Decreased cerebral ATP in pre-motor manifest Huntington's disease: A pilot study

IF 3.4 3区医学 Q2 CLINICAL NEUROLOGY

Parkinsonism & related disorders Pub Date : 2025-09-15 DOI:10.1016/j.parkreldis.2025.108040

Lindsay E. Golden , Jia Xu , Vincent A. Magnotta , Peg C. Nopoulos , Jordan L. Schultz

{"title":"Decreased cerebral ATP in pre-motor manifest Huntington's disease: A pilot study","authors":"Lindsay E. Golden , Jia Xu , Vincent A. Magnotta , Peg C. Nopoulos , Jordan L. Schultz","doi":"10.1016/j.parkreldis.2025.108040","DOIUrl":null,"url":null,"abstract":"<div><h3>Background</h3><div>Huntington's Disease (HD) is characterized by brain metabolic dysfunction, but no studies to date have directly measured cerebral ATP levels in patients with HD.</div></div><div><h3>Objective</h3><div>To compare cerebral ATP between individuals with pre-motor-manifest HD (preHD) and healthy controls (HC).</div></div><div><h3>Methods</h3><div>Cerebral ATP was quantified using <sup>31</sup>Phosphorous Magnetic Resonance Spectroscopy (<sup>31</sup>P-MRS) at 7T in nine preHD subjects and nine HC subjects. Analysis of Covariance models were constructed to compare mean ATP signal (corrected by whole brain volume) between groups after adjusting for age and BMI.</div></div><div><h3>Results</h3><div>PreHD participants exhibited significantly lower total ATP compared to HCs; furthermore, preHD subjects with higher clinical burden of disease had lower ATP levels. Amongst the preHD subjects, lower ATP levels were associated with worsening composite Unified Huntington's Disease Rating Scale scores.</div></div><div><h3>Conclusions</h3><div>Our findings highlight the potential of <sup>31</sup>P-MRS to serve as a direct assay for metabolic dysfunction in HD.</div></div>","PeriodicalId":19970,"journal":{"name":"Parkinsonism & related disorders","volume":"140 ","pages":"Article 108040"},"PeriodicalIF":3.4000,"publicationDate":"2025-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Parkinsonism & related disorders","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S1353802025007813","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}

引用次数: 0

Abstract

Background

Huntington's Disease (HD) is characterized by brain metabolic dysfunction, but no studies to date have directly measured cerebral ATP levels in patients with HD.

Objective

To compare cerebral ATP between individuals with pre-motor-manifest HD (preHD) and healthy controls (HC).

Methods

Cerebral ATP was quantified using ³¹Phosphorous Magnetic Resonance Spectroscopy (³¹P-MRS) at 7T in nine preHD subjects and nine HC subjects. Analysis of Covariance models were constructed to compare mean ATP signal (corrected by whole brain volume) between groups after adjusting for age and BMI.

Results

PreHD participants exhibited significantly lower total ATP compared to HCs; furthermore, preHD subjects with higher clinical burden of disease had lower ATP levels. Amongst the preHD subjects, lower ATP levels were associated with worsening composite Unified Huntington's Disease Rating Scale scores.

Conclusions

Our findings highlight the potential of ³¹P-MRS to serve as a direct assay for metabolic dysfunction in HD.

查看原文本刊更多论文

运动前显性亨廷顿病的脑ATP减少：一项初步研究

亨廷顿舞蹈病（HD）的特点是脑代谢功能障碍，但迄今为止还没有研究直接测量亨廷顿舞蹈病患者的脑ATP水平。目的比较运动前显性HD （preHD）与健康对照（HC）的脑ATP水平。方法采用31磷磁共振波谱法（31P-MRS）对9例预hd组和9例HC组在7T时的脑ATP进行定量分析。构建协方差分析模型，比较各组在调整年龄和BMI后的平均ATP信号（以全脑容量校正）。结果与hc相比，sprehd参与者的总ATP明显降低；此外，临床疾病负担较高的hd前期受试者ATP水平较低。在未患亨廷顿舞蹈症的受试者中，较低的ATP水平与亨廷顿舞蹈症综合评定量表评分的恶化有关。结论31P-MRS可作为HD患者代谢功能障碍的直接检测方法。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

Parkinsonism & related disorders 医学-临床神经学

CiteScore

6.20

自引率

4.90%

发文量

292

审稿时长

39 days

期刊介绍： Parkinsonism & Related Disorders publishes the results of basic and clinical research contributing to the understanding, diagnosis and treatment of all neurodegenerative syndromes in which Parkinsonism, Essential Tremor or related movement disorders may be a feature. Regular features will include: Review Articles, Point of View articles, Full-length Articles, Short Communications, Case Reports and Letter to the Editor.