Creutzfeldt-Jakob 病(Creutzfeldt-Jakob diseae,CJD)又称皮质-纹状体-脊髓变形,是指由朊蛋白(prion protein PrP)感染而表现为精神障碍、痴呆、帕金森样表现、共济失调、肌阵挛、肌肉萎缩等的慢性或亚急性、进展性疾病。
DWI比常规MRI敏感,常可以在CJD早期特异性地表现为沿皮层走行的带状高信号(飘带征)和(或)双侧基地核区异常信号,该表现比EEG甚至比临床痴呆症状和肌阵挛更早(约发病后1个月)。
Young等报道40例CJD患者中灰质异常最常见于新皮质(89%),其次见于边缘系统皮质(79%),纹状体(69%)以及丘脑(34%)。而在新皮质异常信号中最常见于额叶(84%),其次是顶叶(72%),颞叶(65%)。
Young等还提出了对CJD的诊断分级标准:
肯定CJD:
a.单侧或双侧纹状体异常信号,至少1处皮质彩带样高信号,T1WI正常。
b.广泛的大脑皮质彩带样高信号(至少3处)相应的皮质下白质正常,T1WI正常。
可能CJD:
a.FLAIR序列异常而DWI正常。
b.FLAIR及DWI均异常但病变范围局限。
c.单侧或双侧丘脑后内侧异常而皮质无异常。
可能不是CJD:
a.灰质异常信号轻微,位于岛叶、扣带回。
b.异常信号可能为伪影。
肯定不是CJD:
影像学表现正常或异常影像学表现与CJD不符。
沈天真,陈荣星.神经影像学.1版.上海.上海科学技术出版社.2005,420-421。
高波,吕翠.神经系统疾病影像诊断流程.1版.北京.人民卫生出版社.2014,204-206。
Creutzfeldt Jakob disease also known as cortico striato spinal deformation, refers to by the prion protein (PrP)infection and for mental disorders, dementia, Parkinson's appearance, ataxia, myoclonus, muscle atrophy and other chronic or subacute and progress of the disease.
DWI than conventional MRI sensitive, often can be in CJD early specific to walk along the cortex of banded high signal (streamers sign) and (or) of abnormal signals in bilateral basal ganglia and the performance than EEG and compared to the clinical symptoms of dementia, myoclonus earlier (about after the onset of a month).
Gray matter abnormalities in 40 patients with CJD were most commonly found in the new cortex (89%), followed by limbic system cortex (79%), striatum (69%), and thalamus (34%). In the new cortical abnormalities, the most common in the frontal lobe (84%), followed by the parietal lobe (72%), the temporal lobe (65%).
Young and other criteria for the diagnosis and classification of CJD are also presented:
Sure CJD:
A. unilateral or bilateral striatal abnormal signal, at least 1 cortical ribbon like high signal, T1WI normal.
B.'s extensive cortical ribbon like high signal (at least 3) corresponding to the subcortical white matter in normal, normal T1WI.
May CJD:
A.FLAIR sequence anomalies and DWI normal.
Both b.FLAIR and DWI were abnormal but the lesion range was limited.
C. unilateral or bilateral thalamus, the medial abnormalities and cortical abnormalities.
May not be CJD:
A. gray matter anomaly signal is slight, is located in the island leaf, the buckle to bring back.
B. abnormal signal may be artifact.
Certainly not CJD:
Imaging performance is normal or abnormal imaging performance and CJD does not match.
Shen Tianzhen, Chen Rongxing. Neuroimaging.1. Shanghai. Shanghai science and Technology Publishing House,.2005,420-421。
Gao Bo, Lu Cui. Image diagnosis process of nervous system diseases.1 ed. Beijing. People's Medical Publishing House,.2014,204-206。