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Altered connectivity of central autonomic network: effects of dysautonomia in hereditary transthyretin amyloidosis with polyneuropathy.

Authors :
Su, Tsai-Jou
Lin, Chien-Ho
Liu, Yen-Lin
Hsueh, Hsueh-Wen
Hsieh, Sung-Tsang
Chao, Chi-Chao
Chiang, Ming-Chang
Source :
Amyloid. Dec2024, Vol. 31 Issue 4, p257-265. 9p.
Publication Year :
2024

Abstract

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. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
13506129
Volume :
31
Issue :
4
Database :
Academic Search Index
Journal :
Amyloid
Publication Type :
Academic Journal
Accession number :
181133669
Full Text :
https://doi.org/10.1080/13506129.2024.2383450