The Early Presentation of Creutzfeldt-Jacob Disease: Two Cases Report in Middle Taiwan

庫賈氏症的早期表現：中台灣兩病例報告

莊介森(Chieh-Sen Chuang) ； 巫錫霖(Shey-Lin Wu) ； 羅敏智(Min-Chih Lo) ； 沈銘鏡(Ming-Ching Shen) ； 劉尊榮(Tsun-Jung Liu)

《內科學誌》 19卷1期 (2008/02) Pp. 78-82

https://doi.org/10.6314/JIMT.2008.19(1).12

摘要

庫賈氏症是常見的傳染性人類海綿樣腦病變，臨床的表現爲進展快速的智能退化而成爲植物人狀態。我們在此報告兩例在早期斷的庫賈氏症個案，這兩位個案在初期皆有食慾不振、健忘、人格改變、失眼、頭暈等非典型的症狀，之後表現視覺障礙、肌震顫、運動失調、口齒不清的神經學表現。磁振造影除了腦部萎縮之外在擴散影像可見到大腦皮質有彩帶樣高信號表現，而腦波在初期是瀰漫性慢波，但中後期則可見癲癇樣放電的銳波，兩位個案在發病後約四到六個月爲完全失能的狀態。庫賈氏症初期症狀不典型，但發病後進展快速而且又具有傳染性，因此早期的診斷對患者本身、家屬、醫護人員或看護者皆有很重要的意義。

並列摘要

BACKGROUND: Creutzfeldt-Jacob disease (CJD) is the most common transmissible human spongiform encephalopathy. We describe the early presentation of clinical course and special diagnostic findings of two sporadic CJD in middle Taiwan. CASES: Both patients presented with non-specific symptoms such as poor appetite, forgetful, change in personality, sleep disorder, and dizziness before seeking medical advice. Afterwards the two patients had impairment of multiple neurologic symptoms such as visual disorder, myoclonic jerks, ataxia and dysarthria. Magnetic resonance image (MRI) of brain in diffusion-weighted image (DWI) showed ribbon-like area involving cerebral cortex in the early stage. Electroencephalography revealed lateralized epileptiform discharges. Deterioration in their clinical condition was progressing to a dependent state within 4 to 6 months. The treatment remains palliative including anticonvulsant for myoclonic jerks and rehabilitation. CONCLUSION: The early features of CJD can be non-specific. The brain image findings and clinical presenting attributes of patients provide diagnostic indications. Early diagnosis is important for patients, family and healthcare providers.

並列關鍵字

Creutzfeldt-Jacob disease ； CJD ； Prion disease ； Diffusion-weighted image of MRI