Abnormal Distribution of GABAA Receptors in Brain of Duchenne Muscular Dystrophy Patients.
Abnormal Distribution of GABAA Receptors in Brain of Duchenne Muscular Dystrophy Patients.
复制标题
杜氏肌营养不良症患者大脑中 GABAA 受体的异常分布。
作者：
Suzuki Y;Higuchi S;Aida I;Nakajima T;Nakada T
在这项研究中，我们试图：(1)确定GABAA受体（GABAA‐Rs）在杜氏肌营养不良症（DMD）患者大脑中的分布；(2)确定其分布模式是否与认知功能障碍相关。方法：14例DMD患者[青年（n= 7, 18-25岁）和老年人（n= 7, 30-37岁）组]和16名年龄匹配的正常志愿者参与研究。使用123i - IMZ - SPECT评估GABAA - R分布。神经心理学评估采用3种不同的测试单元，即WAIS‐III、WMS‐R和威斯康星卡片分类测试（WCST）。结果：所有DMD患者前额皮质123i‐IMZ摄取显著下降（P< 0.05）。虽然在WAIS‐III和WMS‐R中没有发现差异，但DMD患者的WCST评分（2.8±1.9）明显低于正常志愿者（5.4±0.7）（P< 0.01）。这两种异常在老年患者中更为明显。结论：研究结果表明，DMD伴随着GABAA‐Rs在前额叶皮层分布的减少。中国生物医学工程学报，2016,33 (5):591-595
Introduction:In this study we sought to: (1) determine the distribution of GABAAreceptors (GABAA‐Rs) in the brain of Duchenne muscular dystrophy (DMD) patients; and (2) ascertain if the distribution pattern correlates with cognitive dysfunction.Methods:Fourteen DMD patients [young adult (n= 7, 18–25 years old) and older adult (n= 7, 30–37 years old) groups] and 16 age‐matched normal volunteers participated. GABAA‐R distribution was assessed using123I‐IMZ‐SPECT. Neuropsychological assessments were performed using 3 different test batteries, the WAIS‐III, WMS‐R, and Wisconsin Card Sorting Test (WCST).Results:All DMD patients showed significant decline in123I‐IMZ uptake in the prefrontal cortex (P< 0.05). Although no differences were detected in the WAIS‐III and WMS‐R, the WCST scores of DMD patients (2.8 ± 1.9) were significantly lower (P< 0.01) than those of normal volunteers (5.4 ± 0.7). Both abnormalities were more pronounced in older adult patients.Conclusion:The findings demonstrate that DMD is accompanied by a reduction in the prefrontal cortex distribution of GABAA‐Rs.Muscle Nerve55: 591–595, 2017