文摘
摘要目的:评估进步大脑退化在活的有机体内在肌萎缩性脊髓侧索硬化症(ALS)和比较异常临床亚组之间用核磁共振光谱学(夫人)。
背景:ALS与变性的电机和extra-motor的大脑区域,并与变量的发病和发展模式。夫人允许评估脑退化在活的有机体内量化的neurometabolite N-acetylaspartate (NAA)标记的神经元完整性。
设计/方法:七十六年59 ALS患者和健康对照组是从加拿大招募ALS神经影像财团。受试者接受临床评估和随访评估3 t MRI扫描4和8个月。NAA比率是量化运动皮层和内侧前额叶皮层(mPFC)。疾病进展速度,上运动神经元(学院)的迹象,和认知状态被用于分层患者到子组。线性混合模型用于基线和纵向比较的代谢物比率。
结果:与健康对照组相比,ALS患者减少NAA电动机地区(P< 0.001)。更快的进步ALS患者显著降低运动皮层NAA相比缓慢进展患者(P< 0.05)。更大的学院负担降低了患者运动皮层NAA相比降低患者负担(P< 0.05)。NAA mPFC只有减少认知障碍患者(P< 0.05)。显著下降,乙酰天冬氨酸在运动皮层观察到只有快速进展(P< 0.01)和高学院负担(P< 0.01)军团。
结论:运动皮层的进行性变性肌萎缩性侧索硬化症的夫人与更积极的临床表现,如更快的发展速度和更大程度的学院的迹象。这些发现提供了生物学的证据变量空间和时间脑变性疾病异质性与ALS。
披露:丹尼尔助教没有披露。阿卜杜拉Ishaque没有披露。斯利瓦斯塔瓦先生没有披露。克里斯托弗Hanstock没有披露。彼得·赛里斯没有披露。院长Eurich没有披露。陆医生没有披露。Briemberg博士已经收到个人薪酬在5000 - 9999美元的范围作为顾问生原体。立即家庭成员的机构Briemberg博士接到CIHR研究支持。Briemberg博士已经收到相关出版物的出版版税卫生保健。 An immediate family member of Dr. Briemberg has received publishing royalties from a publication relating to health care. The institution of Richard Frayne has received research support from CIHR, NSERC, and HSFC. Dr. Genge has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for AL-S Pharma. Dr. Genge has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Biogen. Dr. Genge has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Cytokinetics. Dr. Genge has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amylyx. Dr. Genge has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Quralis. Dr. Genge has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Alexion. Dr. Genge has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for MTPA. Dr. Genge has received personal compensation in the range of $500-$4,999 for serving as a Consultant for WAVE. Simon Graham has received personal compensation in the range of $100,000-$499,999 for serving as a Consultant for Various Canadian Hospitals. The institution of Simon Graham has received research support from Canadian Granting Agency. Simon Graham has received intellectual property interests from a discovery or technology relating to health care. Dr. Korngut has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen. Dr. Korngut has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubishi Tanabe. Dr. Korngut has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Dr. Zinman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen and MTP. Dr. Zinman has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen and MTP. The institution of Dr. Kalra has received research support from Brain Canada Foundation.
