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Research paper
Insight into the frequent occurrence of dura mater graft-associated Creutzfeldt-Jakob disease in Japan
  1. Tsuyoshi Hamaguchi1,
  2. Kenji Sakai1,
  3. Moeko Noguchi-Shinohara1,
  4. Ichiro Nozaki1,
  5. Ichiro Takumi2,
  6. Nobuo Sanjo3,
  7. Atsuko Sadakane4,
  8. Yosikazu Nakamura4,
  9. Tetsuyuki Kitamoto5,
  10. Nobuhito Saito6,
  11. Hidehiro Mizusawa3,
  12. Masahito Yamada1
  1. 1Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan
  2. 2Department of Neurosurgery, Nippon Medical School Musashi Kosugi Hospital, Kawasaki, Kanagawa, Japan
  3. 3Department of Neurology and Neurological Science, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan
  4. 4Department of Public Health, Jichi Medical University, Shimotsuke, Japan
  5. 5Department of Prion Protein Research, Division of CJD Science and Technology, Tohoku University Graduate School of Medicine, Sendai, Japan
  6. 6Department of Neurosurgery, Faculty of Medicine, The University of Tokyo, Tokyo, Japan
  1. Correspondence to Professor Masahito Yamada, Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, 13-1 Takara-machi, Kanazawa 920-8640, Japan; m-yamada{at}


Objective More than 60% of patients worldwide with Creutzfeldt-Jakob disease (CJD) associated with dura mater graft (dCJD) have been diagnosed in Japan. The remarkable frequency of dura mater grafts in Japan may possibly contribute to the elevated incidence of dCJD, but reasons for the disproportionate use of this procedure in Japan remain unclear. We investigated differences between dCJD patients in Japan and those elsewhere to help explain the more frequent use of cadaveric dura mater and the high incidence of dCJD in Japan.

Methods We obtained data on dCJD patients in Japan from the Japanese national CJD surveillance programme and on dCJD patients in other countries from the extant literature. We compared the demographic, clinical and pathological features of dCJD patients in Japan with those from other countries.

Results Data were obtained for 142 dCJD patients in Japan and 53 dCJD patients elsewhere. The medical conditions preceding dura mater graft transplantation were significantly different between Japan and other countries (p<0.001); in Japan, there were more cases of cerebrovascular disease and hemifacial spasm or trigeminal neuralgia. Patients with dCJD in Japan received dura mater graft more often for non-life-threatening conditions, such as meningioma, hemifacial spasm and trigeminal neuralgia, than in other countries.

Conclusions Differences in the medical conditions precipitating dura mater graft may contribute to the frequent use of cadaveric dura mater and the higher incidence of dCJD in Japan.

  • Creutzfeldt-Jakob Disease
  • Infectious Diseases

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