Decreased striatal dopamine transporter density in JNCL patients with parkinsonian symptoms
Decreased striatal dopamine transporter density in JNCL patients with parkinsonian symptoms
复制标题
DOI：
10.1212/wnl.54.5.1069
复制
发表时间：
2000-03-14
期刊：
影响因子：
9.9
通讯作者：
Santavuori, P
中科院分区：
文献类型：
--
作者：
Åberg, L;Liewendahl, K;Santavuori, P
目的：探讨纹状体多巴胺转运蛋白是否参与伴有锥体外系体征的青少年神经元蜡样质脂褐质沉积症（JNCL）。方法：17 名 JNCL 患者进入研究（平均年龄，15 岁；年龄范围，10 至 31 岁）。为了进行临床评估，作者使用了统一帕金森病评定量表 (UPDRS) 的运动部分。为了研究纹状体中多巴胺转运蛋白的密度，他们使用碘 123 标记的 2β-甲氧基-3β-(4-碘苯基)托烷作为 SPECT 示踪剂。 SPECT 图像进行视觉评估，示踪剂积累从横向切片半定量为纹状体与小脑的活动比率。测量纹状体的 MRI (1.5-T) 信号强度并与丘脑的信号强度进行比较。结果：平均 UPDRS 评分为 20（范围：2 至 41）。 SPECT 中，患者的平均纹状体与小脑摄取比率低于对照受试者（3.1 +/- 0.6 与 6.8 +/- 1.0；p < 0.001），壳核中的下降比尾状核中更为明显。在 MRI 上，患者的平均纹状体与丘脑信号强度比高于对照组（1.14 +/- 0.02 对比 1.08 +/- 0.02；p < 0.001）。 SPECT 的摄取率与 UPDRS 评分呈负相关，MRI 的摄取率与 UPDRS 呈正相关。 SPECT 和 MRI 比率也显着相关，为黑质纹状体、纹状体和丘脑功能障碍对帕金森症状的影响提供了额外的证据。结论：JNCL 中观察到的纹状体多巴胺转运蛋白密度降低为多巴胺能药物治疗该疾病的试验提供了合理的基础。
Objective: To explore whether striatal dopamine transporters are involved in juvenile neuronal ceroid lipofuscinosis (JNCL) with extrapyramidal signs. Methods: Seventeen patients with JNCL entered the study (mean age, 15 years; age range, 10 to 31 years). For clinical evaluation, the authors used the motor section of the Unified Parkinson's Disease Rating Scale (UPDRS). For studying the density of dopamine transporters in the striatum, they employed iodine-123-labeled 2 beta-carbomethoxy-3 beta-(4-iodophenyl) tropane as a SPECT tracer. The SPECT images were evaluated visually, and tracer accumulation was semiquantified from transverse slices as striatum-to-cerebellum activity ratios. MRI (1.5-T) signal intensities of the striatum were measured and compared with those of the thalamus. Results: The mean UPDRS score was 20 (range, 2 to 41). On SPECT, the mean striatum-to-cerebellum uptake ratio was lower in patients than in control subjects (3.1 +/- 0.6 versus 6.8 +/- 1.0; p < 0.001), with the decrease being more pronounced in the putamen than in the caudate nucleus. On MRI, the mean striatum-to-thalamus signal intensity ratio was higher in patients than in control subjects (1.14 +/- 0.02 versus 1.08 +/- 0.02; p < 0.001). There was a negative correlation between uptake ratios in SPECT and UPDRS scores, and a positive correlation between the MRI ratios and UPDRS. The SPECT and MRI ratios also correlated significantly, providing additional evidence for the contributions of nigrostriatal, striatal, and thalamic dysfunction to the parkinsonian symptoms. Conclusions: The observed decrease in the striatal dopamine transporter density in JNCL offers a rational basis for a trial of dopaminergic drugs in this disease.