TY - JOUR
T1 - Wilson's disease with and without rapid eye movement sleep behavior disorder compared to healthy matched controls
AU - Tribl, Gotthard G.
AU - Trindade, Mateus C.
AU - Bittencourt, Thais
AU - Lorenzi-Filho, Geraldo
AU - Cardoso Alves, Rosana
AU - Ciampi de Andrade, Daniel
AU - Fonoff, Erich T.
AU - Bor-Seng-Shu, Edson
AU - Machado, Alexandre A.
AU - Schenck, Carlos H.
AU - Teixeira, Manoel J.
AU - Barbosa, Egberto R.
N1 - Funding Information:
The authors would like to acknowledge the support of the São Paulo Research Foundation (FAPESP, Fundação de Amparo à Pesquisa do Estado de São Paulo), grant 2012/05403-3 . The sponsor had no influence on the conduct of the research and/or the content of the article.
Publisher Copyright:
© 2015 Elsevier B.V.
PY - 2015/6/10
Y1 - 2015/6/10
N2 - Objective: Quantitative data are reported on rapid eye movement (REM) sleep behavior disorder (RBD) in a cohort of predominantly neurological Wilson's disease (WD). Methods: A total of 41 patients with WD and 41 healthy, age- and gender-matched controls were studied by conducting face-to-face interviews, neurological and clinical examinations, laboratory tests, and WD- and RBD-specific scales. Video-polysomnography and quantification of REM sleep without atonia (RWA) were conducted in 35 patients and 41 controls. Results: Patients with WD showed significantly worse sleep quality, less sleep efficiency, increased wakefulness after sleep onset, and more arousals compared to healthy controls. Five patients with WD (four women) fulfilled the diagnostic criteria for RBD with significantly higher values in RWA, RBD Questionnaire-Hong Kong, and RBD Screening Questionnaire compared to patients with WD without RBD. In three patients with WD, RBD had manifested before any other symptom that could be attributed to WD. Percentage of RWA was significantly lower in WD without RBD than in WD with RBD, but still significantly increased compared to controls. Conclusions: RBD can be comorbid with WD. RWA is commonly present in WD, both in the presence or absence of clinical RBD. A causal connection is possible, though retrospective determination of RBD onset and the low number of patients do not allow a definitive conclusion at this point. However, screening for WD in idiopathic RBD is available at low cost and is recommended. Early-stage copper chelation therapy provides a highly effective treatment to prevent further WD manifestations and might also control the comorbid RBD.
AB - Objective: Quantitative data are reported on rapid eye movement (REM) sleep behavior disorder (RBD) in a cohort of predominantly neurological Wilson's disease (WD). Methods: A total of 41 patients with WD and 41 healthy, age- and gender-matched controls were studied by conducting face-to-face interviews, neurological and clinical examinations, laboratory tests, and WD- and RBD-specific scales. Video-polysomnography and quantification of REM sleep without atonia (RWA) were conducted in 35 patients and 41 controls. Results: Patients with WD showed significantly worse sleep quality, less sleep efficiency, increased wakefulness after sleep onset, and more arousals compared to healthy controls. Five patients with WD (four women) fulfilled the diagnostic criteria for RBD with significantly higher values in RWA, RBD Questionnaire-Hong Kong, and RBD Screening Questionnaire compared to patients with WD without RBD. In three patients with WD, RBD had manifested before any other symptom that could be attributed to WD. Percentage of RWA was significantly lower in WD without RBD than in WD with RBD, but still significantly increased compared to controls. Conclusions: RBD can be comorbid with WD. RWA is commonly present in WD, both in the presence or absence of clinical RBD. A causal connection is possible, though retrospective determination of RBD onset and the low number of patients do not allow a definitive conclusion at this point. However, screening for WD in idiopathic RBD is available at low cost and is recommended. Early-stage copper chelation therapy provides a highly effective treatment to prevent further WD manifestations and might also control the comorbid RBD.
KW - Movement disorders
KW - Parasomnias
KW - Parkinsonian syndromes
KW - Rapid eye movement sleep behavior disorder
KW - Sleep disorders
KW - Wilson's disease
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U2 - 10.1016/j.sleep.2015.09.003
DO - 10.1016/j.sleep.2015.09.003
M3 - Article
C2 - 26763676
AN - SCOPUS:84952690908
SN - 1389-9457
VL - 17
SP - 179
EP - 185
JO - Sleep Medicine
JF - Sleep Medicine
ER -