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首页> 外文期刊>American Medical Journal >PROBABLE CREUTZFELDT JAKOB DISEASE: CASE REPORT | Science Publications
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PROBABLE CREUTZFELDT JAKOB DISEASE: CASE REPORT | Science Publications

机译：可能的CREUTZFELDT JAKOB病：病例报告|科学出版物

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> Prion diseases are associated with the accumulation of an abnormal isoformof cellular prion protein (PrPSc), which is the principal constituent of prions. Prions replicate in lymphoreticular tissues before neuroinvasion, suggesting that lymphoreticular biopsy samples may allow early diagnosis by detection of PrPSc. Creutzfeldt-Jakob Disease (CJD) is the most frequent prion disease in humans. Clinical diagnosis of sporadic (CJD) is based on the evaluation of rapidly progressive dementia, ataxia, myoclonus, changes on the electroencephalogram and other neurological signs. We report a problable (CJD) case in Santa Barbara surgical center, Rio de Janeiro, Brazil. From our clinical case, we decided to do a brief review about (CJD) researching at MedLine and Pubmed, using terms Creutzfeldt-Jakob disease and Prion diseases. A definite diagnosis, however, is confined to cases that have been evaluated neuropathlogically or by equivalent diagnostic techniques. The range in clinical expression of the disease is better appreciated and the existence of ?atypical? cases of CJD is increasingly recognized. The clinical characteristics, laboratory findings, differential diagnosis, mechanisms of transmission and the actual therapeutic approach are discussed.
机译： > on病毒疾病与细胞病毒的异常同工型（PrP Sc ）的积累有关，后者是病毒的主要成分。 ions病毒在神经入侵之前在淋巴网状组织中复制，这表明淋巴网状活检样本可通过检测PrP Sc 进行早期诊断。克雅氏病（CJD）是人类最常见的病毒病。散发性（CJD）的临床诊断基于对快速进行性痴呆，共济失调，肌阵挛，脑电图变化和其他神经系统症状的评估。我们在巴西里约热内卢的圣塔芭芭拉外科中心报告了一个可疑（CJD）病例。从我们的临床病例中，我们决定使用Creutzfeldt-Jakob病和Prion病来对MedLine和Pubmed的（CJD）研究进行简要回顾。但是，明确的诊断仅限于经过神经病理或等效诊断技术评估的病例。疾病的临床表达范围得到了更好的理解，并且存在“非典型”症状。克雅氏病的案例越来越多。讨论了临床特征，实验室检查结果，鉴别诊断，传播机制和实际治疗方法。

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