Neurology Service, Hospital Clinic de Barcelona, IDIBAPS, CIBERNED, Barcelona, Spain.
Psychiatry Service, Hospital Clinic de Barcelona, Barcelona, Spain.
Ann Neurol. 2017 Sep;82(3):419-428. doi: 10.1002/ana.25026.
To determine the usefulness of dopamine transporter (DAT) imaging to identify idiopathic rapid eye movement sleep behavior disorder (IRBD) patients at risk for short-term development of clinically defined synucleinopathy.
Eighty-seven patients with polysomnography-confirmed IRBD underwent I-FP-CIT DAT-SPECT. Results were compared to 20 matched controls without RBD who underwent DAT-SPECT. In patients, FP-CIT uptake was considered abnormal when values were two standard deviations below controls' mean uptake. After DAT-SPECT, patients were followed up during 5.7 ± 2.2 (range, 2.6-9.9) years.
Baseline DAT deficit was found in 51 (58.6%) patients. During follow-up, 25 (28.7%) subjects developed clinically defined synucleinopathy (Parkinson's disease in 11, dementia with Lewy bodies in 13, and multiple system atrophy in 1) with mean latency of 3.2 ± 1.9 years from imaging. Kaplan-Meier survival analysis showed increased risk of incident synucleinopathy in patients with abnormal DAT-SPECT than with normal DAT-SPECT (20% vs 6% at 3 years, 33% vs 18% at 5 years; log rank test, p = 0.006). Receiver operating characteristics curve revealed that reduction of FP-CIT uptake in putamen greater than 25% discriminated patients with DAT deficit who developed synucleinopathy from patients with DAT deficit that remained disease free after 3 years of follow-up. At 5-year follow-up, DAT-SPECT had 75% sensitivity, 51% specificity, 44% positive predictive value, 80% negative predictive value, and likelihood ratio 1.54 to predict synucleinopathy.
DAT-SPECT identifies IRBD patients at short-term risk for synucleinopathy. Decreased FP-CIT putamen uptake greater than 25% predicts synucleinopathy after 3 years' follow-up. These observations may be useful to select candidates for disease modification trials in IRBD. Ann Neurol 2017;82:419-428.
确定多巴胺转运体（DAT）成像在识别特发性快速眼动睡眠行为障碍（IRBD）患者短期发展为临床定义的突触核蛋白病风险中的作用。
87 例经多导睡眠图证实的 IRBD 患者接受了 I-FP-CIT DAT-SPECT 检查。结果与 20 名无 RBD 的匹配对照组进行了比较，这些对照组进行了 DAT-SPECT 检查。如果患者的 FP-CIT 摄取值低于对照组平均值的两个标准差，则认为摄取值异常。在 DAT-SPECT 后，患者接受了 5.7±2.2（范围，2.6-9.9）年的随访。
基线时发现 51 例（58.6%）患者存在 DAT 缺陷。在随访期间，25 例（28.7%）患者出现了临床定义的突触核蛋白病（11 例帕金森病，13 例路易体痴呆，1 例多系统萎缩），从影像学检查到发病的平均潜伏期为 3.2±1.9 年。Kaplan-Meier 生存分析显示，DAT-SPECT 异常患者发生突触核蛋白病的风险高于 DAT-SPECT 正常患者（3 年时分别为 20%和 6%，5 年时分别为 33%和 18%；对数秩检验，p=0.006）。受试者工作特征曲线显示，壳核中 FP-CIT 摄取减少大于 25%可以区分 DAT 缺陷且在 3 年随访后未发生疾病的患者与 DAT 缺陷且在 5 年内发生突触核蛋白病的患者。在 5 年随访时，DAT-SPECT 的敏感性为 75%，特异性为 51%，阳性预测值为 44%，阴性预测值为 80%，优势比为 1.54，可预测突触核蛋白病。
DAT-SPECT 可识别 IRBD 患者短期发生突触核蛋白病的风险。壳核中 FP-CIT 摄取减少大于 25%可预测 3 年后发生突触核蛋白病。这些观察结果可能有助于选择 IRBD 患者进行疾病修饰试验的候选者。
