ADC mapping of neuro degeneration in the brainstem and cerebellum of patients with progressive ataxias
ADC mapping of neuro degeneration in the brainstem and cerebellum of patients with progressive ataxias
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DOI：
10.1016/j.neuroimage.2004.01.035
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发表时间：
2004-06-01
期刊：
影响因子：
5.7
通讯作者：
Mascalchi, M
中科院分区：
文献类型：
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作者：
Della Nave, R;Foresti, S;Mascalchi, M
从弥散加权磁共振成像中获得的表观弥散系数（ADC）图分析正在成为一种可重复的、灵敏的和定量的工具，用于评估白色和灰质疾病的脑损伤。为了探索ADC图分析在退行性共济失调中的潜力，我们检查了28例患者和26例年龄匹配的对照者的T1、T2和弥散（沿三个主要身体轴的B值0-1000沿着）加权MR图像。24例患者有遗传性疾病，包括脊髓小脑性共济失调1型（SCA 1）（n = 9），脊髓小脑性共济失调2型（SCA 2）（n = 8），和弗里德赖希共济失调（EA）（n = 7），而4例患者有散发性成人发作的纯小脑性共济失调（3例特发性，1例谷蛋白不耐受）。后颅窝（PCF）中所含CNS结构的面积和线性测量初步将患者分为三个形态学类别，反映了大体病理学结果，即橄榄脑桥小脑萎缩（OPCA）（n = 10：6例SCA 2和4例SCA 1）、脊髓萎缩（SA）（n = 7：所有FA）和小脑皮质萎缩（CCA）（n = 4：3例特发性和1例谷蛋白不耐受）。7例SCA 1（n = 5）或SCA 2（n = 2）患者的形态学变化令人联想到OPCA，但其值仍在正常范围下限内，并被归类为未定义。生成整个大脑的平均扩散率（D）图，并测量髓质、脑桥、小脑中脚和齿状白色物质中的感兴趣区域（ROI）的D。此外，排除后的头骨与手动分割和CSF的应用程序的阈值，直方图获得D的脑干和小脑和15个大脑半球。与对照组相比，在OPCA和未定义的患者组中，观察到延髓、小脑中脚和齿周白色质中的D增加（P < 0.001），这些患者组的脑干和小脑D直方图中第25和第50百分位数的值也显着增加。在CCA中（P = 0.01），在脑干和小脑直方图中观察到h值的第25和第50百分位数增加。SA组仅髓质D增加（P < 0.001）。在SCA 1或SCA 2患者中观察到遗传性共济失调临床评定量表（IACRS）评估的临床严重程度与脑干和小脑直方图中D值的第50百分位数（r = 0.69）之间的相关性。弥散磁共振成像显示，在退行性共济失调中，脑干、小脑和大脑半球的D增加的变化模式与已知的神经病理学变化分布相匹配。(C)2004爱思唯尔公司All rights reserved.
Analysis of the apparent diffusion coefficient (ADC) maps derived from diffusion-weighted MR imaging is emerging as a reproducible, sensitive, and quantitative tool to evaluate brain damage in diseases of the white and gray matter. To explore the potentials of ADC maps analysis in degenerative ataxias, we examined 28 patients and 26 age-matched controls with T1, T2, and diffusion (b values 0-1000 along the three main body axes)-weighted MR images. Twenty-four patients had inherited genetically proven diseases including spinocerebellar ataxia type 1 (SCA1) (n = 9), spinocerebellar ataxia type 2 (SCA2) (n = 8), and Friedreich's ataxia (EA) (n = 7), whereas four patients had sporadic adult onset pure cerebellar ataxia (three idiopathic, one gluten intolerance). Area and linear measurements of the CNS structures contained in the posterior cranial fossa (PCF) preliminary enabled classification of the patients in the three morphological categories reflecting the gross pathology findings, namely olivopontocerebellar atrophy (OPCA) (n = 10: six SCA2 and four SCA1), spinal atrophy (SA) (n = 7: all FA), and cortical cerebellar atrophy (CCA) (n = 4: three idiopathic and one gluten intolerance). Seven patients with SCA1 (n = 5) or SCA2 (n = 2) had morphologic changes reminiscent of OPCA, but their values were still in the lower normal range and were classified as undefined. Mean diffusivity (D) maps of the entire brain were generated and D was measured with regions of interest (ROI) in the medulla, pons, middle cerebellar peduncles, and the peridentate white matter. Moreover, after exclusion of the skull with manual segmentation and of the CSF with application of a threshold value, histograms were obtained for D of the brainstem and cerebellum and for 15 of the cerebral hemispheres. As compared to controls, a (P < 0.001) increase of D was observed in the medulla, middle cerebellar peduncles, and peridentate white matter in OPCA and undefined patients groups who had also significantly increased values of the 25th and 50th percentiles in the brainstem and cerebellum D histogram. In CCA (P = 0.01), an increase of the 25th and 50th percentile of the h value was observed in the brainstem and cerebellum histograms. The SA group showed (P < 0.001) an increased D in the medulla only. A correlation between clinical severity as assessed with the Inherited Ataxias Clinical Rating Scale (IACRS) and the 50th percentile of the D value in the brainstem and cerebellum histogram (r = 0.69) was observed in patients with SCA1 or SCA2. Diffusion MR imaging reveals variable patterns of increase of D in the brainstem, cerebellum, and cerebral hemispheres in degenerative ataxias that match the known distribution of the neuropathological changes. (C) 2004 Elsevier Inc. All rights reserved.