慢性進(jìn)展性散發(fā)型克-雅病一例

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摘要：散發(fā)型克-雅?。╯CJD）是一種由朊病毒導(dǎo)致的中樞神經(jīng)系統(tǒng)退行性疾病，典型的臨床表現(xiàn)為快速進(jìn)展性癡呆，大部分患者病程小于1年，僅2%～3%患者大于2年?，F(xiàn)報(bào)道1例以言語(yǔ)表達(dá)障礙起病、進(jìn)展緩慢的sCJD病例，總結(jié)其臨床表現(xiàn)、腦電圖特點(diǎn)、影像及病理特征，以加強(qiáng)對(duì)慢性進(jìn)展性sCJD的認(rèn)識(shí)。

關(guān)鍵詞：散發(fā)型克-雅??；朊蛋白；腦活檢

中圖分類號(hào)： R742  文獻(xiàn)標(biāo)志碼： B  文章編號(hào)：1000-503X（2023）05-0859-04

DOI：10.3881/j.issn.1000-503X.15280

Sporadic Creutzfeldt-Jakob Disease With Slow Progression：Report of One Case

ZHAO Jiahua1，WU Lei1，JIN Wei2，GUI Qiuping2，ZHANG Jiatang1，HUANG Dehui1

1Department of Neurology，2Department of Pathology，The First Medical Centre of Chinese PLA General Hospital，Beijing 100853，China

Corresponding author：WU Lei  Tel：010-55499336，E-mail：[email protected]

ABSTRACT：Sporadic Creutzfeldt-Jakob disease（sCJD）is a prion-caused degenerative disease of the central nervous system，with the typical clinical manifestation of rapidly progressive dementia.The course of disease is less than 1 year in most patients and more than 2 years in only 2% to 3% patients.We reported a case of sCJD with expressive language disorder and slow progression in this paper.By summarizing the clinical manifestations and the electroencephalograhpy，MRI，and pathological features，we aimed to enrich the knowledge about the sCJD with slow progression.

Key words：sporadic Creutzfeldt-Jakob disease；prion；brain biopsy

Acta Acad Med Sin，2023，45（5）：859-862

克-雅?。–reutzfeldt-Jakob disease，CJD）又稱亞急性海綿狀腦病，目前認(rèn)為其病因可能是由于內(nèi)源性朊病毒蛋白基因（prion protein gene，PRNP）突變或外源性朊病毒感染致使自身編碼的細(xì)胞朊粒蛋白（cellular prion protein，PrPc）結(jié)構(gòu)發(fā)生改變，在腦內(nèi)積聚形成具有潛在神經(jīng)毒性的羊瘙癢病朊粒蛋白（scrapie prion protein，PrPsc），破壞神經(jīng)元和神經(jīng)膠質(zhì)細(xì)胞，最終導(dǎo)致腦組織海綿狀變性［1-2］。（剩余5826字）