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基于髓母细胞瘤患儿病变图谱的小脑缄默综合征预测模型。

A predictive model for cerebellar mutism syndrome based on lesion map in children with medulloblastoma.

作者信息

Yang Wei, Ren Sikang, Li Yiming, Cai Yingjie, Peng Xiaojiao, Sun Hailang, Chen Jiashu, Chai Xinyi, Zhang Hong, Zhu Kaiyi, Ma Wenping, Tian Yongji, Ge Ming

机构信息

Department of Neurosurgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China.

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100070, China.

出版信息

Eur Radiol. 2025 Jun;35(6):3294-3304. doi: 10.1007/s00330-024-11264-8. Epub 2024 Dec 10.

Abstract

BACKGROUND

This study aimed to establish a voxel-based map to predict the occurrence of cerebellar mutism syndrome (CMS) and investigate the relationship between CMS and motor dysfunction.

METHOD

This multicenter study cohort included 224 patients diagnosed with medulloblastoma at Beijing Children's Hospital (n = 88) and Beijing Tiantan Hospital (n = 136). The dataset was randomly divided into training (n = 95), test (n = 41), and validation (n = 88) datasets. Voxel-based lesion-symptom mapping (VLSM) was used to identify the corresponding tumoral voxels for CMS and motor dysfunction. A prediction model was constructed based on the VLSM results.

RESULTS

The two cohorts showed significant differences in hydrocephalus (58.0 vs. 32.4%, p < 0.001), paraventricular edema (83.0 vs. 59.6%, p < 0.001), and presurgical ventricular-peritoneal shunt placement (53.7 vs. 10.2%, p < 0.001). The CMS group had a significantly higher rate of hydrocephalus than the non-CMS group (54.6 vs. 33.1%; p = 0.002). VLSM revealed that the left inferior cerebellar peduncle (32.26%), right dentate gyrus (24.23%), and right X lobule (21.79%) were the most impaired structures associated with CMS. CMS-related areas overlapped with motor planning and verbal fluency regions, while areas associated with motor dysfunction overlapped with motor planning. The prediction model achieved areas under the curve of 0.733, and 0.702 for the testing and external validation datasets, respectively.

CONCLUSIONS

VLSM-based models are used to predict CMS occurrence prior to surgery. CMS was related to motor planning and verbal fluency regions of the cerebellum. Motor dysfunction was associated with CMS functionally.

KEY POINTS

Question Cerebellar mutism syndrome (CMS) is a severe postoperative complication of medulloblastoma for which the specific responsible anatomical correlates remain unclear. Findings We identified areas associated with CMS in Voxel-based Lesion-Symptom Mapping (VLSM), which were used to establish the CMS predicting model. Clinical relevance VLSM analysis helps develop a reliable predictive model for CMS and explore its association with motor dysfunction, The establishment of this model will facilitate the prediction of CMS in clinical practice.

摘要

背景

本研究旨在建立基于体素的图谱以预测小脑缄默综合征(CMS)的发生,并探讨CMS与运动功能障碍之间的关系。

方法

这项多中心研究队列包括在北京儿童医院(n = 88)和北京天坛医院(n = 136)诊断为髓母细胞瘤的224例患者。数据集被随机分为训练集(n = 95)、测试集(n = 41)和验证集(n = 88)。基于体素的病变-症状映射(VLSM)用于识别与CMS和运动功能障碍相对应的肿瘤体素。基于VLSM结果构建预测模型。

结果

两个队列在脑积水(58.0%对32.4%,p < 0.001)、脑室旁白质水肿(83.0%对59.6%,p < 0.001)和术前脑室-腹腔分流术置入(53.7%对10.2%,p < 0.001)方面存在显著差异。CMS组的脑积水发生率显著高于非CMS组(54.6%对33.1%;p = 0.002)。VLSM显示,左小脑下脚(32.26%)、右齿状回(24.23%)和右X小叶(21.79%)是与CMS相关的最易受损结构。与CMS相关的区域与运动规划和语言流畅性区域重叠,而与运动功能障碍相关的区域与运动规划重叠。预测模型在测试集和外部验证集上的曲线下面积分别为0.733和0.702。

结论

基于VLSM的模型用于术前预测CMS的发生。CMS与小脑的运动规划和语言流畅性区域有关。运动功能障碍在功能上与CMS相关。

关键点

问题 小脑缄默综合征(CMS)是髓母细胞瘤严重的术后并发症,其具体的责任解剖关联尚不清楚。发现 我们在基于体素的病变-症状映射(VLSM)中确定了与CMS相关的区域,这些区域用于建立CMS预测模型。临床意义 VLSM分析有助于开发可靠的CMS预测模型并探索其与运动功能障碍的关联,该模型的建立将有助于临床实践中对CMS的预测。

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