Abstract：
Early precision diagnosis and effective treatment of opsoclonus myoclonus ataxia syndrome (OMAS) patients presenting with neuroblastoma can prevent serious neurological outcomes.
To assess the diagnostic value of 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) imaging in pediatric OMAS with neuroblastoma.
A retrospective evaluation of 45 patients diagnosed with OMAS who underwent 18F-FDG PET/CT was performed. A univariate analysis was performed to compare clinical characteristics between OMAS with and without neuroblastoma. Univariate and multivariate logistic regression analyses were applied to identify independent risk factors for OMAS with neuroblastoma and to develop the clinical model. Finally, independent risk factors and PET/CT were fitted to build the combined model for the diagnosis of OMAS with neuroblastoma and presented as a nomogram. Receiver operating characteristic curve, decision curve, and calibration curve analyses were conducted to evaluate the performance of the models.
Among 45 patients, 27 were PET/CT-positive, 23/27 lesions were neuroblastoma, and four were false positives. One of the false positive patients was confirmed to be adrenal reactive hyperplasia by postoperative pathology, and the symptoms of OMAS disappeared in the remaining three cases during clinical follow-up. The average maximal standardized uptake value of PET/CT-positive lesions was 2.6. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of PET/CT were 100%, 81.8%, 85.2%, 100%, and 91.1%, respectively. Age at diagnosis, lactate dehydrogenase, and neuron-specific enolase showed statistically significant differences between OMAS with and without neuroblastoma. Lactate dehydrogenase was identified as the independent risk factor to develop the clinical model, and the clinical model demonstrated an area under the curve (AUC) of 0.82 for the diagnosis of OMAS with neuroblastoma, with an AUC as high as 0.91 when combined with PET/CT. The decision curve analysis and calibration curve demonstrated that the nomogram had good consistency and clinical usefulness.
In patients with OMAS, 18F-FDG PET/CT has a high diagnostic accuracy in detecting tumors of the neuroblastoma, especially when combined with the independent risk factor serum lactate dehydrogenase.
To assess the diagnostic value of 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) imaging in pediatric OMAS with neuroblastoma.
A retrospective evaluation of 45 patients diagnosed with OMAS who underwent 18F-FDG PET/CT was performed. A univariate analysis was performed to compare clinical characteristics between OMAS with and without neuroblastoma. Univariate and multivariate logistic regression analyses were applied to identify independent risk factors for OMAS with neuroblastoma and to develop the clinical model. Finally, independent risk factors and PET/CT were fitted to build the combined model for the diagnosis of OMAS with neuroblastoma and presented as a nomogram. Receiver operating characteristic curve, decision curve, and calibration curve analyses were conducted to evaluate the performance of the models.
Among 45 patients, 27 were PET/CT-positive, 23/27 lesions were neuroblastoma, and four were false positives. One of the false positive patients was confirmed to be adrenal reactive hyperplasia by postoperative pathology, and the symptoms of OMAS disappeared in the remaining three cases during clinical follow-up. The average maximal standardized uptake value of PET/CT-positive lesions was 2.6. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of PET/CT were 100%, 81.8%, 85.2%, 100%, and 91.1%, respectively. Age at diagnosis, lactate dehydrogenase, and neuron-specific enolase showed statistically significant differences between OMAS with and without neuroblastoma. Lactate dehydrogenase was identified as the independent risk factor to develop the clinical model, and the clinical model demonstrated an area under the curve (AUC) of 0.82 for the diagnosis of OMAS with neuroblastoma, with an AUC as high as 0.91 when combined with PET/CT. The decision curve analysis and calibration curve demonstrated that the nomogram had good consistency and clinical usefulness.
In patients with OMAS, 18F-FDG PET/CT has a high diagnostic accuracy in detecting tumors of the neuroblastoma, especially when combined with the independent risk factor serum lactate dehydrogenase.
摘要：
背景：对以神经母细胞瘤表现为神经母细胞瘤的神经阵挛性肌阵挛性共济失调综合征（OMAS）患者进行早期精确诊断和有效治疗可以预防严重的神经系统预后。
目的：评估18F-氟代脱氧葡萄糖（FDG）正电子发射断层扫描/计算机断层扫描（PET/CT）成像在小儿OMAS合并神经母细胞瘤中的诊断价值。
方法：对45例诊断为OMAS且接受18F-FDGPET/CT检查的患者进行回顾性评估。进行单变量分析以比较有和没有神经母细胞瘤的OMAS之间的临床特征。应用单变量和多变量逻辑回归分析来确定OMAS合并神经母细胞瘤的独立危险因素并建立临床模型。最后,对独立危险因素和PET/CT进行拟合,以建立诊断OMAS合并神经母细胞瘤的联合模型,并显示为列线图.接收机工作特性曲线,决策曲线,和校准曲线分析进行评估模型的性能。
结果：在45名患者中，27例PET/CT阳性,23/27病灶为神经母细胞瘤，四个是假阳性。其中1例假阳性患者经术后病理证实为肾上腺反应性增生,其余3例患者在临床随访中OMAS症状消失。PET/CT阳性病灶的平均最大标准化摄取值为2.6。敏感性,特异性，正预测值，负预测值，PET/CT的准确率为100%,81.8%,85.2%,100%,91.1%，分别。诊断时的年龄，乳酸脱氢酶,和神经元特异性烯醇化酶在有和没有神经母细胞瘤的OMAS之间显示出统计学上的显着差异。乳酸脱氢酶被确定为建立临床模型的独立危险因素，并且临床模型显示出0.82的曲线下面积（AUC）用于诊断OMAS伴神经母细胞瘤，当与PET/CT结合时，AUC高达0.91。决策曲线分析和校准曲线表明，列线图具有良好的一致性和临床实用性。
结论：在OMAS患者中，18F-FDGPET/CT对神经母细胞瘤的诊断准确率高,尤其是合并血清乳酸脱氢酶的独立危险因素。
目的：评估18F-氟代脱氧葡萄糖（FDG）正电子发射断层扫描/计算机断层扫描（PET/CT）成像在小儿OMAS合并神经母细胞瘤中的诊断价值。
方法：对45例诊断为OMAS且接受18F-FDGPET/CT检查的患者进行回顾性评估。进行单变量分析以比较有和没有神经母细胞瘤的OMAS之间的临床特征。应用单变量和多变量逻辑回归分析来确定OMAS合并神经母细胞瘤的独立危险因素并建立临床模型。最后,对独立危险因素和PET/CT进行拟合,以建立诊断OMAS合并神经母细胞瘤的联合模型,并显示为列线图.接收机工作特性曲线,决策曲线,和校准曲线分析进行评估模型的性能。
结果：在45名患者中，27例PET/CT阳性,23/27病灶为神经母细胞瘤，四个是假阳性。其中1例假阳性患者经术后病理证实为肾上腺反应性增生,其余3例患者在临床随访中OMAS症状消失。PET/CT阳性病灶的平均最大标准化摄取值为2.6。敏感性,特异性，正预测值，负预测值，PET/CT的准确率为100%,81.8%,85.2%,100%,91.1%，分别。诊断时的年龄，乳酸脱氢酶,和神经元特异性烯醇化酶在有和没有神经母细胞瘤的OMAS之间显示出统计学上的显着差异。乳酸脱氢酶被确定为建立临床模型的独立危险因素，并且临床模型显示出0.82的曲线下面积（AUC）用于诊断OMAS伴神经母细胞瘤，当与PET/CT结合时，AUC高达0.91。决策曲线分析和校准曲线表明，列线图具有良好的一致性和临床实用性。
结论：在OMAS患者中，18F-FDGPET/CT对神经母细胞瘤的诊断准确率高,尤其是合并血清乳酸脱氢酶的独立危险因素。
