脑脊髓性肌萎缩症（肯尼迪病）中的脑受累：PET 的初步研究。
Cerebral involvement in spinal and bulbar muscular atrophy (Kennedy's disease): a pilot study of PET.
机构信息
Department of Neurology, National Yang-Ming University, Taipei, Taiwan; Institute of Neuroscience, National Yang-Ming University, Taipei, Taiwan; Division of Neurology, Department of Internal Medicine, Far Eastern Memorial Hospital, New Taipei City, Taiwan; Department of Neurology, Taipei Veterans General Hospital, Taipei, Taiwan.
出版信息
J Neurol Sci. 2013 Dec 15;335(1-2):139-44. doi: 10.1016/j.jns.2013.09.016. Epub 2013 Sep 21.
OBJECTIVE
To investigate possible cerebral involvement in patients with spinal and bulbar muscular atrophy (SBMA) by (18)F-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.
目的
通过（18）F-氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）研究脊髓延髓肌萎缩症（SBMA）患者可能存在的脑部受累情况。
设计
招募了 10 名经分子证实的 SBMA 患者和 5 名年龄和性别匹配的健康对照者进行脑 FDG-PET 研究。使用统计参数映射（SPM）方法对数据进行分析和比较。
结果
在 SBMA 患者的大脑额叶区域发现葡萄糖代谢低下。然而，与临床变量（如 CAG 重复长度、发病年龄或血清睾酮水平）无显著相关性。
结论
SBMA 患者大脑葡萄糖代谢的紊乱表明 SBMA 不仅仅是一种单纯的下运动神经元和感觉神经元综合征。雄激素受体基因突变可能对大脑的影响比以前认识的更为广泛。
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