MRI lesion profiles in sporadic Creutzfeldt-Jakob disease
MRI lesion profiles in sporadic Creutzfeldt-Jakob disease
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DOI：
10.1212/wnl.0b013e3181a96e5d
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发表时间：
2009-06-09
期刊：
影响因子：
9.9
通讯作者：
Zerr, I.
中科院分区：
文献类型：
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作者：
Meissner, B.;Kallenberg, K.;Zerr, I.
背景：对于散发性克雅氏病 (sCJD)，已描述了六种分子亚型（MM1、MM2、MV1、MV2、VV1 和 VV2），这些亚型在发病年龄、病程、早期症状和神经病理学方面有所不同。据报道，MRI 信号改变与不同的克雅氏病 (CJD) 亚型相关。这项多中心、国际研究旨在描述与每种 sCJD 分子亚型相关的脑部 MRI 结果。 方法：在 7 个国家收集了经病理学证实的具有密码子 129 基因型（MM、MV 和 VV）、PrPSc 型以及液体衰减反转恢复 (FLAIR) 或扩散加权成像 (DWI) 的 sCJD 病例。根据涵盖七个皮质区域、基底神经节、丘脑和小脑的标准方案评估所有 MRI 扫描的信号变化。结果：对 211 名克雅氏病患者（98 MM1、23 MM2、19 MV1、30 MV2、9 VV1 和 32 VV2）的 MRI 扫描进行了评估。基底神经节高信号最常见于 MV2、VV2 和 MM1 亚型（79%、77% 和 70%）。广泛的大脑皮层信号增加在 VV1、MM2 和 MV1 亚型中最常见（86%、77% 和 77%）。丘脑高信号最常发生在 VV2 (45%) 和 MV2 (43%)。大多数亚型中最一致的发现是基底神经节中的高信号，63% (FLAIR) 和 71% (DWI) 中发现这些异常。结论：在所有分子散发性克雅氏病亚型中，MRI 均检测到基底神经节和丘脑的皮质信号增加和高信号。我们的研究结果表明，每种分子亚型都可能出现特征性 MRI 病变模式。神经病学（R）2009； 72：1994-2001
Background: With respect to sporadic Creutzfeldt-Jakob disease (sCJD), six molecular subtypes (MM1, MM2, MV1, MV2, VV1, and VV2) have been described, which vary with respect to age at disease onset, disease duration, early symptoms, and neuropathology. MRI signal alterations were reported to correlate with distinct Creutzfeldt-Jakob disease (CJD) subtypes. This multicenter, international study aimed to describe the brain MRI findings associated with each of the sCJD molecular subtypes.Methods: Pathologically confirmed sCJD cases with codon 129 genotype (MM, MV, and VV), PrPSc type, and fluid-attenuated inversion recovery (FLAIR) or diffusion-weighted imaging (DWI) were collected in seven countries. All MRI scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus, and cerebellum.Results: MRI scans were evaluated in 211 CJD patients (98 MM1, 23 MM2, 19 MV1, 30 MV2, 9 VV1, and 32 VV2). Basal ganglia hyperintensities occurred most frequently in MV2, VV2, and MM1 subtypes (79, 77, and 70%). Wide cerebral cortical signal increase was most common in VV1, MM2, and MV1 subtypes (86, 77, and 77%). Thalamic hyperintensities occurred most often in VV2 (45%) and MV2 (43%). The most consistent finding across most subtypes was high signal in basal ganglia, with these abnormalities found in 63% (FLAIR) and 71% (DWI).Conclusion: Cortical signal increase and hyperintensities in the basal ganglia and thalamus are detected by MRI across all molecular sporadic Creutzfeldt-Jakob disease subtypes. Our findings argue that characteristic MRI lesion patterns may occur for each molecular subtype. Neurology (R) 2009; 72: 1994-2001