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偏侧帕金森综合征-偏侧萎缩综合征的代谢地形图

Metabolic topography of the hemiparkinsonism-hemiatrophy syndrome.

作者信息

Przedborski S, Giladi N, Takikawa S, Ishikawa T, Dhawan V, Spetsieris P, Chaly T, Fahn S, Eidelberg D

机构信息

Department of Neurology, Columbia University, New York, NY.

出版信息

Neurology. 1994 Sep;44(9):1622-8. doi: 10.1212/wnl.44.9.1622.

DOI:10.1212/wnl.44.9.1622
PMID:7936286
Abstract

We estimated regional and global metabolic rates for glucose using 18F-fluorodeoxyglucose (FDG) and PET in six patients with hemiparkinsonism-hemiatrophy syndrome (HPHA; mean age, 41.0 +/- 12.4 years). We used 18F-fluorodopa (FDOPA) and PET in two patients to quantify presynaptic nigrostriatal dopaminergic function. We compared measures of brain glucose metabolism and striatal FDOPA uptake with those calculated for 10 age-matched normal volunteers (mean age, 35.1 +/- 8.0 years) and 10 patients with typical unilateral Parkinson's disease (unilat-PD; mean age, 58.2 +/- 13.8 years). All six HPHA patients demonstrated significant metabolic reductions (> 3 SD) in the contralateral basal ganglia or frontal cortex as compared with normal control values. Mean normalized glucose metabolism was reduced in the contralateral caudate and lentiform nuclei (p < 0.005) as compared with that in unilat-PD and normal controls. In both patients studied with FDOPA, contralateral striatal uptake was significantly reduced (> 3 SD) as compared with normal control values. These results suggest that the clinical manifestations of HPHA arise through a combination of pre- and postsynaptic nigrostriatal dopaminergic dysfunction. FDG and PET may be useful in differentiating this disorder from typical unilat-PD.

摘要

我们使用18F-氟脱氧葡萄糖(FDG)和正电子发射断层扫描(PET)对6例偏侧帕金森病-偏侧萎缩综合征(HPHA;平均年龄41.0±12.4岁)患者的区域和全球葡萄糖代谢率进行了估计。我们对2例患者使用18F-氟多巴(FDOPA)和PET来量化突触前黑质纹状体多巴胺能功能。我们将脑葡萄糖代谢和纹状体FDOPA摄取的测量值与10名年龄匹配的正常志愿者(平均年龄35.1±8.0岁)和10例典型单侧帕金森病(unilat-PD;平均年龄58.2±13.8岁)患者计算得出的测量值进行了比较。与正常对照值相比,所有6例HPHA患者对侧基底神经节或额叶皮质均表现出显著的代谢降低(>3个标准差)。与unilat-PD和正常对照相比,对侧尾状核和豆状核的平均标准化葡萄糖代谢降低(p<0.005)。在使用FDOPA研究的2例患者中,与正常对照值相比,对侧纹状体摄取均显著降低(>3个标准差)。这些结果表明,HPHA的临床表现是由突触前和突触后黑质纹状体多巴胺能功能障碍共同引起的。FDG和PET可能有助于将这种疾病与典型的unilat-PD区分开来。

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引用本文的文献

1
Hidden Gems in Neurology: The Syndrome of Hemiparkinsonism-Hemiatrophy.神经病学中的隐藏瑰宝:偏侧帕金森氏症 - 偏侧萎缩综合征
J Parkinsons Dis. 2024;14(7):1519-1525. doi: 10.3233/JPD-240290.
2
Hemiparkinsonism or Hemidystonia With Hemiatrophy Syndrome: A Case Series With Follow-Up.偏侧帕金森综合征或伴有偏侧萎缩的偏侧肌张力障碍综合征:随访病例系列
Front Neurosci. 2020 Feb 4;14:64. doi: 10.3389/fnins.2020.00064. eCollection 2020.