Cerebral involvement in spinal and bulbar muscular atrophy (Kennedy's disease): A pilot study of PET

被引：23

作者：

Lai, Tzu-Hsien ^{[1
,2
,3
,4
]}

Liu, Ren-Shyan ^{[5
]}

Yang, Bang-Hung ^{[5
]}

Wang, Po-Shan ^{[1
,6
]}

Lin, Kon-Ping ^{[1
,4
]}

Lee, Yi-Chung ^{[1
,4
]}

Soong, Bing-Wen ^{[1
,4
]}

机构：

[1] Natl Yang Ming Univ, Dept Neurol, Taipei 112, Taiwan

[2] Natl Yang Ming Univ, Inst Neurosci, Taipei 112, Taiwan

[3] Far Eastern Mem Hosp, Dept Internal Med, Div Neurol, New Taipei City, Taiwan

[4] Taipei Vet Gen Hosp, Dept Neurol, Taipei, Taiwan

[5] Taipei Vet Gen Hosp, Dept Nucl Med, Natl PET Cyclotron Ctr, Taipei, Taiwan

[6] Taipei Municipal Gan Dau Hosp, Dept Neurol, Taipei, Taiwan

来源：

JOURNAL OF THE NEUROLOGICAL SCIENCES | 2013年 / 335卷 / 1-2期

关键词：

Androgen receptor; Kennedy's disease; Polyglutamine disease; Positron emission tomography; Spinal and bulbar muscular atrophy; Motor neuron disease; BULBOSPINAL NEURONOPATHY; ANDROGEN RECEPTOR; BRAIN; METABOLISM; ALS;

D O I：

10.1016/j.jns.2013.09.016

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Objective: To investigate possible cerebral involvement in patients with spinal and bulbar muscular atrophy (SBMA) by F-18-fluorodeoxyglucose-positron emission tomography (FDG-PET). Design: Ten patients with molecularly-confirmed SBMA and 5 age- and gender-matched healthy controls were recruited for brain FDG-PET studies. The data were analyzed and compared using the statistical parametric mapping (SPM) method. Results: Glucose hypometabolism in frontal areas of the cerebrum was found in patients with SBMA. However, no significant correlation with clinical variables, such as CAG repeat length, age at onset, or serum testosterone levels, was noted. Conclusions: The perturbation of cerebral glucose metabolism in patients with SBMA argues against SBMA being a pure lower motor and sensory neuron syndrome. Mutations in the androgen receptor gene might have a more widespread effect in the cerebrum than previously recognized. (C) 2013 Elsevier B.V. All rights reserved.

引用

页码：139 / 144

页数：6

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