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Wilson's disease presenting as rapid eye movement sleep behavior disorder: a possible window to early treatment.
Tribl, Gotthard G; Bor-Seng-Shu, Edson; Trindade, Mateus C; Lucato, Leandro T; Teixeira, Manoel J; Barbosa, Egberto R.
Afiliação
  • Tribl GG; Divisão de Neurologia e Neurocirurgia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
  • Bor-Seng-Shu E; Divisão de Neurologia e Neurocirurgia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
  • Trindade MC; Divisão de Neurologia e Neurocirurgia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
  • Lucato LT; Departamento de Radiologia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
  • Teixeira MJ; Divisão de Neurologia e Neurocirurgia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
  • Barbosa ER; Divisão de Neurologia e Neurocirurgia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, SP, Brazil.
Arq Neuropsiquiatr ; 72(9): 653-8, 2014 Sep.
Article em En | MEDLINE | ID: mdl-25120201
OBJECTIVE: To describe characteristics of REM sleep behavior disorder in Wilson's disease. METHOD: 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. CONCLUSION: 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.
Assuntos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Transtorno do Comportamento do Sono REM / Degeneração Hepatolenticular Tipo de estudo: Diagnostic_studies / Prognostic_studies / Screening_studies Limite: Adult / Female / Humans / Male Idioma: En Revista: Arq Neuropsiquiatr Ano de publicação: 2014 Tipo de documento: Article País de afiliação: Brasil País de publicação: Alemanha

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Transtorno do Comportamento do Sono REM / Degeneração Hepatolenticular Tipo de estudo: Diagnostic_studies / Prognostic_studies / Screening_studies Limite: Adult / Female / Humans / Male Idioma: En Revista: Arq Neuropsiquiatr Ano de publicação: 2014 Tipo de documento: Article País de afiliação: Brasil País de publicação: Alemanha