NR ALGL
AU Sun,C.K.; Chong,X.Q.; Huang,Y.G.
TI Spongiform encephalopathies in mainland China
QU Lancet 1996 Aug 24; 348(9026): 544
PT letter
VT
Sir - Bovine spongiform encephalopathy (BSE) and a new variant of human Creutzfeldt-Jakob disease (CJD) in the UK have had international medical, economic, and political repercussions.1,2 Not a single case of BSE or scrapie has been reported in the People's Republic of China. Yet more than 30 confirmed and probably naturally occurring, sporadic or family, cases of spongiform encephalopathy including more than 20 cases of CJD and five of the slower progressing Gerstmann-Sträussler-Scheinker syndrome, have been described since 1980. None of the patients had a history of ever having received any probable contaminated medical treatment. Nor were they medical workers who had ever contacted any person with spongiform encephalopathy. The onset of progressive neuropsychiatric dysfunction at an early or late age is rare in these cases. However, there were six cases of spongy degeneration of the brain in infancy reported 12 years ago.3
The patients with confirmed or probable spongiform encephalopathy were mainly identified in eastern China, where the medical science and technology for diagnosis is more advanced. The diagnosis was mainly based on clinical features, electroencephalography, necropsy, or biopsy findings.3,4 Animal studies of transmissibility, and scrapie prion protein (PrPsc) immunohistochemical studies, were done only recently.5 It is likely, therefore, that the apparent rarity of spongiform encephalopathy in the People's Republic of China may be in part attributed to lack of appropriate diagnostic techniques, especially in the more remote regions, and the lack of necessary vigilance. It is expected that studies of the disease in China will soon also be reinforced by Chinese experts - ie, neurologists, veterinarians and other bioscientists having a common interest in the subject.
*Chang-Kai Sun, Xiao-Qin Chong, Yuan-Gui Huang, *The Institute of Neurosciences, Fourth Military Medical University, Xi'an 710032, People's Republic of China
1 Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996; 347: 921-25.
2 O'Brien C. Mad cow disease: scant data cause widespread concern. Science 1996; 271: 1798.
3 Luo Y, Huang KW. Spongy degeneration of the CNS in infancy. Arch Neurol 1984; 41: 164-70.
4 Guo YP, Masters CL, Feng YK, et al. Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease): a report of 10 cases with clinicopathological study. Chin J Neurol 1989; 22: 289-93.
5 Lin SH, Zhao JX, Jiang XM, et al. Clinicopathological, immunohistochemical and transmission studies of Creutzfeldt-Jakob disease. Chin J Neurol 1996; 29: 29-32.
ZR 5
MH China/epidemiology; Human; Prion Diseases/diagnosis/*epidemiology
SP englisch
PO England