Regional and progressive thinning of the cortical ribbon in Huntington's disease
Regional and progressive thinning of the cortical ribbon in Huntington's disease
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DOI：
10.1212/wnl.58.5.695
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发表时间：
2002-03-12
期刊：
影响因子：
9.9
通讯作者：
Fischl, B
中科院分区：
文献类型：
--
作者：
Rosas, HD;Liu, AK;Fischl, B
背景资料：亨廷顿氏病（HD）是一种致命的进行性神经退行性疾病，伴有不自主运动、认知功能障碍和精神症状。虽然进行性纹状体变性是已知的发生，但很少有人知道疾病如何影响皮质，包括哪些皮质区域受到影响，变性如何进行，以及皮质变性与临床症状的关系。皮质一直很难在神经退行性疾病的研究，主要是因为其复杂的折叠模式和区域变异性，然而，了解皮质是如何受到疾病的影响，可能会提供重要的新见解it.Methods：新的自动表面重建和高分辨率的MR图像的11例HD和13个年龄匹配的主题被用来获得皮质厚度测量。同样的分析进行了两个死后的大脑，以验证这些方法。结果：HD患者的皮质带存在区域特异性的不均匀变薄。变薄发生在早期，在不同临床阶段的疾病患者之间存在差异，并且似乎随着疾病进展从后部皮质区域向前部皮质区域进行。感觉运动区在统计学上受影响最大。对尸检大脑的MR图像进行类似分析的测量结果与使用传统神经病理学方法获得的测量结果在0.25 mm范围内，并且在统计学上无法区分。结论：作者提出，皮质在疾病早期退化，区域选择性皮质退化可以解释HD临床表达的异质性。这些措施可能会提供一个敏感的前瞻性替代指标的神经保护药物的临床试验。
Background: Huntington's disease (HD) is a fatal and progressive neurodegenerative disease that is accompanied by involuntary movements, cognitive dysfunction, and psychiatric symptoms. Although progressive striatal degeneration is known to occur, little is known about how the disease affects the cortex, including which cortical regions are affected, how degeneration proceeds, and the relationship of the cortical degeneration to clinical symptoms. The cortex has been difficult to study in neurodegenerative diseases primarily because of its complex folding patterns and regional variability; however, an understanding of how the cortex is affected by the disease may provide important new insights into it. Methods: Novel automated surface reconstruction and high-resolution MR images of 11 patients with HD and 13 age-matched subjects were used to obtain cortical thickness measurements. The same analyses were performed on two postmortem brains to validate these methods. Results: Regionally specific heterogeneous thinning of the cortical ribbon was found in subjects with HD. Thinning occurred early, differed among patients in different clinical stages of disease, and appeared to proceed from posterior to anterior cortical regions with disease progression. The sensorimotor region was statistically most affected. Measurements performed on MR images of autopsy brains analyzed similarly were within 0.25 mm of those obtained using traditional neuropathologic methods and were statistically indistinguishable. Conclusions: The authors propose that the cortex degenerates early in disease and that regionally selective cortical degeneration may explain the heterogeneity of clinical expression in HD. These measures might provide a sensitive prospective surrogate marker for clinical trials of neuroprotective medications.