进行性核上性麻痹临床不对称的神经元相关性。

Neuronal correlates of clinical asymmetry in progressive supranuclear palsy.

机构信息

From the *Department of Neurology, †Freiburg Brain Imaging (FBI), ‡Department of Psychiatry and Psychotherapy, §Department of Nuclear Medicine, University Medical Center Freiburg, Freiburg; and ¶Department of Psychiatry and Psychotherapy, University Medical Center Mainz, Mainz, Germany.

出版信息

Clin Nucl Med. 2014 Apr;39(4):319-25. doi: 10.1097/RLU.0000000000000373.

DOI:10.1097/RLU.0000000000000373

PMID:24566396

Abstract

PURPOSE

Progressive supranuclear palsy (PSP) is characterized by a symmetric hypokinetic syndrome with early falls and vertical supranuclear gaze palsy. However, clinically asymmetric manifestations occur, resembling idiopathic Parkinson disease or corticobasal degeneration. The aim of this study was to determine the neuronal correlates of patients suffering from PSP with a lateralized disease manifestation (hemi-PSP) in comparison to patients with symmetric clinical presentation (symPSP) and corticobasal degeneration.

METHODS

Twenty-three patients with PSP and 8 patients with corticobasal degeneration according to standard diagnostic criteria underwent F-fluorodeoxyglucose (FDG) PET scans to assess disease-specific patterns of regional cerebral glucose metabolism reflecting neuronal activity. Group differences were analyzed by statistical parametric mapping and region-of-interest analyses.

RESULTS

Clinically, 14 patients presented with symPSP while 9 patients were considered as hemi-PSP. Patients with symPSP or hemi-PSP showed similar bilateral medial frontal hypometabolism compared to corticobasal degeneration patients. In contrast, corticobasal degeneration patients exhibited a prominent parietal hypometabolism compared to both symPSP and hemi-PSP patients. SymPSP patients showed no significant hypometabolism compared to hemi-PSP, whereas hemi-PSP patients presented with significant hypometabolism of the motor thalamus, middle cingulate gyrus, and sensorimotor cortex contralateral to the most affected body side compared to symPSP patients.

CONCLUSIONS

The present study demonstrates that a more pronounced and asymmetric involvement of cortical and subcortical motor areas is associated with a lateralized disease manifestation of PSP. Furthermore, these findings strongly suggest that FDG PET imaging may assist the challenging clinical differentiation between hemi-PSP and corticobasal degeneration by depicting disease-specific patterns of regional cerebral glucose metabolism.

摘要

目的

进行性核上性麻痹（PSP）的特征为以早期跌倒和垂直核上凝视麻痹为特征的对称运动徐缓综合征。然而，临床上也会出现不对称的表现，类似于特发性帕金森病或皮质基底节变性。本研究旨在确定具有侧化疾病表现（半 PS ）的 PSP 患者与具有对称临床表现（symPSP）和皮质基底节变性患者的神经元相关性。

方法

根据标准诊断标准，23 例 PSP 患者和 8 例皮质基底节变性患者接受 F-氟脱氧葡萄糖（FDG）PET 扫描，以评估反映神经元活动的区域性脑葡萄糖代谢的疾病特异性模式。通过统计参数映射和感兴趣区分析来分析组间差异。

结果

临床上，14 例患者表现为 symPSP，9 例患者被认为是半 PS 。与皮质基底节变性患者相比，symPSP 或半 PS 患者的双侧内侧额部代谢均明显降低。相比之下，皮质基底节变性患者与 symPSP 和半 PS 患者相比，顶叶代谢明显降低。与半 PS 患者相比，symPSP 患者无明显代谢低下，而半 PS 患者对侧运动丘脑、中扣带回和感觉运动皮层的代谢明显低下。