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Published in: Neurological Sciences 5/2020

01-05-2020 | Liver Transplantation | Original Article

The frequency of central nervous system complications in the Cypriot cohort of ATTRV30M neuropathy transplanted patients

Authors: Efthimios Dardiotis, Savanna Andreou, Athina-Maria Aloizou, Elena Panayiotou, Vasileios Siokas, Mihaela Nedea Ioannou, Emmelia Vounou, Kyproula Christodoulou, George A. Tanteles, Demos Michaelides, Theodoros Kyriakides

Published in: Neurological Sciences | Issue 5/2020

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Abstract

Background

Hereditary transthyretin amyloidosis (ATTR) is a hereditary, sensorimotor and autonomic neuropathy caused by deposits of mutated transthyretin (TTR). The commonest TTR mutation is V30M (ATTRV30M) with patients usually living for about 10 years after disease onset. Liver transplantation (LT) until recently was considered the standard treatment.

Objective and methods

This study aims to assess the frequency of CNS complications in post-LT patients from the Cypriot cohort. Epidemiological data were collected for all genetically confirmed ATTRV30M neuropathy patients diagnosed at CING since 1992, and CNS-associated symptoms were assessed and evaluated by two neurology specialists.

Results

Out of the 48 transplanted patients, 10 (20.8%) presented with a CNS complication. All patients had ocular involvement, mainly glaucoma (7/10). Eight presented with transient focal neurological episodes (TFNEs), with expressive dysphasia being reported by four of them. The mean time of TFNE-emergence was 16.6 years after the LT. Three died from cerebral hemorrhage.

Conclusions

CNS complications in post-LT ATTRV30M patients are not rare and usually manifest themselves at a time that surpasses the mean time the patients would have survived without a LT. CNS involvement is associated with increased mortality, due to cerebral hemorrhage.
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Metadata
Title
The frequency of central nervous system complications in the Cypriot cohort of ATTRV30M neuropathy transplanted patients
Authors
Efthimios Dardiotis
Savanna Andreou
Athina-Maria Aloizou
Elena Panayiotou
Vasileios Siokas
Mihaela Nedea Ioannou
Emmelia Vounou
Kyproula Christodoulou
George A. Tanteles
Demos Michaelides
Theodoros Kyriakides
Publication date
01-05-2020
Publisher
Springer International Publishing
Published in
Neurological Sciences / Issue 5/2020
Print ISSN: 1590-1874
Electronic ISSN: 1590-3478
DOI
https://doi.org/10.1007/s10072-019-04176-9

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