查詢結果分析
來源資料
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題 名 | Creutzfeldt-Jakob Disease: Clinical Analysis of Eight Cases=Creutzfeldt-Jakob氏症:八個病例之臨床分析 |
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作 者 | 謝明哲; 王寶玉; 賈力耕; 高嘉鴻; 文美卿; | 書刊名 | 中華醫學雜誌 |
卷 期 | 57:4 1996.04[民85.04] |
頁 次 | 頁284-288 |
分類號 | 415.83 |
關鍵詞 | Creutzfeldt-Jakob氏症; Prion疾病; Creutzfeldt-jakob disease; Prion disease; |
語 文 | 英文(English) |
中文摘要 | 背景:Creutzfeldt-Jakob氏症,是一種罕見的,致命性中樞神經系統海棉狀腦部病變。其臨床上之診斷相當具挑戰性,所以我們作此回顧性之研究。 方法:本文針對八位經診斷為Creutzfeldt-Jakob氏症的病患作一回顧性之研究。此病之診斷主要是根據其臨床表現,其中兩例經腦部病理切片證實。 結果:本文包括了四位男性及四位女性,他們的年紀由53歲到81歲不等,平均約66歲。其中六例已經死亡,平均從發病到死亡約6.5個月。其臨床表現相當多樣性,包括失智症,失語症,步伐不穩,錐體路徑病徵,錐體路徑外病徵,肌肉陣發性抽搐及視覺上之問題。 結論:對於Creutzfeldt-Jakob氏症之診斷,臨床上之高度警覺和懷疑非常重要。腦波仍為診斷此病最有價值的工具,假使初期的腦波並不典型,應持續追蹤腦波檢查。單光子放射電腦斷層檢查,可能對於死前之診斷有所幫助,但其敏感性及特異性須進一步研究。腦部病理切片檢查對於已有典型的臨床、腦波及單光子放射電腦斷層表現之病患,似乎並不需要。 |
英文摘要 | Background: Creutzfeldt-Jakob disease (CJD) or spongiform encephalopathy in humans, is a rare, rapidly progressive and fatal disorder of the central nervous system. Since clinical diagnosis is challenging, this retrospective investigation was performed. Methods: Eight cases who had been diagnosed as CJD from 1987 to 1995 were reviewed thoroughly, with a resulting clinical diagnosis of CJD. Two cases were proved by brain biopsy. Results: Four males and four females were included; their ages were 53 to 81 years with a mean of 66 years. The mean illness duration of the six patients who died was six and a half months. Clinical findings were variable and included dementia, aphasia, ataxic gait, pyramidal, extrapyramidal, myoclonus and visual problems. Conclusions: For diagnosis of CJD, clinical suspicion is very important. Electroencephalogram (EEG) remains the most helpful laboratory diagnostic tool; serial recordings are necessary if the initial EEG finding is nonspecific. Single photon emission computed tomography (SPECT) may be helpful for premorten diagnosis of CJD, but sensitivity and specificity need further investigation. Brain biopsy seemed to be unnecessary if the clinical, EEG and SPECT findings are typical. |
本系統中英文摘要資訊取自各篇刊載內容。