Publication Cover
Amyloid
The Journal of Protein Folding Disorders
Volume 30, 2023 - Issue 1
199
Views
0
CrossRef citations to date
0
Altmetric
Research Articles

Predictors of cognitive dysfunction in hereditary transthyretin amyloidosis with liver transplant

, , , , , , ORCID Icon & show all
Pages 119-126 | Received 26 Dec 2021, Accepted 27 Sep 2022, Published online: 17 Oct 2022
 

Abstract

Background

Cognitive dysfunction is part of the broad spectrum of clinical manifestations in older untreated hereditary transthyretin amyloidosis patients with peripheral polyneuropathy.

Objective

The objective of this study is to systematically explore cognitive dysfunction in ATTRV30M amyloidosis patients whose disease course was modified by liver transplant (LT).

Methods

A series of 269 carriers of TTRVal30Met mutation treated with LT underwent a neuropsychological assessment. Clinical charts were reviewed to identify focal neurological episodes (FNEs), cognitive complaints and laboratory results. Chi-square and Mann–Whitney tests explored potential predictors of cognitive dysfunction.

Results

Cognitive dysfunction was identified in 35 patients (13%)−14 (5%) had mild and 21 (8%) had moderate dysfunction. In comparison to normal cognition, both mild and moderate cognitive dysfunction patients had older age, higher mPND score and elevated NT-proBNP and Cystatin C values. Mild cognitive dysfunction was associated with longer disease duration and history of FNEs, whereas moderate dysfunction was related to older age at disease onset and more cognitive complaints and depression symptoms.

Conclusions

Consistent with the natural history of the disease, older age and higher severity of the disease are significantly associated and potentially predictors of cognitive dysfunction in ATTRV30M patients treated with LT. The level of cognitive dysfunction may depend on some clinical variables.

Acknowledgements

The authors thank all the patients who participated in this study. They also thank all the staff who assisted with data collection.

Disclosure statement

No potential conflict of interest was approved by the authorship and/or publication of this article.

Log in via your institution

Log in to Taylor & Francis Online

PDF download + Online access

  • 48 hours access to article PDF & online version
  • Article PDF can be downloaded
  • Article PDF can be printed
USD 65.00 Add to cart

Issue Purchase

  • 30 days online access to complete issue
  • Article PDFs can be downloaded
  • Article PDFs can be printed
USD 903.00 Add to cart

* Local tax will be added as applicable

Related Research

People also read lists articles that other readers of this article have read.

Recommended articles lists articles that we recommend and is powered by our AI driven recommendation engine.

Cited by lists all citing articles based on Crossref citations.
Articles with the Crossref icon will open in a new tab.