title: Wilson's disease with and without rapid eye movement sleep behavior disorder compared to healthy matched controls.
abstract: 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.
authors.affiliation: Division of Neurology and Neurosurgery, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Eneas de Carvalho Aguiar, 255, 5° andar, sala 5084, Pinheiros, 05403-900 Sao Paulo, Brazil; Sleep Laboratory, Pulmonary Division, InCor, University of Sao Paulo School of Medicine, Av. Dr. Eneas de Carvalho Aguiar, 44, Cerqueira Cesar, 05403-000 Sao Paulo, Brazil. Electronic address: gtribl@hotmail.com.
authors.forename: Gotthard G
authors.initials: GG
authors.lastname: Tribl
authors.affiliation: Division of Neurology and Neurosurgery, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Eneas de Carvalho Aguiar, 255, 5° andar, sala 5084, Pinheiros, 05403-900 Sao Paulo, Brazil.
authors.forename: Mateus C
authors.initials: MC
authors.lastname: Trindade
authors.affiliation: Sleep Laboratory, Pulmonary Division, InCor, University of Sao Paulo School of Medicine, Av. Dr. Eneas de Carvalho Aguiar, 44, Cerqueira Cesar, 05403-000 Sao Paulo, Brazil.
authors.forename: Thais
authors.initials: T
authors.lastname: Bittencourt
authors.affiliation: Sleep Laboratory, Pulmonary Division, InCor, University of Sao Paulo School of Medicine, Av. Dr. Eneas de Carvalho Aguiar, 44, Cerqueira Cesar, 05403-000 Sao Paulo, Brazil.
authors.forename: Geraldo
authors.initials: G
authors.lastname: Lorenzi-Filho
authors.affiliation: Division of Neurology and Neurosurgery, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Eneas de Carvalho Aguiar, 255, 5° andar, sala 5084, Pinheiros, 05403-900 Sao Paulo, Brazil.
authors.forename: Rosana
authors.initials: R
authors.lastname: Cardoso Alves
authors.affiliation: Division of Neurology and Neurosurgery, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Eneas de Carvalho Aguiar, 255, 5° andar, sala 5084, Pinheiros, 05403-900 Sao Paulo, Brazil.
authors.forename: Daniel
authors.initials: D
authors.lastname: Ciampi de Andrade
authors.affiliation: Division of Neurology and Neurosurgery, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Eneas de Carvalho Aguiar, 255, 5° andar, sala 5084, Pinheiros, 05403-900 Sao Paulo, Brazil.
authors.forename: Erich T
authors.initials: ET
authors.lastname: Fonoff
authors.affiliation: Division of Neurology and Neurosurgery, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Eneas de Carvalho Aguiar, 255, 5° andar, sala 5084, Pinheiros, 05403-900 Sao Paulo, Brazil.
authors.forename: Edson
authors.initials: E
authors.lastname: Bor-Seng-Shu
authors.affiliation: Division of Neurology and Neurosurgery, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Eneas de Carvalho Aguiar, 255, 5° andar, sala 5084, Pinheiros, 05403-900 Sao Paulo, Brazil.
authors.forename: Alexandre A
authors.initials: AA
authors.lastname: Machado
authors.affiliation: Department of Psychiatry, Minnesota Regional Sleep Disorders Center, Hennepin County Medical Center and University of Minnesota Medical School, 701 Park Ave., Minneapolis, MN 55415, USA.
authors.forename: Carlos H
authors.initials: CH
authors.lastname: Schenck
authors.affiliation: Division of Neurology and Neurosurgery, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Eneas de Carvalho Aguiar, 255, 5° andar, sala 5084, Pinheiros, 05403-900 Sao Paulo, Brazil.
authors.forename: Manoel J
authors.initials: MJ
authors.lastname: Teixeira
authors.affiliation: Division of Neurology and Neurosurgery, Hospital das Clinicas, University of Sao Paulo School of Medicine, Av. Dr. Eneas de Carvalho Aguiar, 255, 5° andar, sala 5084, Pinheiros, 05403-900 Sao Paulo, Brazil.
authors.forename: Egberto R
authors.initials: ER
authors.lastname: Barbosa
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D017286:Polysomnography; D020187:REM Sleep Behavior Disorder / Q000150:complications* / Q000503:physiopathology; D011795:Surveys and Questionnaires
keywords: Movement disorders; Parasomnias; Parkinsonian syndromes; Rapid eye movement sleep behavior disorder; Sleep disorders; Wilson's disease