MRI Detection of the Cerebellar Syndrome in Creutzfeldt-Jakob Disease
MRI Detection of the Cerebellar Syndrome in Creutzfeldt-Jakob Disease
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DOI：
10.1007/s12311-009-0106-8
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发表时间：
2009-09-01
期刊：
影响因子：
3.5
通讯作者：
Prohovnik, Isak
中科院分区：
文献类型：
--
作者：
Cohen, Oren S.;Hoffmann, Chen;Prohovnik, Isak
克雅氏病（Creutzfeldt-Jakob Disease，CJD）的特征是T2 W和弥散加权成像（DWI）磁共振成像（MRI）扫描上双侧基底节高信号，与其锥体外系神经系统表现一致。MRI对小脑症状的诊断无意义，在CJD中同样突出。这项研究旨在解释这一明显的悖论。从一项大型前瞻性研究中选择了11名具有明确小脑或脑干症状的CJD患者，以及11名年龄和性别匹配的健康对照。所有受试者均参加标准化MRI方案，包括SPGR、液体衰减反转恢复（FLAIR）、DWI和扩散张量成像（DTI）。所有受试者都接受了由对放射学结果不知情的神经科医生进行的详细检查，该医生预测了脑异常的预期部位。MRI显示对皮质（80-90%）和基底节（100%）异常预测的敏感性良好。标准MRI序列，包括DWI、DTI和FLAIR，均未显示小脑或脑干的任何组织异常。表观扩散系数（ADC）值，然而，在几个小脑结构，体积（VBM）分析也显示脑脊液体积升高，这表明在这些CJD患者局灶性小脑萎缩显着升高。在CJD患者中，DWI对皮质和基底节的扩散率降低敏感，但对小脑受累不敏感。我们认为CJD小脑病理学的放射学标志是萎缩，通过VBM和弥散度升高定量显示，这在ADC图上是可以识别的，但在非定量DWI图像中难以观察到。
Creutzfeldt-Jakob Disease (CJD) is characterized by bilateral basal ganglia hyperintensities on T2W and diffusion-weighted imaging (DWI) magnetic resonance imaging (MRI) scans, consistent with its extrapyramidal neurological manifestations. MRI is diagnostically uninformative about the cerebellar symptoms, equally prominent in CJD. This study was undertaken to explain this apparent paradox. Eleven CJD patients with definite cerebellar or brain stem symptoms were selected from a large prospective study, as well as 11 healthy controls matched for age and gender. All subjects participated in a standardized MRI protocol, including SPGR, fluid-attenuated inversion recovery (FLAIR), DWI and diffusion tensor imaging (DTI). All subjects underwent detailed examination by a neurologist blinded to the radiological findings, who predicted the expected site of cerebral abnormalities. MRI showed good sensitivity for the abnormalities predicted in the cortex (80-90%) and basal ganglia (100%). None of the standard MRI sequences, including DWI, DTI, and FLAIR, revealed any tissue abnormalities in cerebellum or brain stem. Apparent diffusion coefficient (ADC) values, however, were substantially and significantly elevated in several cerebellar structures, where also the volumetric (VBM) analysis revealed elevated cerebrospinal fluid volume, suggesting focal cerebellar atrophy in these CJD patients. In patients with CJD, DWI appears sensitive to the reduced diffusivity in cortex and basal ganglia but insensitive to cerebellar involvement. We propose that the radiological hallmark of cerebellar pathology in CJD is atrophy, revealed quantitatively by both VBM and elevated diffusivity, which is identifiable on ADC maps but poorly visualized in nonquantitative DWI images.