[1]郭方亮,胡社静,李涛.散发型克雅病7例患者的临床、脑电图及影像学分析[J].卒中与神经疾病杂志,2017,24(03):217-222.[doi:10.3969/j.issn.1007-0478.2017.03.012]
　Guo Fangliang,Hu Shejing,Li Tao..The study on clinical feature, electroencephalography and MRI in seven cases of sporadic Creutzfeldt-Jakob disease[J].Stroke and Nervous Diseases,2017,24(03):217-222.[doi:10.3969/j.issn.1007-0478.2017.03.012]
点击复制

散发型克雅病7例患者的临床、脑电图及影像学分析()
分享到：

《卒中与神经疾病》杂志[ISSN:1007-0478/CN:42-1402/R]

卷:
第24卷
期数:
2017年03期
页码:
217-222
栏目:
论 著
出版日期:
2017-06-26

文章信息/Info

Title:
The study on clinical feature, electroencephalography and MRI in seven cases of sporadic Creutzfeldt-Jakob disease
文章编号:
1007-0478(2017)03-0217-06
作者:
郭方亮; 胡社静; 李涛
430060 武汉大学人民医院神经内科[郭方亮 胡社静 李涛(通信作者)]
Author(s):
Guo Fangliang;  Hu Shejing;  Li Tao.
Department of Neurology, Renmin Hospital of Wuhan University, Wuhan 430060
关键词:
散发型克雅病 脑电图 磁共振成像 14-3-3蛋白
Keywords:
Sporadic Creutzfeldt-Jakob disease Electroencephalography MRI 14-3-3 protein
分类号:
R742
DOI:
10.3969/j.issn.1007-0478.2017.03.012
摘要:
目的 探讨散发型克雅病(sCJD)的临床、脑电图及影像学特点。方法 回顾性分析7例散发型克雅病患者的临床表现、脑电图、影像学特点。结果 本组亚急性起病5例,慢性起病2例,主要的临床症状和体征有进行性痴呆、精神行为异常、视觉障碍、头晕、共济失调、肌阵挛、言语笨拙、锥体外系症状和锥体束征等; EEG检查均有异常,其中6例脑电图检查示典型的周期性三相波发放,1例患者入院脑电图检查未见异常波发放,1月后复查脑电图发现周期性三相波; 7例均行颅脑MRI检查,T2加权序列(T2WI)、液体衰减反转恢复序列(T2 FLAIR)及弥散加权成像(DWI)在皮质、尾状核、壳核等发现异常高信号,其中1例在DWI像上发现随着疾病进展尾状核、壳核、皮层信号先明显增高,后稍微下降; 6例行脑脊液14-3-3蛋白检测,其中4例为阳性,2例为阴性。结论 临床上对快速进展型痴呆的患者,应考虑克雅病的可能,尽早行脑电图、颅脑MRI以及脑脊液14-3-3蛋白检测有助于临床早期诊断; 脑电图、颅脑MRI在疾病早期可无典型改变,则应短期内复查,动态观察。
Abstract:
ObjectiveTo investigate the clinical feature, electroencephalography(EEG)and MRI of sporadic Creutzfeldt-Jakob disease(sCJD).Methods The clinical feature, EEG and MRI of 7cases of sCJD were analyzed retrospectively.Results 5 cases were subacute onset, 2 cases were chronic onset. The major clinical symptoms and signs were progressive dementia, mental and behavior disorder, visual abnormalities, dizziness, ataxia, myoclonus, speech clumsy, extrapyramidal symptoms and pyramidal sign etc. The EEG results were all abnormal, and 6 cases showed typical periodic triphasic wave. One patient showed no abnormal waves when admitted to hospital, but showed periodic triphasic wave on reexamination of EEG one month later. Head magnetic resonance imaging(MRI)was performed in all cases and showed hyperintense signals in cerebral cortex, caudate nucleus and putamen on T2-weighted image(T2WI), T2 fluid-attenuated inversion recovery image(T2 FLAIR)and diffusion-weighted image(DWI). One patient showed the hyperintense signals in cerebral cortex, caudate nucleus and putamen enhanced first and then attenuated with the progression of the disease. Detection of 14-3-3 protein in cerebrospinal fluid was performed in 6 cases and 4 cases showed positive results.Conclusion Patients with rapidly progressive dementia should be taken into consideration of CJD. Examination with EEG, head MRI and detection of 14-3-3 protein are helpful for establishing the early diagnosis in suspected cases. Sometimes no typical findings of CJD were found on EEG and head MRI in early stage, reexamination of EEG and MRI should be performed in a short time.

参考文献/References:

[1] Steinhoff BJ,Zerr I,Glatting M,et al.Diagnostic value of periodic complexes in Creutzfeldt-Jakob disease[J].Ann Neurol,2004,56(5):702-708.
[2] Tschampa HJ,Kallenberg K,Urbach H,et al.MRI in the diagnosis of sporadic Creutzfeldt-Jakob disease: a study on inter-observer agreement[J].Brain,2005,128(Pt 9):2026-2033.
[3] Ladogana A,Puopolo M,Croes EA,et al.Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada[J].Neurology,2005,64(9):1586-1591.
[4] Linsell L,Cousens SN,Smith PG,et al.A case-control study of sporadic Creutzfeldt-Jakob disease in the United Kingdom: analysis of clustering[J].Neurology,2004,63(11):2077-2083.
[5] Brown P,Gibbs CJ,Rodgers-Johnson P,et al.Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease[J].Ann Neurol,1994,35(5):513-529.
[6] Will RG,Matthews WB.A retrospective study of Creutzfeldt-Jakob disease in England and Wales 1970-79. I: Clinical features[J].J Neurol Neurosurg Psychiatry,1984,47(2):134-140.
[7] Johnson RT,Gibbs CJ.Creutzfeldt-Jakob disease and related transmissible spongiform encephalopathies[J].N Engl J Med,1998,339(27):1994-2004.
[8] Johnson RT.Prion diseases[J].Lancet Neurol,2005,4(10):635-642.
[9] 黄勋,范学工.克雅病研究进展[J].临床内科杂志,2010,27(5):293-295.
[10] Newey CR,Sarwal A,Wisco D,et al.Variability in diagnosing Creutzfeldt-Jakob disease using standard and proposed diagnostic criteria[J].J Neuroimaging,2013,23(1):58-63.
[11] Young GS,Geschwind MD,Fischbein NJ,et al.Diffusion-weighted and fluid-attenuated inversion recovery imaging in Creutzfeldt-Jakob disease: high sensitivity and specificity for diagnosis[J].AJNR Am J Neuroradiol,2005,26(6):1551-1562.
[12] Fragoso DC,Gon alves Filho AL,Pacheco FT,et al.Imaging of Creutzfeldt-Jakob disease: imaging patterns and their differential diagnosis[J].Radiographics,2017,37(1):234-257.
[13] Collie DA,Summers DM,Sellar RJ,et al.Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: Mr imaging findings in 86 neuropathologically confirmed cases[J].AJNR Am J Neuroradiol,2003,24(8):1560-1569.
[14] Hamlin C,Puoti G,Berri S,et al.A comparison of tau and 14-3-3 protein in the diagnosis of Creutzfeldt-Jakob disease[J].Neurology,2012,79(6):547-552.
[15] Kim MO,Geschwind MD.Clinical update of Jakob-Creutzfeldt disease[J].Curr Opin Neurol,2015,28(3):302-310.
[16] Manix M,Kalakoti P,Henry M,et al.Creutzfeldt-Jakob disease: updated diagnostic criteria, treatment algorithm, and the utility of brain biopsy[J].Neurosurg Focus,2015,39(5):E2.
[17] Orrú CD,Bongianni M,Tonoli G,et al.A test for Creutzfeldt-Jakob disease using nasal brushings[J].N Engl J Med,2014,371(6):519-529.
[18] Zerr I,Kallenberg K,Summers DM,et al.Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease[J].Brain,2009,132(Pt 10):2659-2668.

更新日期/Last Update: 2017-06-20