Creutzfeldt-Jakob disease, new variant Creutzfeldt-Jakob disease, and bovine spongiform encephalopathy

C. C. Weihl, R. P. Roos

Research output: Contribution to journalArticlepeer-review

23 Scopus citations


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.

Original languageEnglish
Pages (from-to)835-859
Number of pages25
JournalNeurologic Clinics
Issue number4
StatePublished - 1999


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