NR AMHK
AU Weihl,C.C.; Roos,R.P.
TI Creutzfeldt-Jakob disease, new variant Creutzfeldt-Jakob disease, and bovine spongiform encephalopathy
QU Neurologic Clinics 1999 Nov; 17(4): 835-59
PT journal article; review; review, tutorial
AB Creutzfeldt-Jakob disease (CJD) is a subacute spongiform encephalopathy (SSE) that is manifested by a variety of neurologic signs that usually include dementia, myoclonus, and an abnormal electroencephalogram (EEG). In 1996, a new variant of CJD (nvCJD) with a somewhat distinctive clinical presentation and neuropathology was reported in adolescents and young adults, a cohort of patients not normally affected with CJD. The appearance of nvCJD coincided temporally and geographically with the emergence of an SSE in cattle known as bovine spongiform encephalopathy (BSE), or mad cow disease. This article discusses the clinical syndrome, pathology, and pathogenesis of classical CJD, nvCJD, and other human SSEs, as well as the link between BSE and nvCJD.
ZR 89
MH Adult; Animal; Case Report; Cattle; Creutzfeldt-Jakob Syndrome/*diagnosis; Diagnosis, Differential; Encephalopathy, Bovine Spongiform/*diagnosis; Frontal Lobe/pathology; Human; Male
AD Department of Neurology, University of Chicago Medical Center, Chicago, Illinois.
SP englisch
PO USA