Imai Tomohiko, Sakamoto Kenichi, Hasegawa Tatsuji, Shioda Yoko, Tsutsumi Yoshiyuki, Sakaue Satoshi, Imamura Toshihiko, Morimoto Akira, Iehara Tomoko
Department of Pediatrics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, 465 Kajii-Cho, Kawaramachi-Hirokoji, Kamigyo-Ku, Kyoto, 602-8566, Japan.
Departments of Pediatrics, Shiga University of Medical Science, Shiga, Japan.
Neuroradiology. 2024 Jan;66(1):43-54. doi: 10.1007/s00234-023-03249-z. Epub 2023 Nov 20.
To confirm the hypothesis that brain white matter damage is involved in the pathogenesis and disease progression of Langerhans cell histiocytosis (LCH)-associated neurodegenerative disease (ND), we aimed to analyze pediatric patients with LCH using diffusion tensor imaging (DTI).
We enrolled 33 patients with LCH and obtained 33 DTI datasets. Using DTI-based tractography, fractional anisotropy (FA), apparent diffusion coefficient (ADC), axial diffusivity (AD), and radial diffusivity (RD) were measured in the cerebral and cerebellar white matter tracts. The participants were divided into three groups-non-ND, ND without clinical symptoms (r-ND), and ND with clinical symptoms (c-ND)-according to their clinical status during the examination with DTI. We compared the DTI parameters in white matter tracts were compared among the three groups.
In the order of non-ND, r-ND, and c-ND groups, the FA in superior cerebellar peduncle (SCP) and middle cerebellar peduncle (MCP) significantly decreased, the ADC, AD, and RD of MCP, and the RD of SCP were significantly elevated (FA-SCP; p < 0.001, FA-MCP; p = 0.026, ADC-MCP; p < 0.001, AD-MCP; p = 0.002, RD-MCP; p = 0.003, and RD-SCP; p = 0.018). Furthermore, in the simple linear regression analysis, the FA, ADC, AD, and RD values in the MCP and the FA value in the SCP were significantly influenced by the presence of neurological symptoms and ND findings on MRI (all p < 0.001).
In LCH-ND, we identified microstructural damage in the SCP and MCP. DTI parameters in these tracts may help monitor LCH-ND; therefore, future studies are required to validate these results in a large cohort.
为了证实脑白质损伤参与朗格汉斯细胞组织细胞增多症（LCH）相关神经退行性疾病（ND）的发病机制和疾病进展这一假说，我们旨在使用扩散张量成像（DTI）对儿科LCH患者进行分析。
我们纳入了33例LCH患者，并获得了33个DTI数据集。使用基于DTI的纤维束成像技术，在大脑和小脑白质纤维束中测量了分数各向异性（FA）、表观扩散系数（ADC）、轴向扩散率（AD）和径向扩散率（RD）。根据DTI检查时的临床状态，将参与者分为三组：非ND组、无临床症状的ND组（r-ND）和有临床症状的ND组（c-ND）。我们比较了三组之间白质纤维束中的DTI参数。
按非ND组、r-ND组和c-ND组的顺序，小脑上脚（SCP）和小脑中脚（MCP）的FA显著降低，MCP的ADC、AD和RD以及SCP的RD显著升高（FA-SCP；p<0.001，FA-MCP；p=0.026，ADC-MCP；p<0.001，AD-MCP；p=0.002，RD-MCP；p=0.003，RD-SCP；p=0.018）。此外，在简单线性回归分析中，MCP中的FA、ADC、AD和RD值以及SCP中的FA值受MRI上神经症状和ND表现的显著影响（所有p<0.001）。
在LCH-ND中，我们发现了SCP和MCP中的微观结构损伤。这些纤维束中的DTI参数可能有助于监测LCH-ND；因此，未来需要进行研究以在大型队列中验证这些结果。
