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Epilepsy Is Heterogeneous in Early-Life Tuberous Sclerosis Complex.
Ihnen, S Katie Z; Capal, Jamie K; Horn, Paul S; Griffith, Molly; Sahin, Mustafa; Bebin, E Martina; Wu, Joyce Y; Northrup, Hope; Krueger, Darcy A.
Afiliación
  • Ihnen SKZ; Division of Neurology, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio. Electronic address: katie.ihnen@cchmc.org.
  • Capal JK; Department of Neurology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.
  • Horn PS; Division of Neurology, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio.
  • Griffith M; Division of Neurology, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio.
  • Sahin M; Department of Neurology and F.M Kirby Neurobiology Center, Boston Children's Hospital, Harvard Medical School, Harvard University, Boston, Massachusetts.
  • Bebin EM; Department of Neurology, University of Alabama at Birmingham, Birmingham, Alabama.
  • Wu JY; Division of Neurology, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
  • Northrup H; Department of Pediatrics, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, Texas.
  • Krueger DA; Division of Neurology, Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio.
Pediatr Neurol ; 123: 1-9, 2021 10.
Article en En | MEDLINE | ID: mdl-34343869
BACKGROUND: Epilepsy in tuberous sclerosis complex (TSC) typically presents with early onset, multiple seizure types, and intractability. However, variability is observed among individuals. Here, detailed individual data on seizure characteristics collected prospectively during early life were used to define epilepsy profiles in this population. METHODS: Children aged zero to 36 months were followed longitudinally. Caregivers kept daily seizure diaries, including onset and daily counts for each seizure type. Patients with >70% seizure diary completion and >365 diary days were included. Developmental outcomes at 36 months were compared between subgroups. RESULTS: Epilepsy was seen in 124 of 156 (79%) participants. Seizure onset occurred from zero to 29.5 months; 93% had onset before age 12 months. Focal seizures and epileptic spasms were most common. Number of seizures (for median 897 days) ranged from 1 to 9128. Hierarchical clustering based on six metrics of seizure burden (age of onset, total seizures, ratio of seizure days to nonseizure days, seizures per seizure day, and worst seven- and 30-day stretches) revealed two distinct groups with broadly favorable and unfavorable epilepsy profiles. Subpopulations within each group showed clinically meaningful differences in seizure burden. Groups with higher seizure burden had worse developmental outcomes at 36 months. CONCLUSIONS: Although epilepsy is highly prevalent in TSC, not all young children with TSC have the same epilepsy profile. At least two phenotypic subpopulations are discernible based on seizure burden. Early and aggressive treatments for epilepsy in TSC may be best leveraged by targeting specific subgroups based on phenotype severity.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Esclerosis Tuberosa / Epilepsia Tipo de estudio: Etiology_studies / Observational_studies / Risk_factors_studies Límite: Child, preschool / Female / Humans / Infant / Male / Newborn Idioma: En Revista: Pediatr Neurol Asunto de la revista: NEUROLOGIA / PEDIATRIA Año: 2021 Tipo del documento: Article Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Esclerosis Tuberosa / Epilepsia Tipo de estudio: Etiology_studies / Observational_studies / Risk_factors_studies Límite: Child, preschool / Female / Humans / Infant / Male / Newborn Idioma: En Revista: Pediatr Neurol Asunto de la revista: NEUROLOGIA / PEDIATRIA Año: 2021 Tipo del documento: Article Pais de publicación: Estados Unidos