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@ARTICLE{Dieter:268503,
author = {Dieter, Munker and Peikert, Kevin and Tobias, Veit and
Hermann, Andreas and Lorenz, Nowak and Kathrin, Kahnert and
Nikolaus, Kneidinger and Juergen, Behr and Jan, Remi and
Adrian, Danek},
title = {{P}olysomnographic findings in the ultra-rare {M}c{L}eod
syndrome: further documentation of sleep apnea as a possible
feature.},
journal = {Journal of clinical sleep medicine},
volume = {20},
number = {3},
issn = {1550-9389},
address = {Westchester, Ill.},
publisher = {[Verlag nicht ermittelbar]},
reportid = {DZNE-2024-00249},
pages = {339 - 344},
year = {2024},
abstract = {McLeod syndrome is a very rare multisystemic
neurodegenerative disease linked to mutations in the XK
gene. It has cardiac, neurologic, and neuromuscular
manifestations and shares similarities with Huntington's
disease. The aim of this study was to evaluate sleep
patterns of patients affected by McLeod syndrome.This
retrospective case series of four males who underwent
diagnostic polysomnography (mean age 53.8 ± 2.5 years)
includes self-reported and objective evaluation of sleep
using the Epworth Sleepiness Scale, genetic tests,
documentation of clinical course and features, and
laboratory-based full-night attended
video-polysomnography.In three out of four patients, an
Epworth Sleepiness Scale score ≥ 7 was evident. The
average apnea-hypopnea index was 45.0 ± 19.0, with
predominantly obstructive phenotype in three patients and
predominant central events (central sleep apnea syndrome) in
one patient. A significantly increased periodic limb
movement index during sleep was observed in all patients.
All patients tolerated continuous positive airway pressure
or pressure controlled therapy.Polysomnography of all
patients confirmed sleep apnea syndrome as a feature of
McLeod syndrome. Three patients were diagnosed with
obstructive sleep apnea and one with central sleep apnea
syndrome. In addition, periodic limb movement index was
increased in all patients.Dieter M, Kevin P, Tobias V, et
al. Polysomnographic findings in the ultra-rare McLeod
syndrome: further documentation of sleep apnea as a possible
feature. J Clin Sleep Med. 2024;20(3):339-344.},
keywords = {Male / Humans / Middle Aged / Sleep Apnea, Central /
Neurodegenerative Diseases / Retrospective Studies /
Sleepiness / Sleep Apnea Syndromes: complications / Sleep
Apnea Syndromes: diagnosis / Documentation /
Neuroacanthocytosis / McLeod syndrome (Other) / XK gene
(Other) / central sleep apnea (Other) / obstructive sleep
apnea (Other) / periodic limb movements (Other) /
polysomnography (Other)},
cin = {AG Hermann},
ddc = {610},
cid = {I:(DE-2719)1511100},
pnm = {353 - Clinical and Health Care Research (POF4-353)},
pid = {G:(DE-HGF)POF4-353},
typ = {PUB:(DE-HGF)16},
pmc = {pmc:PMC11019222},
pubmed = {pmid:37811906},
doi = {10.5664/jcsm.10854},
url = {https://pub.dzne.de/record/268503},
}