract: OBJECTIVE
  Details of abnormal prion protein (PrP(Sc)) propagation in the human central nervous system (CNS) are unclear. To assess the spread of PrP(Sc) through the human CNS, we evaluated dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) cases focusing on sites of grafting and dCJD pathological subtypes.
  
  
  METHODS
  A cross-sectional study.
  
  
  METHODS
  nationwide surveillance data of human prion diseases in Japan over the past 12 years were applied for the study.
  
  
  METHODS
  Clinical data were obtained from 84 dCJD patients.
  
  
  METHODS
  The clinical courses in cases of dCJD were analysed according to the grafting sites (supratentorial and infratentorial groups) and the pathological subtypes (non-plaque and plaque types).
  
  
  RESULTS
  Of the 84 cases of dCJD in this study, 36 (43%) were included in the supratentorial group and 39 (46%) were included in the infratentorial group. As initial manifestations, vertigo (p=0.007) and diplopia (p=0.041) were significantly more frequent in the infratentorial group than in the supratentorial group. During their clinical course, cerebellar signs appeared more frequently in the infratentorial group than in the supratentorial group (p=0.024). In the non-plaque type cases (n=53), the infratentorial group developed vertigo more frequently than the supratentorial group (p=0.017); moreover, cerebellar signs appeared more frequently in the infratentorial group (p=0.014). However, there was no significant difference between groups in the plaque type (n=18).
  
  
  CONCLUSIONS
  The high frequency of clinical manifestations related to brain stem and cerebellar dysfunction in the non-plaque type dCJD with infratentorial grafting suggests that PrP(Sc) commonly shows direct propagation into the CNS from contaminated dura mater grafts.
authors: Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
authors: Kenji
authors: K
authors: Sakai
authors: Tsuyoshi
authors: T
authors: Hamaguchi
authors: Moeko
authors: M
authors: Noguchi-Shinohara
authors: Ichiro
authors: I
authors: Nozaki
authors: Ichiro
authors: I
authors: Takumi
authors: Nobuo
authors: N
authors: Sanjo
authors: Yosikazu
authors: Y
authors: Nakamura
authors: Tetsuyuki
authors: T
authors: Kitamoto
authors: Nobuhito
authors: N
authors: Saito
authors: Hidehiro
authors: H
authors: Mizusawa
authors: Masahito
authors: M
authors: Yamada
keywords: Infectious Diseases
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