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Sudden Unexpected Death in Epilepsy Among Patients With Benign Childhood Epilepsy With Centrotemporal Spikes.
Doumlele, Kyra; Friedman, Daniel; Buchhalter, Jeffrey; Donner, Elizabeth J; Louik, Jay; Devinsky, Orrin.
Afiliação
  • Doumlele K; Department of Neurology, New York University School of Medicine, New York.
  • Friedman D; Department of Neurology, New York University School of Medicine, New York.
  • Buchhalter J; Department of Pediatrics, Alberta Children's Hospital, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada3Department of Clinical Neurosciences, Alberta Children's Hospital, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.
  • Donner EJ; Division of Neurology, Department of Paediatrics, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada.
  • Louik J; Department of Neurology, New York University School of Medicine, New York.
  • Devinsky O; Department of Neurology, New York University School of Medicine, New York.
JAMA Neurol ; 74(6): 645-649, 2017 06 01.
Article em En | MEDLINE | ID: mdl-28384699
ABSTRACT
Importance Children with benign epilepsy with centrotemporal spikes (BECTS) have traditionally been considered to have a uniformly good prognosis. However, benign may be a misnomer because BECTS is linked to cognitive deficits, a more severe phenotype with intractable seizures, and the potential for sudden unexpected death in epilepsy (SUDEP).

Objective:

To determine if cases of BECTS are present in the North American SUDEP Registry (NASR). Design, Setting, and

Participants:

The NASR is a clinical and biospecimen repository established in 2011 to promote SUDEP research. The NASR database, which includes medical records, results of electroencephalographic tests, and interviews with family members of patients with epilepsy who died suddenly without other identifiable causes of death, was queried from June 3, 2011, to June 3, 2016, for cases of BECTS. The patients with epilepsy had died suddenly without other identifiable causes of death (eg, drowning, trauma, exposure to toxic substances, or suicide); SUDEP classification was determined by the consensus of 2 epileptologists. Main Outcomes and

Measures:

Cases of SUDEP among children who received a diagnosis of BECTS among patients reported in the NASR.

Results:

Three boys (median age at death, 12 years; range, 9-13 years) who received a diagnosis of BECTS by their pediatric epileptologist or neurologists were identified among 189 cases reported in the NASR. The median age of epilepsy onset was 5 years (range, 3-11 years), and the median duration of epilepsy was 4 years (range, 1-10 years). Two deaths were definite SUDEP, and 1 was probable SUDEP. Independent review of clinical and electroencephalographic data supported the diagnosis of BECTS in all 3 patients. None of the patients was prescribed antiseizure drugs, either owing to physician recommendation or mutual decision by the physician and parents. All 3 patients were found dead in circumstances typical of SUDEP. The 3 patients spanned the spectrum of BECTS severity 1 had only a few seizures, 1 had more than 30 focal motor seizures, and 1 had 4 witnessed generalized tonic-clonic seizures and approximately 30 suspected generalized tonic-clonic seizures. Conclusions and Relevance Sudden unexpected death in epilepsy is a very rare outcome in BECTS that clinicians should consider discussing in appropriate circumstances and possibly factoring into treatment decisions.
Assuntos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Sistema de Registros / Epilepsia Rolândica / Morte Súbita Tipo de estudo: Guideline / Prognostic_studies Limite: Adolescent / Child / Humans / Male País/Região como assunto: America do norte Idioma: En Revista: JAMA Neurol Ano de publicação: 2017 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Sistema de Registros / Epilepsia Rolândica / Morte Súbita Tipo de estudo: Guideline / Prognostic_studies Limite: Adolescent / Child / Humans / Male País/Região como assunto: America do norte Idioma: En Revista: JAMA Neurol Ano de publicação: 2017 Tipo de documento: Article