Department of Psychiatry, Mount Sinai School of Medicine, New York, New York 10029, USA.
AJNR Am J Neuroradiol. 2012 Nov;33(10):1945-50. doi: 10.3174/ajnr.A3125. Epub 2012 May 10.
Human prion diseases are known to cause gray matter degeneration in specific cerebral structures, but evidence for white matter involvement is scarce. We used DTI to test the hypothesis that white matter integrity is disrupted in human CJD during the early stages of the disease.
Twenty-one patients with the E200K variant of CJD and 19 controls participated in DTI studies conducted on a 1.5T MR imaging scanner. The data were quantitatively analyzed and mapped with a voxelwise TBSS method.
We found significant reductions of FA in patients with CJD in distinct and functionally relevant white matter pathways, including the corticospinal tract, internal capsule, external capsule, fornix, and posterior thalamic radiation. Moreover, these FA deficits increased with disease duration, and were mainly determined by increase of radial diffusivity, suggesting elevated permeability of axonal membranes.
The findings suggest that some of the symptoms of CJD may be caused by a functional dysconnection syndrome, and that the leukoencephalopathy is progressive and detectable fairly early in the course of the disease.
已知人类朊病毒病可导致特定脑结构的灰质变性，但脑白质受累的证据很少。我们使用 DTI 来检验假设，即在疾病的早期，人类 CJD 患者的脑白质完整性受到破坏。
21 例 E200K 变异型 CJD 患者和 19 例对照者参与了在 1.5T MR 成像扫描仪上进行的 DTI 研究。对数据进行了定量分析，并使用体素 TBSS 方法进行了映射。
我们发现 CJD 患者在特定的、功能相关的白质通路中存在 FA 值明显降低，包括皮质脊髓束、内囊、外囊、穹窿和丘脑后辐射。此外，这些 FA 缺陷随疾病持续时间而增加，主要由径向弥散度的增加决定，提示轴突膜通透性增加。
这些发现表明，CJD 的一些症状可能是由功能连接综合征引起的，而且脑白质病变是进行性的，在疾病过程中相当早就能检测到。
