Department of Neurobiology, Neurology and Geriatrics, Xuanwu Hospital of Capital Medical University, Beijing Institute of Geriatrics, Beijing, China.
Department of Neurology, The Affiliated Hospital of Xuzhou Medical University, Xuzhou, Jiangsu, China.
Int J Neurosci. 2020 Dec;130(12):1278-1281. doi: 10.1080/00207454.2020.1732965. Epub 2020 Mar 5.
Progressive supranuclear palsy (PSP) is a progressive neurodegenerative brain disease which has been rarely described in association with hyperkinetic symptoms. Here, we report a case of PSP that was presented with hyperkinetic movement disorder, hemiplegic dystonia, and other clinical features that overlap with behavioral variant frontotemporal dementia (bvFTD) and corticobasal syndrome (CBS). A 63-year-old female presented to our hospital with a history of frontal lobe symptoms, impaired cognition, hyperkinetic movement disorders, dystonia, and frequent falls. Her magnetic resonance imaging (MRI) scan showed atrophy of midbrain and right temporal lobe. [F]FDG PET result revealed reduced 18F-FDG uptake with obvious laterality (right > left). [F]THK5317 PET scan showed evident increased uptake in the brain stem and basal ganglia. Treatment with Tiapride significantly improved hyperkinetic symptoms, but other motor symptoms were not alleviated. Three years later, the patient could hardly walk even with assistance. PSP can present hyperkinetic movement disorders and asymmetry in image that widen the existing phenotypic spectrum.
进行性核上性麻痹（PSP）是一种进行性神经退行性脑疾病，其与多动症状相关的病例很少见。在这里，我们报告了一例 PSP 病例，其表现为多动性运动障碍、偏瘫性肌张力障碍和其他与行为变异额颞叶痴呆（bvFTD）和皮质基底节综合征（CBS）重叠的临床特征。一位 63 岁女性因额叶症状、认知障碍、多动性运动障碍、肌张力障碍和频繁跌倒而到我院就诊。她的磁共振成像（MRI）扫描显示中脑和右侧颞叶萎缩。[F]FDG PET 结果显示右侧明显偏侧性（右侧＞左侧）摄取减少。[F]THK5317 PET 扫描显示脑干部位和基底节明显摄取增加。用泰必利治疗显著改善了多动症状，但其他运动症状没有缓解。三年后，即使在他人帮助下，患者也几乎无法行走。PSP 可表现为多动性运动障碍和影像学上的不对称，从而拓宽了现有的表型谱。
