Creutzfeldt-Jakob disease with florid plaques after cadaveric dura mater graft.
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.
Mochizuki
Yoko
Y
Division of Neurology, Department of Medicine, Nihon University School of Medicine,Tokyo, Japan. mochi@ica.nihon-u.ne.jp
Mizutani
Tomohiko
T
Tajiri
Nobuo
N
Oinuma
Toshinori
T
Nemoto
Norimichi
N
Kakimi
Shigeo
S
Kitamoto
Tetsuyuki
T
D001259:Ataxia / Q000209:etiology; D015153:Blotting, Western; D001921:Brain / Q000473:pathology / Q000648:ultrastructure; D016380:Brain Tissue Transplantation / Q000009:adverse effects; D002102:Cadaver; D007562:Creutzfeldt-Jakob Syndrome / Q000145:classification* / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D003704:Dementia / Q000209:etiology; D004388:Dura Mater / Q000473:pathology / Q000637:transplantation* / Q000648:ultrastructure; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008875:Middle Aged; D058225:Plaque, Amyloid / Q000473:pathology; D011328:Prions / Q000378:metabolism; D013997:Time Factors
D011328:Prions