| 王祥杰,徐睿,曾永娟,聂文琦,胡晓,王荣品,曾宪春,王美鑫.肝豆状核变性患者纹状体和丘脑γ-氨基丁酸浓度变化及其与运动障碍的相关性[J].中国医学影像技术,2026,42(6):926~930 |
| 肝豆状核变性患者纹状体和丘脑γ-氨基丁酸浓度变化及其与运动障碍的相关性 |
| Changes of γ-aminobutyric acid concentration in striatum and thalamus and correlations with movement disorders in patients with hepatolenticular degeneration |
| 投稿时间：2026-02-25 修订日期：2026-06-13 |
| DOI：10.13929/j.issn.1003-3289.2026.06.028 |
| 中文关键词: 肝豆状核变性 运动障碍 γ-氨基丁酸 磁共振波谱 |
| 英文关键词:hepatolenticular degeneration movement disorders gamma-aminobutyric acid magnetic resonance spectroscopy |
| 基金项目:贵州省高层次创新型人才"千层次"人才培养计划(GZSYQCC【2023】009-1)。 |
| 作者 | 单位 | E-mail | | 王祥杰 | 贵州省精准影像诊疗国际科技合作基地, 贵州省先进医学成像与智能计算全省重点实验室, 贵州省人民医院医学影像科, 贵州 贵阳 550002 | | | 徐睿 | 贵州省精准影像诊疗国际科技合作基地, 贵州省先进医学成像与智能计算全省重点实验室, 贵州省人民医院医学影像科, 贵州 贵阳 550002 | xuruily9@163.com | | 曾永娟 | 贵州省精准影像诊疗国际科技合作基地, 贵州省先进医学成像与智能计算全省重点实验室, 贵州省人民医院医学影像科, 贵州 贵阳 550002
遵义医科大学研究生院, 贵州 遵义 563000 | | | 聂文琦 | 贵州省精准影像诊疗国际科技合作基地, 贵州省先进医学成像与智能计算全省重点实验室, 贵州省人民医院医学影像科, 贵州 贵阳 550002
遵义医科大学研究生院, 贵州 遵义 563000 | | | 胡晓 | 贵州省人民医院神经内科, 贵州 贵阳 550002 | | | 王荣品 | 贵州省精准影像诊疗国际科技合作基地, 贵州省先进医学成像与智能计算全省重点实验室, 贵州省人民医院医学影像科, 贵州 贵阳 550002 | | | 曾宪春 | 贵州省人民医院核医学科, 贵州 贵阳 550002 | | | 王美鑫 | 贵州省人民医院神经内科, 贵州 贵阳 550002 | |
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| 中文摘要: |
| 目的 观察肝豆状核变性(HLD)患者纹状体和丘脑γ-氨基丁酸(GABA)浓度变化及其与运动障碍严重程度的相关性。方法 前瞻性纳入31例HLD患者,包括19例神经型(神经型组)和12例肝病型(肝病型组),以22名健康人作为健康对照(HC)组。以Meshcher-Garwood点分辨光谱(MEGA-PRESS)序列行颅脑MR扫描,于左侧纹状体及左侧丘脑放置ROI,定量分析其内GABA,并以水信号为参照计算GABA浓度[以国际单位(i.u.)表示]。采用威尔逊病统一评定量表神经量表(UWDRS-N)评估神经型组运动障碍严重程度。以性别和年龄为协变量,利用协方差分析比较3组GABA浓度,并以Tukey事后检验进行分析。绘制受试者工作特征(ROC)曲线,计算曲线下面积(AUC),评估GABA浓度诊断HLD及分型的效能。采用年龄校正的多元线性回归分析评估神经型组GABA浓度与UWDRS-N评分的关联。结果 相比对照组,神经型组与肝病型组纹状体GABA浓度均降低(校正P均＜0.05),且神经型组降低程度大于肝病型组(校正P＜0.05)。GABA浓度诊断HLD及区分神经型与肝病型HLD的AUC分别为0.870及0.873。神经型HLD患者纹状体GABA相对浓度每降低0.1 i.u.,则其UWDRS-N评分升高3.7分(P＜0.05)。结论 HLD患者纹状体GABA浓度降低、尤以神经型为著,且与运动障碍严重程度密切相关。 |
| 英文摘要: |
| Objective To observe changes of γ-aminobutyric acid (GABA) concentration in striatum and thalamus in hepatolenticular degeneration (HLD) patients and correlations with severity of movement disorders. Methods A total of 31 cases of HLD, including 19 cases of neurological HLD (neurological group) and 12 cases of hepatic HLD (hepatic group), as well as 22 healthy controls (HC) (HC group) were prospectively enrolled. Brain MR scanning was performed using MEscher-GArwood Point RESolved Spectroscopy (MEGA-PRESS) sequence. ROI were placed in the left striatum and left thalamus. Then GABA was quantified, and GABA concentrations were calculated taking water signal as the reference and expressed in international units (i.u.). The severity of movement disorders in neurological group was assessed using the unified Wilson's disease rating scale neurological subscale (UWDRS-N). Taken gender and age as covariates, analysis of covariance was employed to compare GABA concentrations among 3 groups, and Tukey's honest significant difference test was used between each 2 groups. Receiver operating characteristic (ROC) curves were plotted, and the area under the curve (AUC) was used to evaluate the efficacy of GABA concentrations for diagnosing HLD and differentiating types of HLD. Age-adjusted multiple linear regression was employed to analyze the associations of GABA concentration and UWDRS-N score in neurological group. Results Compared with HC group, striatal GABA concentration decreased in both neurological group and hepatic group (both adjusted P＜0.05), which was more obvious in neurological group than in hepatic group (adjusted P＜0.05). The AUC of GABA concentration for diagnosing HLD and distinguishing neurological from hepatic HLD was 0.870 and 0.873, respectively. In patients with neurological HLD, each 0.1 i.u. decrease of striatal GABA concentration indicated a 3.7-point increase of UWDRS-N score (P＜0.05). Conclusion Striatal GABA concentration decreased in HLD patients, especially in neurological type HLD, which was closely associated with the severity of movement disorders. |
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