Open AccessCREUTZFELDT‐JAKOB DISEASE: An Autopsy Case of the Panencephalopathic Type and a Review of the Literature
Author(s) -
Ueda Norifumi,
Miyazaki Kichihei,
Imai Shiro,
Fukunishi Ryo
Publication year - 1985
Publication title -
acta patholigica japonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
ISSN - 0001-6632
DOI - 10.1111/j.1440-1827.1985.tb01445.x
Subject(s) - pathology , white matter , autopsy , degeneration (medical) , disease , creutzfeldt jakob syndrome , senile plaques , medicine , alzheimer's disease , prion protein , magnetic resonance imaging , radiology
This is a case report of a purported panencephalopathic type of Creutzfeldt‐Jakob (C‐J) disease in a 61‐year‐old Japanese farmer. He died nine months after the onset of clinical symptoms. This variety of C‐J disease was named and reported in 1981 by M izutani . The characteristic feature is extensive degeneration of cerebral white matter not related to cortical damage in addition to the spongiform changes in the cortical gray matter. The case was diagnosed clinically and at post mortem marked neural loss, astrocytic macrogliosis, and degeneration of subcortical white matter were found. Senile plaques and neurofibrillary tangles were virtually absent. Degeneration of the white matter is uncommon in C‐J disease, and when it occurs is usually mild, and limited in distribution. ACTA PATHOL. JPN. 35: 1483–1494, 1985.