CAJ | 학술논문

分析3例CJD的临床、EEG、MRI、CSF中14-3-3蛋白、脑病理组织学及PrP的免疫组化资料。本组CJD发病年龄平均在64岁，均为急性起病，除痴呆和肌阵挛表现明显外，视觉改变及小脑征出现相对较早，而锥体束损害相对不明显。3例EEG显示周期性尖慢复合波，1例脑CT显示底节钙化，3例脑MRI示脑萎缩不明显，1例T2加权像示双侧脑室旁信号增加，额叶及枕叶各1例脑活检证实为海绵状脑病，1例额叶活检的病例PrP免疫组化检查阳性而CSF中14-3-3蛋白检测为阴性，另1例临床诊断为可能CJD而14-3-3检测1次为阴性，1次为可疑。国人CJD存在着以下特点: ①发病年龄相对较早;② 多数为急性起病;③除痴呆和肌阵挛表现明显外，视力损伤和小脑征出现相对较早，而锥体束损害相对不太明显；④脑萎缩不明显;⑤ 脑MRI的改变尚需进一步研究;⑥14-3-3蛋白阴性者也不能排除CJD的诊断。
분석3례CJD적림상、EEG、MRI、CSF중14-3-3단백、뇌병리조직학급PrP적면역조화자료。본조CJD발병년령평균재64세，균위급성기병，제치태화기진련표현명현외，시각개변급소뇌정출현상대교조，이추체속손해상대불명현。3례EEG현시주기성첨만복합파，1례뇌CT현시저절개화，3례뇌MRI시뇌위축불명현，1례T2가권상시쌍측뇌실방신호증가，액협급침협각1례뇌활검증실위해면상뇌병，1례액협활검적병례PrP면역조화검사양성이CSF중14-3-3단백검측위음성，령1례림상진단위가능CJD이14-3-3검측1차위음성，1차위가의。국인CJD존재착이하특점: ①발병년령상대교조;② 다수위급성기병;③제치태화기진련표현명현외，시력손상화소뇌정출현상대교조，이추체속손해상대불태명현；④뇌위축불명현;⑤ 뇌MRI적개변상수진일보연구;⑥14-3-3단백음성자야불능배제CJD적진단。
To study the features of sporadic Creutzfeldt-Jakob Disease(CJD) in China. Three cases of CJD diagnosed by clinical， electroencephalography (EEG)， MRI，14-3-3 brain protein in cerebrospinal fluid (CSF)，pathology and immunocytochemistry prion protein (PrP) were analysed. The average age of the onset of CJD was sixty four years.The onset was acute in all three cases. Visual and cerebellar signs appeared comparatively early besides mental and myoclonic symptoms，but signs of pyramidal damage were not obvious relatively. In all the three cases EEG showed periodic sharp wave complexes. Calcification of pallidumes was showed by CT in one case. Brain atrophy was not so obvious in three cases on MRI. One case showed increased signal surrounding both lateral ventricles of the brain on T2-weighted images. Biopsy，narked changes in the frontal lobe in one case,and in the occiptal lobe in another case were observed，with disappearance of nerve cells and extensive astroglial proliferation and spongiform state. Anti-PrP immunocytochemistry had detected PrP deposits in the one biopsy，but the 14-3-3 brain protein was negative in CSF. The diagnosis of CJD was made clinically, but the 14-3-3 brain protein was suspicious one time and negative in another test. It suggested that CJD in Chinese patients may consist of the following features: 1. The onset was comparatively early in age. 2. The onset was acute. 3. Visual and cerebellar signs appeared comparatively early besides mental and myoclonic changes，but signs of pyramidal damage were not obvious relatively. 4. Brain atrophy was not remarkable. 5. Farther study with MRI should be carried out. 6. Negative 14-3-3 brain protein test could not rule out CJD.