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1 型神经纤维瘤病患儿伴脑干局灶性异常信号强度时听觉通路异常的存在:弥散张量成像特征。

Presence of Auditory Pathway Abnormalities in Children With Neurofibromatosis Type 1 With Brainstem Focal Areas of Abnormal Signal Intensity: Diffusion Tensor Imaging Features.

机构信息

Department of Radiology, Bezmialem Vakıf University Hospital, Istanbul, Turkey.

Department of Otorhinolaryngology, Abdurrahman Yurtaslan Oncology Training and Research Hospital, Ankara, Turkey.

出版信息

J Child Neurol. 2024 Jun;39(7-8):253-259. doi: 10.1177/08830738241261110. Epub 2024 Jun 10.

Abstract

To investigate whether there is a difference in mean diffusivity (MD) and fractional anisotropy (FA) values in the auditory pathways of neurofibromatosis type 1 patients with and without focal areas of abnormal signal intensity (FASI) compared to healthy controls by using diffusion tensor imaging (DTI). Patients were classified as group 1 with focal areas of abnormal signal intensity in the brainstem, group 2 without focal areas of abnormal signal intensity, and healthy control group 3 according to the MRI findings. Mean diffusivity and fractional anisotropy values of lateral lemniscus, inferior colliculus, corpus geniculatum mediale, Heschl gyrus, and brainstem were compared between groups. The correlation between mean diffusivity and fractional anisotropy values of auditory pathways and age was investigated. There was a significant difference between group 1 and group 2 in terms of mean diffusivity and fractional anisotropy values at lateral lemniscus, inferior colliculus, corpus geniculatum mediale, and Heschl gyrus. Increased mean diffusivity and decreased fractional anisotropy values at brainstem were found in group 1. There was a significant difference between group 1 and group 3 in terms of mean diffusivity values at all auditory pathways. Fractional anisotropy values obtained from lateral lemniscus, inferior colliculus, and Heschl gyrus decreased in group 1 compared with group 3. There was a negative correlation between mean diffusivity values and positive correlation between fractional anisotropy values at lateral lemniscus, inferior colliculus, Heschl gyrus, and age. Our diffusion tensor imaging findings show that the neuronal integrity of the auditory pathways is affected in neurofibromatosis type 1 patients with brainstem focal areas of abnormal signal intensity. We think that the disappearance of brainstem focal areas of abnormal signal intensity associated with myelin repair and the regression of diffusion tensor imaging changes in the auditory pathways occur simultaneously with advancing age in patients with neurofibromatosis type 1.

摘要

为了通过弥散张量成像(DTI)研究 1 型神经纤维瘤病患者是否存在听觉通路的平均弥散度(MD)和各向异性分数(FA)值与有或无异常信号强度灶(FASI)的脑桥局灶性异常信号强度的患者与健康对照组之间的差异。根据 MRI 结果,将患者分为 1 组(脑桥有局灶性异常信号强度)、2 组(脑桥无局灶性异常信号强度)和 3 组(健康对照组)。比较各组外侧丘系、下丘、内侧膝状体、Heschl 回和脑桥的平均弥散度和各向异性分数值。研究了听觉通路的平均弥散度和各向异性分数值与年龄之间的相关性。外侧丘系、下丘、内侧膝状体和 Heschl 回的平均弥散度和各向异性分数值在 1 组和 2 组之间存在显著差异。1 组脑桥的平均弥散度增加,各向异性分数值降低。所有听觉通路的平均弥散度值在 1 组和 3 组之间存在显著差异。与 3 组相比,1 组外侧丘系、下丘和 Heschl 回的各向异性分数值降低。外侧丘系、下丘、Heschl 回的平均弥散度值与年龄呈负相关,各向异性分数值呈正相关。我们的弥散张量成像研究结果表明,1 型神经纤维瘤病患者脑桥局灶性异常信号强度会影响听觉通路的神经元完整性。我们认为,1 型神经纤维瘤病患者随着年龄的增长,脑桥局灶性异常信号强度的消失与髓鞘修复以及听觉通路弥散张量成像变化的消退同时发生。

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