title: Wilson's disease presenting as rapid eye movement sleep behavior disorder: a possible window to early treatment.
abstract: OBJECTIVE
  To describe characteristics of REM sleep behavior disorder in Wilson's disease.
  
  
  METHODS
  Questionnaire-based interviews (patients and relatives), neurological examinations, two-week prospective dream-diary, video-polysomnography, transcranial sonography, MRI.
  
  
  RESULTS
  Four Wilson's disease cases with REM sleep behavior disorder were described; three had REM sleep behavior disorder as initial symptom. All showed mesencephalic tegmental/tectal sonographic hyperechogenicities and two presented ponto-mesencephalic tegmental MRI hyperintensities.
  
  
  CONCLUSIONS
  This first description of REM sleep behavior disorder in Wilson's disease in literature documents REM sleep behavior disorder as a possible presenting symptom of Wilson's disease and adds further evidence to the parallelism of Parkinson's disease and Wilson's disease in phenotype and brainstem topography, which ought to be further studied. REM sleep behavior disorder has prognostic relevance for neurodegeneration in α-synucleinopathies. In Wilson's disease, usefulness of early diagnosis and treatment are already well established. REM sleep behavior disorder in Wilson's disease offers a possible theoretical model for potential early treatment in this extrapyramidal and brainstem paradigm syndrome, previewing the possibility of neuroprotective treatment for REM sleep behavior disorder in "pre-clinical" Parkinson's disease.
authors.affiliation: Divisão de Neurologia e Neurocirurgia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
authors.forename: Gotthard G
authors.initials: GG
authors.lastname: Tribl
authors.affiliation: Divisão de Neurologia e Neurocirurgia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
authors.forename: Edson
authors.initials: E
authors.lastname: Bor-Seng-Shu
authors.affiliation: Divisão de Neurologia e Neurocirurgia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
authors.forename: Mateus C
authors.initials: MC
authors.lastname: Trindade
authors.affiliation: Departamento de Radiologia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
authors.forename: Leandro T
authors.initials: LT
authors.lastname: Lucato
authors.affiliation: Divisão de Neurologia e Neurocirurgia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
authors.forename: Manoel J
authors.initials: MJ
authors.lastname: Teixeira
authors.affiliation: Divisão de Neurologia e Neurocirurgia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
authors.forename: Egberto R
authors.initials: ER
authors.lastname: Barbosa
mesh_terms: D000328:Adult; D042241:Early Diagnosis; D005260:Female; D006527:Hepatolenticular Degeneration / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D019636:Neurodegenerative Diseases / Q000503:physiopathology; D009460:Neurologic Examination; D017286:Polysomnography; D020187:REM Sleep Behavior Disorder / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D011795:Surveys and Questionnaires; D017585:Ultrasonography, Doppler, Transcranial; D055815:Young Adult