Sedation with dexmedetomidine for conducting electroencephalogram in a patient with Angelman syndrome: a case report

Magda Lourenço Fernandes; Maria do Carmo Santos; Renato Santiago Gomez

doi:10.1016/j.bjane.2013.06.020

Clinical Informations

Sedation with dexmedetomidine for conducting electroencephalogram in a patient with Angelman syndrome: a case report

Sedação com dexmedetomidina para realização de eletroencefalograma em paciente portadora de síndrome de Angelmann: relato de caso

Magda Lourenço Fernandes; Maria do Carmo Santos; Renato Santiago Gomez

http://dx.doi.org/10.1016/j.bjane.2013.06.020 Braz J Anesthesiol, vol.66, n2, p.212-214, 2016

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Abstract

ABSTRACT INTRODUCTION: 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. CASE REPORT: 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. CONCLUSION: Dexmedetomidine promoted satisfactory sedation, was well tolerated and enabled the interpretation of the electroencephalogram in a patient with Angelman syndrome and seizure disorder.

Keywords

Angelman syndrome, EEG, Deep sedation, Dexmedetomidine

Resumo

RESUMO INTRODUÇÃO: a síndrome de Angelman (SA) é caracterizada por retardo mental grave, distúrbio da fala e desordem convulsiva. Essa condição genética rara está associada a alterações do receptor GABA-A. Pacientes portadores de SA necessitam ser sedados durante a feitura de eletroencefalograma (EEG), indicado para fins diagnósticos ou controle evolutivo. A dexmedetomidina, cuja ação independe do receptor GABA, promove sono semelhante ao fisiológico e pode viabilizar a feitura desse exame em pacientes com SA. RELATO DE CASO: paciente feminina, 14 anos, portadora de SA, fez EEG sob sedação com dexmedetomidina. O procedimento transcorreu sem intercorrências e não foi registrada bradicardia ou depressão respiratória. O exame foi interpretado com sucesso e atividade epileptiforme não foi observada. CONCLUSÃO: a dexmedetomidina promoveu sedação satisfatória, foi bem tolerada e possibilitou a interpretação do EEG em paciente com SA e desordem convulsiva.

Palavras-chave

Síndrome de Angelman, Eletroencefalograma, Sedação profunda, Dexmedetomidina

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Sedation with dexmedetomidine for conducting electroencephalogram in a patient with Angelman syndrome: a case report

Sedação com dexmedetomidina para realização de eletroencefalograma em paciente portadora de síndrome de Angelmann: relato de caso

Magda Lourenço Fernandes; Maria do Carmo Santos; Renato Santiago Gomez

Abstract

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