title: Sedation with dexmedetomidine for conducting electroencephalogram in a patient with Angelman syndrome: a case report.
abstract: BACKGROUND
  Angelman syndrome is characterized by severe mental retardation and speech and seizure disorders. This rare genetic condition is associated with changes in GABAA receptor. Patients with Angelman syndrome need to be sedated during an electroencephalogram ordered for diagnostic purposes or evolutionary control. Dexmedetomidine, whose action is independent of GABA receptor, promotes a sleep similar to physiological sleep and can facilitate the performing of this examination in patients with Angelman syndrome.
  
  
  METHODS
  Female patient, 14 years old, with Angelman syndrome; electroencephalogram done under sedation with dexmedetomidine. The procedure was uneventful and bradycardia or respiratory depression was not recorded. The examination was successfully interpreted and epileptiform activity was not observed.
  
  
  CONCLUSIONS
  Dexmedetomidine promoted satisfactory sedation, was well tolerated and enabled the interpretation of the electroencephalogram in a patient with Angelman syndrome and seizure disorder.
authors.affiliation: Santa Casa de Belo Horizonte, Belo Horizonte, MG, Brazil. Electronic address: fernandesmagda@yahoo.com.br.
authors.forename: Magda Lourenço
authors.initials: ML
authors.lastname: Fernandes
authors.affiliation: Santa Casa de Belo Horizonte, Belo Horizonte, MG, Brazil.
authors.forename: Maria
authors.initials: M
authors.lastname: do Carmo Santos
authors.affiliation: Santa Casa de Belo Horizonte, Belo Horizonte, MG, Brazil.
authors.forename: Renato Santiago
authors.initials: RS
authors.lastname: Gomez
mesh_terms: D000293:Adolescent; D017204:Angelman Syndrome / Q000150:complications* / Q000503:physiopathology; D020927:Dexmedetomidine / Q000008:administration & dosage* / Q000009:adverse effects; D004569:Electroencephalography / Q000379:methods*; D005260:Female; D006801:Humans; D006993:Hypnotics and Sedatives / Q000008:administration & dosage* / Q000009:adverse effects
chemical_list: D006993:Hypnotics and Sedatives; D020927:Dexmedetomidine
keywords: Angelman syndrome; Deep sedation; Dexmedetomidina; Dexmedetomidine; EEG; Eletroencefalograma; Sedação profunda; Síndrome de Angelman