Creutzfeldt-Jakob disease with florid plaques after cadaveric dura mater graft.

Abstract

A patient with dura-associated Creutzfeldt-Jakob disease (D-CJD) which occurred about 15 years after a dura mater graft is reported in the present study. The prion protein gene analysis disclosed no mutation. The D-CJD was atypical in: (i), the long interval between the onset of ataxia and the occurrence of dementia; (ii), the presence of transient myoclonus; and (iii), the presence of florid plaques in the brain. The electron-microscopic findings showed bundles of amyloid filaments which radiated from the center of the plaques without degenerating neurites. This case of D-CJD may belong to a new subtype of D-CJD.

• Authors: Yoko Mochizuki, Tomohiko Mizutani, Nobuo Tajiri, Toshinori Oinuma, Norimichi Nemoto, Shigeo Kakimi, Tetsuyuki Kitamoto
• Journal: Neuropathology : official journal of the Japanese Society of Neuropathology (Neuropathology) Vol. 23 Issue 2 Pg. 136-40 (Jun 2003) ISSN: 0919-6544 [Print] Australia
• PMID: 12777102 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Prions

Topics

• Ataxia (etiology)
• Blotting, Western
• Brain (pathology, ultrastructure)
• Brain Tissue Transplantation (adverse effects)
• Cadaver
• Creutzfeldt-Jakob Syndrome (classification, etiology, pathology, physiopathology)
• Dementia (etiology)
• Dura Mater (pathology, transplantation, ultrastructure)
• Fatal Outcome
• Female
• Humans
• Middle Aged
• Plaque, Amyloid (pathology)
• Prions (metabolism)
• Time Factors