BACKGROUND Familial idiopathic basal ganglia calcification (FIBGC) is a rare autosomal dominant disorder that causes bilateral calcification of the basal ganglia and/or cerebellar dentate nucleus, among other location...BACKGROUND Familial idiopathic basal ganglia calcification (FIBGC) is a rare autosomal dominant disorder that causes bilateral calcification of the basal ganglia and/or cerebellar dentate nucleus, among other locations. CASE SUMMARY The aim of this study is to report 10 cases of FIBGC observed in a single family. Seven patients showed calcification on their computed tomography scan, and all of these patients carried the SLC20A2 mutation. However, individuals without the mutation did not show calcification. Three patients among the 7 with calcification were symptomatic, while the remaining 4 patients were asymptomatic. Additionally, we longitudinally observed 10 subjects for ten years. In this paper, we mainly focus on the clinical course and neuroradiological findings in the proband and her son.CONCLUSION The accumulation of more case reports and further studies related to the manifestation of FIBGC are needed.展开更多
Objective:The image data of intracerebral hematoma in hypertensive intracerebral hemorrhage(HICH)patients were obtained by three-dimensional(3D)spiral computed tomography(CT)scan in this study to provide a basis for c...Objective:The image data of intracerebral hematoma in hypertensive intracerebral hemorrhage(HICH)patients were obtained by three-dimensional(3D)spiral computed tomography(CT)scan in this study to provide a basis for clinical minimally invasive surgery and the development and research of related surgical instruments.Methods:From June 2020 to March 2022,33 patients with supratentorial HICH admitted to the Department of Neurosurgery,the First Affiliated Hospital,Zhejiang University,School of Medicine were selected.All patients underwent 3D spiral CT scanning.Multiplanar reconstruction(MPR)was used to reconstruct along any plane to obtain coronal,sagittal,cross-sectional,or arbitrary angle reconstructed images.Then,we observed and measured relevant data indicators on these three planes by measuring tools.Results:All hemorrhage sites of these 33 HICH patients were basal ganglia hemorrhage,including left basal ganglia hemorrhage in 13 cases and right basal ganglia hemorrhage in 20 cases.It was also found that basal ganglia hematomas were usually elliptical,and the anteroposterior diameter was significantly larger than the transverse diameter,almost twice the size of the transverse diameter[(62±10)mm vs.(35±9)mm,P<0.05].Although the depth of the hematoma on the transfrontal(sagittal)approach was significantly greater than that on the transtemporal(transverse)approach[(100±15)mm vs.(59±14)mm,P<0.05],the angle of the hematoma on the transfrontal approach was significantly smaller than that on the transtemporal approach[(37±11)°vs.(70±17)°,P<0.05],which was conducive to improving the clearance rate of the hematoma.Conclusion:During neuroendoscopic surgery for HICH patients,different lengths of the tubular port should be selected according to the transfrontal or transtemporal surgical approach to meet the needs of hematoma removal.展开更多
目的:探讨基于CT平扫的影像组学模型预测基底节区脑出血血肿周围水肿(perihematomal edema,PHE)早期扩张风险的价值。方法:回顾性纳入2021年1月—2023年12月在汕头市中心医院住院的267例基底节区脑出血患者,其中男性165例,女性102例,年...目的:探讨基于CT平扫的影像组学模型预测基底节区脑出血血肿周围水肿(perihematomal edema,PHE)早期扩张风险的价值。方法:回顾性纳入2021年1月—2023年12月在汕头市中心医院住院的267例基底节区脑出血患者,其中男性165例,女性102例,年龄(63±11)岁,早期PHE扩张患者169例,非早期PHE扩张患者98例。将所有研究对象按7∶3比例随机分为训练集(187例)和测试集(80例),分别进行模型建立和内部验证。通过3D-Slicer软件在基线CT平扫图像上分别半自动分割脑血肿和周围水肿2个靶区,再用FAE影像组学软件分别提取2个靶区的影像组学特征。利用logistic回归建立基于临床因素和常规CT征象(临床-常规CT模型)、影像组学特征(影像组学模型)和两者联合(联合模型)的预测模型。采用受试者工作特征曲线下面积(area under curve,AUC)、校准曲线和决策曲线评估模型的预测价值。结果:由年龄、格拉斯哥昏迷评分、血肿体积、岛征和影像组学评分构建的联合模型在训练集和测试集中的AUC值(分别为0.956、0.943)高于临床-常规CT模型(分别为0.732、0.683)和影像组学模型(分别为0.912、0.871)(均P<0.05)。联合模型在训练集和测试集中的校准曲线Brier评分(分别为0.077、0.090)低于临床-常规CT模型(分别为0.194、0.218)和影像组学模型(分别为0.109、0.147)。在决策曲线分析中,联合模型在训练集和测试集中的大部分阈值概率范围内均具有最高的净获益。结论:基于CT平扫的影像组学联合模型在预测基底节区脑出血PHE的早期扩张风险方面具有较好的预测效能。展开更多
基金Supported by the grant-in-Aid for Scientific Research(C)from the Japan Society for the Promotion of Science(JSPS)No.17K103112
文摘BACKGROUND Familial idiopathic basal ganglia calcification (FIBGC) is a rare autosomal dominant disorder that causes bilateral calcification of the basal ganglia and/or cerebellar dentate nucleus, among other locations. CASE SUMMARY The aim of this study is to report 10 cases of FIBGC observed in a single family. Seven patients showed calcification on their computed tomography scan, and all of these patients carried the SLC20A2 mutation. However, individuals without the mutation did not show calcification. Three patients among the 7 with calcification were symptomatic, while the remaining 4 patients were asymptomatic. Additionally, we longitudinally observed 10 subjects for ten years. In this paper, we mainly focus on the clinical course and neuroradiological findings in the proband and her son.CONCLUSION The accumulation of more case reports and further studies related to the manifestation of FIBGC are needed.
基金This work was supported by the Project of Zhejiang Medical Science and Technology Plan[2020PY044].
文摘Objective:The image data of intracerebral hematoma in hypertensive intracerebral hemorrhage(HICH)patients were obtained by three-dimensional(3D)spiral computed tomography(CT)scan in this study to provide a basis for clinical minimally invasive surgery and the development and research of related surgical instruments.Methods:From June 2020 to March 2022,33 patients with supratentorial HICH admitted to the Department of Neurosurgery,the First Affiliated Hospital,Zhejiang University,School of Medicine were selected.All patients underwent 3D spiral CT scanning.Multiplanar reconstruction(MPR)was used to reconstruct along any plane to obtain coronal,sagittal,cross-sectional,or arbitrary angle reconstructed images.Then,we observed and measured relevant data indicators on these three planes by measuring tools.Results:All hemorrhage sites of these 33 HICH patients were basal ganglia hemorrhage,including left basal ganglia hemorrhage in 13 cases and right basal ganglia hemorrhage in 20 cases.It was also found that basal ganglia hematomas were usually elliptical,and the anteroposterior diameter was significantly larger than the transverse diameter,almost twice the size of the transverse diameter[(62±10)mm vs.(35±9)mm,P<0.05].Although the depth of the hematoma on the transfrontal(sagittal)approach was significantly greater than that on the transtemporal(transverse)approach[(100±15)mm vs.(59±14)mm,P<0.05],the angle of the hematoma on the transfrontal approach was significantly smaller than that on the transtemporal approach[(37±11)°vs.(70±17)°,P<0.05],which was conducive to improving the clearance rate of the hematoma.Conclusion:During neuroendoscopic surgery for HICH patients,different lengths of the tubular port should be selected according to the transfrontal or transtemporal surgical approach to meet the needs of hematoma removal.
文摘目的:探讨基于CT平扫的影像组学模型预测基底节区脑出血血肿周围水肿(perihematomal edema,PHE)早期扩张风险的价值。方法:回顾性纳入2021年1月—2023年12月在汕头市中心医院住院的267例基底节区脑出血患者,其中男性165例,女性102例,年龄(63±11)岁,早期PHE扩张患者169例,非早期PHE扩张患者98例。将所有研究对象按7∶3比例随机分为训练集(187例)和测试集(80例),分别进行模型建立和内部验证。通过3D-Slicer软件在基线CT平扫图像上分别半自动分割脑血肿和周围水肿2个靶区,再用FAE影像组学软件分别提取2个靶区的影像组学特征。利用logistic回归建立基于临床因素和常规CT征象(临床-常规CT模型)、影像组学特征(影像组学模型)和两者联合(联合模型)的预测模型。采用受试者工作特征曲线下面积(area under curve,AUC)、校准曲线和决策曲线评估模型的预测价值。结果:由年龄、格拉斯哥昏迷评分、血肿体积、岛征和影像组学评分构建的联合模型在训练集和测试集中的AUC值(分别为0.956、0.943)高于临床-常规CT模型(分别为0.732、0.683)和影像组学模型(分别为0.912、0.871)(均P<0.05)。联合模型在训练集和测试集中的校准曲线Brier评分(分别为0.077、0.090)低于临床-常规CT模型(分别为0.194、0.218)和影像组学模型(分别为0.109、0.147)。在决策曲线分析中,联合模型在训练集和测试集中的大部分阈值概率范围内均具有最高的净获益。结论:基于CT平扫的影像组学联合模型在预测基底节区脑出血PHE的早期扩张风险方面具有较好的预测效能。
