Pacemaker Implantation in Juvenile Neuronal Ceroid Lipofuscinosis (CLN3)-A Long-Term Follow-Up Study.
CLN3
Juvenile Neuronal Ceroid Lipofuscinosis
neurodegenerative diseases
pacemaker
sick sinus syndrome
Journal
Frontiers in neurology
ISSN: 1664-2295
Titre abrégé: Front Neurol
Pays: Switzerland
ID NLM: 101546899
Informations de publication
Date de publication:
2022
2022
Historique:
received:
30
12
2021
accepted:
02
02
2022
entrez:
31
3
2022
pubmed:
1
4
2022
medline:
1
4
2022
Statut:
epublish
Résumé
It is well documented that deteriorating heart function due to deposition of ceroid lipopigment is a significant co-morbidity in Juvenile Neuronal Ceroid Lipofuscinosis (CLN3 disease) although the exact disease mechanisms remain unknown in any NCL form. An increasing frequency of cardiac conduction disorders including severe bradycardia and sinus arrest is seen in the late teens, as is a left ventricular hypertrophy in the early 20s. Only a few case reports of pacemaker implantation have been published, and so far, no long-term follow-up study exists. As new treatment options emerge, more patients will live longer and the need for pacemaker will likely increase, why knowledge of long-term outcome is needed. In the present study, we present the course of six patients from the original Danish CLN3-heart population study (
Identifiants
pubmed: 35356463
doi: 10.3389/fneur.2022.846240
pmc: PMC8960059
doi:
Types de publication
Journal Article
Langues
eng
Pagination
846240Informations de copyright
Copyright © 2022 Handrup, Mølgaard, Andersen and Ostergaard.
Déclaration de conflit d'intérêts
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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