- 作者：E. Arranz-Martí ; nez ; ezequielarranz@gmail.com ; G. Trillo-Sá ; nchez-Redondo ; A. Ruiz-Garcí ; a ; S. Ares-Blanco
- 关键词：Enfermedades por priones ; Sí ; ndrome Creutzfeldt-Jakob ; Kuru ; Insomnio familiar letal ; Sí ; ndrome Gerstmann-Strä ; ussler-Scheinker
- 刊名：SEMERGEN - Medicina de Familia
- 出版年：2010
- 出版时间：October 2010
- 年：2010
- 卷：36
- 期：8
- 页码：443-448
- 全文大小：290 K
Prion diseases or prion encephalopathies are a group of rare disorders that share a similar pathophysiology with different clinical characteristics. Sporadic Creutzfeldt-Jakob disease (CJD) is best known. It presents as a rapidly progressive dementia, ataxia and extrapyramidal symptoms. Although accurate diagnosis is by histopathological examination, a diagnosis can be probably achieved using the criteria established by the World Health Organization. We are currently discussing the possibility of integrating the nuclear magnetic resonance into this criteria for increasing the sensitivity of diagnosis. Molecular techniques for studying proteins in cerebrospinal fluid have an increasingly significant role in aiding diagnosis. The diagnosis of other prion encephalopathies is not as advanced as that of CJD. The treatment of these diseases remains palliative.