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Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disease of unknown cause. Patients are usually aged between 50 and 75 and typical clinical features include a rapidly progressive dementia associated, myoclonus and a characteristic electroencephalographic pattern. Neuropathological examination reveals cortical spongiform change, hence the term `spongiform encephalopathy'.
H.G. Creutzfeldt is credited with the first description of the disorder in 1920, although by current diagnostic criteria his case would be highly atypical. A year later another German neurologist, A. Jakob, described four cases, at least two of whom had clinical features suggestive of the entity we recognise as CJD.
Although CJD appears to occur as a predominantly sporadic disorder it can also occur as a dominantly inherited or infective condition. The latter mode of transmission was first elucidated during the study of kuru in the 1950's. This neurodegenerative condition occurs only in the people of the Fore region of Papua New Guinea and is thought to have resulted from the consumption of brains during endocannabalistic funeral rituals. The similarities between kuru and scrapie, the transmissible spongiform encephalopathy of sheep, prompted a veterinary neuropathologist, Hadlow, to suggest that transmission studies of kuru be performed. The success of those studies and the recognition that the neuropathological changes in kuru were similar to those of CJD, was followed by the transmission of CJD to the chimpanzee by intracerebral inoculation of brain tissue. In 1974 a case of iatrogenic CJD due to corneal transplantation occurred and subsequently contaminated neuro-surgical instruments, dural grafts, and brain depth electrodes have all been recognised as transmitting the disease. In 1985 the first case was reported in a recipient of contaminated human derived growth hormone and subsequently over 60 similar cases have arisen world-wide in addition to 4 cases associated with human derived gonadotrophin. The familial occurrence of CJD has been recognised for many years but was unexplained. The discovery of linkage to a region on the short arm of chromosome 20 has led recently to the elucidation of various dominantly inherited mutations.
[此贴子已经被作者于2004-10-5 21:31:37编辑过]
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