摘要： 目的 探讨头部MRI检查及急性多巴丝肼（美多芭）反应试验诊断进行性核上性麻痹及其与帕金森病鉴别诊断的临床应用价值。方法 回顾分析性别、年龄相匹配的17 例进行性核上性麻痹和17 例帕金森病患者的头部MRI 及急性美多芭反应试验结果，总结其临床表现特点。结果 进行性核上性麻痹组患者正中矢状位T1WI 序列均可见中脑上缘呈现平坦或凹陷征象，中脑横截面积［（77.35 ± 15.30）mm²］和中脑面积/脑桥面积比值［（14.31 ± 2.47）%］均低于帕金森病组［（142.35 ± 31.49）mm²、（24.08 ± 4.73）％］，且差异有统计学意义（均P = 0.000）。急性美多芭反应试验显示，进行性核上性麻痹组和帕金森病组统一帕金森病评价量表（UPDRS）Ⅲ评分最大改善率> 30%者分别为2 和16 例，组间差异具有统计学意义（χ² = 23.142，P = 0.000）。结论 进行性核上性麻痹患者中脑MRI 特征和急性美多芭反应试验结果与帕金森病患者存在显著差异，有助于二者的鉴别诊断。

关键词: 核上麻痹, 进行性, 帕金森病, 磁共振成像, 多巴胺, 肼类

Abstract: Objective To investigate the MRI abnormalities and acute madopar responsiveness test in diagnosing progressive supranuclear palsy (PSP) and Parkinson's disease (PD). Methods Seventeen patients with PSP and 17 gender and age matched patients with PD were studied with cranial MRI examinations and results of acute madopar responsiveness test, and the clinical manifestations of PSP were summarized. Results The atrophy of the midbrain tegmentum and hummingbird sign was demonstrated in all of the PSP patients in our study, but was not observed in the PD patients. The areas of the midbrain on mid-sagittal MRI in PSP patients [(77.35 ± 15.30) mm²] were significantly smaller than that in those with PD [(142.35 ± 31.49) mm²]. The average ratio of the area of the midbrain to the area of pons in the patients with PSP [(14.31 ± 2.47)%] was significantly smaller than that in those with PD [(24.08 ± 4.73)%; P = 0.000, for all]. According to the result of acute madopar responsiveness test, the maximum Unified Parkinson's Disease Rating Scale (UPDRS) Ⅲ improvement rate of 2 patients with PSP and 16 patients with PD was more than 30％ (χ² = 23.142, P = 0.000). Conclusion The assessment of the mid-sagittal MRI and acute madopar responsiveness test may be a useful method to differentiate PSP from PD.

Key words: Supranuclear palsy, progressive, Parkinson disease, Magnetic resonance imaging, Dopamine, Hydrazines