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中脑血管瘤所致红核震颤中的多巴胺缺乏:病例报告

Dopamine deficiency in rubral tremor caused by midbrain hemangioma: case report.

作者信息

Weng Y H, Kao P F, Tsai C H, Yen T C, Lu C S

机构信息

First Department of Neurology, Chang Gung Memorial Hospital, Taipei, Taiwan, R.O.C.

出版信息

Chang Gung Med J. 2000 Aug;23(8):485-91.

PMID:11039251
Abstract

To elucidate the nigrostriatal involvement in rubral tremor, we studied single photon emission computed tomography (SPECT) imaging with [2-[[2-[[[3-(4-chlorophenyl)-8-methyl-8-azabicyclo[3.2.1]oct-2- yl]methyl](2-mercaptoethyl)amino]ethyl]amino]ethanethiolato(3-)- N2,N2',S2,S2']oxo-[1R-(exo-exo)]-[99mTc]technetium ([99mTc]TRODAT-1) in a 70-year-old woman with a midbrain hemangioma. She had developed a slow tremor in her right arm and leg after an episode of hemorrhage at the age of 28. The tremor was 3 to 5 Hz in frequency at rest, which was enhanced by outstretching the limb and action. There was no rigidity or bradykinesia. Neurological examination also revealed mild palsy of the left oculomotor nerve. The magnetic resonance imaging (MRI) of the brain showed a small hemangioma in the left midbrain localized mainly in the substantia nigra. The [99mTc]TRODAT-1 SPECT imaging revealed significantly reduced [99mTc]TRODAT-1 uptake in the left caudate and putamen, but it was only mildly reduced in the right striatum. This reduction in uptake was even more severe than that of patients with Parkinson's disease, and indicated that the dopamine function was markedly impaired in the left nigrostriatal system. The tremor had not progressed over the years, and she responded moderately to treatment with levodopa. We concluded that the rubral tremor in the right extremities was probably caused by a dopamine deficiency in the left nigrostriatal system. This suggests that a dopamine deficiency secondary to the midbrain hemangioma might have contributed to the development of the rubral tremor in this patient.

摘要

为阐明黑质纹状体在红核震颤中的作用,我们对一名患有中脑血管瘤的70岁女性进行了单光子发射计算机断层扫描(SPECT)成像研究,使用的示踪剂为[2-[[2-[[[3-(4-氯苯基)-8-甲基-8-氮杂双环[3.2.1]辛-2-基]甲基](2-巯基乙基)氨基]乙基]氨基]乙硫醇盐(3-)-N2,N2',S2,S2']氧代-[1R-(外向-外向)]-99m锝。她在28岁时因一次出血发作后,右侧手臂和腿部出现了缓慢震颤。静止时震颤频率为3至5赫兹,伸展肢体和活动时震颤会加重。没有强直或运动迟缓。神经学检查还发现左侧动眼神经轻度麻痹。脑部磁共振成像(MRI)显示左中脑有一个小血管瘤,主要位于黑质。[99mTc]TRODAT-1 SPECT成像显示左侧尾状核和壳核的[99mTc]TRODAT-1摄取显著减少,但右侧纹状体仅轻度减少。这种摄取减少甚至比帕金森病患者更严重,表明左侧黑质纹状体系统的多巴胺功能明显受损。多年来震颤没有进展,她对左旋多巴治疗有中度反应。我们得出结论,右侧肢体的红核震颤可能是由左侧黑质纹状体系统多巴胺缺乏引起的。这表明中脑血管瘤继发的多巴胺缺乏可能促成了该患者红核震颤的发生。

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