1/3. Creutzfeldt-Jakob disease, new variant creutzfeldt-jakob disease, and bovine spongiform encephalopathy.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.- - - - - - - - - - ranking = 1keywords = cattle (Clic here for more details about this article) |
2/3. A critical review of atypical cerebellum-type Creutzfeldt-Jakob disease: its relationship to "new variant" CJD and bovine spongiform encephalopathy.Shortly after the appearance of bovine spongiform encephalopathy (BSE), Creutzfeldt-Jakob disease (CJD) was identified in young patients with nonclassical presentation such as difficulty in balancing and ataxia. The classical CJD in older patients starts with dementia. To distinguish between the two types, CJD in young persons has been termed "new variant" (nvCJD). The distinguishing features of classical CJD include initial presentation with dementia, confluent spongiform changes are very unusual in the cerebellum, and PrP plaques are rarely observed. For nvCJD, initially, difficulty with balancing and ataxia occurs, confluent spongiform changes are seen in the cerebellum, and a large number of PrP plaques are seen. The icelandic observation of sheep scrapie revealed a predominantly ataxic form of scrapie, termed Type II, rather than the itchy form termed Type I. Both types have been known to exist in europe. Since the clinical signs of Type II scrapie in sheep with trembling and ataxia are similar to those seen in BSE and nvCJD, this suggests that Type II is the cause of BSE and nvCJD. Over 8 years, from 1989 to 1996, I examined the clinical histories of 33 CJD cases aged between the ages of 18 and 84. Six under the age of 40 and 15 over the age of 40 had leading clinical features such as difficulty in balancing and ataxia similar to those seen in the young cases classified as "nvCJD." Brains were examined from the six of 15 cases over the age of 40, which revealed similar pathology to that seen in young patients classified as "nvCJD." These findings suggest that all age groups are susceptible to the strain of the agent derived from BSE cattle.- - - - - - - - - - ranking = 1keywords = cattle (Clic here for more details about this article) |
3/3. BSE, public anxiety and private neurosis.Following the recent focus of media attention on BSE, and the putative link between the cattle disease and cases of variant CJD in humans in the UK, we report two cases of "BSE phobia." The relationship between popular conceptions of science and psychopathology is discussed.- - - - - - - - - - ranking = 1keywords = cattle (Clic here for more details about this article) |