中枢自律神经网络连接的改变:遗传性转甲状腺素淀粉样变性伴有多发性神经病的自律神经失调症的影响。

IF 5.2 2区 医学 Q1 BIOCHEMISTRY & MOLECULAR BIOLOGY
Tsai-Jou Su, Chien-Ho Janice Lin, Yen-Lin Liu, Hsueh-Wen Hsueh, Sung-Tsang Hsieh, Chi-Chao Chao, Ming-Chang Chiang
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引用次数: 0

摘要

背景:遗传性转甲状腺素淀粉样变性多神经病(ATTRv-PN)是一种进行性致命疾病,由突变的转甲状腺素(TTR)淀粉样蛋白主要沉积在神经和心脏中引起。自主神经功能障碍是一种主要的致残表现,90%的晚发性ATTRv-PN患者都会受到影响。本研究旨在探讨与ATTRv-PN患者外周自主神经变性导致的自主神经功能障碍相关的脑功能改变:方法:采集了43名主要为A97S(p.A117S)基因型的ATTRv-PN患者的静息态功能磁共振成像数据,并评估了中枢自主神经区域的功能连接性:与年龄匹配的健康对照组相比,ATTRv-PN 患者表现出:(1)下丘脑、杏仁核、前岛叶和中扣带回皮层等中枢自律神经区域与边缘系统、额叶系统和体感系统等脑区的功能连接性降低;(2)自律神经功能连接性降低与自律神经功能紊乱的严重程度相关,尤其是直立性不耐受、心率变异性降低和临床残疾程度加重:我们的研究结果提供了证据,证明 ATTRv-PN 患者的外周自主神经功能障碍与中枢自主神经网络的连接性改变有关。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Altered connectivity of central autonomic network: effects of dysautonomia in hereditary transthyretin amyloidosis with polyneuropathy.

Background: Hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN) is a progressive fatal disorder caused by deposition of mutant transthyretin (TTR) amyloids mainly in the nerves and heart. Autonomic dysfunction is a major disabling manifestation, affecting 90% of patients with late-onset ATTRv-PN. The current study aimed to investigate brain functional alterations associated with dysautonomia due to peripheral autonomic nerve degeneration in ATTRv-PN.

Methods: Resting-state functional MRI data were acquired from 43 ATTRv-PN patients predominantly of A97S (p.A117S) genotype, and the functional connectivity of central autonomic regions was assessed.

Results: Compared with age-matched healthy controls, the ATTRv-PN patients exhibited (1) reduced functional connectivity of the central autonomic regions such as hypothalamus, amygdala, anterior insula, and middle cingulate cortex with brain areas of the limbic, frontal, and somatosensory systems, and (2) correlations of reduced functional autonomic connectivity with the severity of autonomic dysfunction especially orthostatic intolerance, decreased heart rate variability, and greater clinical disability.

Conclusions: Our findings provide evidence linking peripheral autonomic dysfunction with altered connectivity in the central autonomic network in ATTRv-PN.

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来源期刊
Amyloid-Journal of Protein Folding Disorders
Amyloid-Journal of Protein Folding Disorders 生物-生化与分子生物学
CiteScore
10.60
自引率
10.90%
发文量
48
审稿时长
6-12 weeks
期刊介绍: Amyloid: the Journal of Protein Folding Disorders is dedicated to the study of all aspects of the protein groups and associated disorders that are classified as the amyloidoses as well as other disorders associated with abnormal protein folding. The journals major focus points are: etiology, pathogenesis, histopathology, chemical structure, nature of fibrillogenesis; whilst also publishing papers on the basic and chemical genetic aspects of many of these disorders. Amyloid is recognised as one of the leading publications on amyloid protein classifications and the associated disorders, as well as clinical studies on all aspects of amyloid related neurodegenerative diseases and major clinical studies on inherited amyloidosis, especially those related to transthyretin. The Journal also publishes book reviews, meeting reports, editorials, thesis abstracts, review articles and symposia in the various areas listed above.
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