Mezzapesa D M, Ceccarelli A, Dicuonzo F, Carella A, De Caro M F, Lopez M, Samarelli V, Livrea P, Simone I L
Department of Neurological and Psychiatric Sciences, University of Bari, Bari, Italy.
AJNR Am J Neuroradiol. 2007 Feb;28(2):255-9.
Recent evidence from neuropsychologic and neuroimaging studies suggests that central nervous system involvement in amyotrophic lateral sclerosis (ALS) extends beyond motor neurons. Our purpose was to obtain measures of global and regional atrophy in nondemented patients with ALS to assess subtle structural brain changes.
MR images, acquired from 16 patients and 9 healthy subjects (HS), were processed by using the Structural Imaging Evaluation of Normalized Atrophy (SIENA) software to estimate whole-brain atrophy measures and the voxel-based morphometry (VBM) method to highlight the selective volumetric decrease of single cerebral areas. In addition, each subject underwent a neuropsychologic examination.
In patients with ALS, brain parenchymal fraction was slightly lower compared with HS (P = .012), and seemed to be related to the presence of cognitive impairment. Patients showed a gray matter volume decrease in several frontal and temporal areas bilaterally (P < .001 uncorrected) compared with HS, with a slight prevalence in the right hemisphere. No volume reduction in primary motor cortices of patients was detected. Performances on Symbol Digit Modalities Test were significantly worse in patients compared with HS (P = .025).
The presence of mild whole-brain volume loss and regional frontotemporal atrophy in patients with ALS could explain the presence of cognitive impairment and confirms the idea of ALS as a degenerative brain disease not confined to motor system.
神经心理学和神经影像学研究的最新证据表明，肌萎缩侧索硬化症（ALS）中中枢神经系统的受累范围超出运动神经元。我们的目的是获取非痴呆型ALS患者的全脑和局部萎缩测量值，以评估脑部细微的结构变化。
对16例患者和9名健康受试者（HS）的磁共振成像（MR）图像进行处理，使用标准化萎缩结构成像（SIENA）软件估计全脑萎缩测量值，并采用基于体素的形态学测量（VBM）方法突出单个脑区的选择性体积减小。此外，每位受试者均接受神经心理学检查。
与HS相比，ALS患者的脑实质分数略低（P = 0.012），且似乎与认知障碍的存在有关。与HS相比，患者双侧多个额叶和颞叶区域的灰质体积减小（未校正P < 0.001），右侧半球略有优势。未检测到患者初级运动皮层的体积减小。与HS相比，患者在符号数字模态测试中的表现明显更差（P = 0.025）。
ALS患者存在轻度全脑体积丢失和局部额颞叶萎缩，这可以解释认知障碍的存在，并证实了ALS是一种不限于运动系统的退行性脑病的观点。
