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Pediatric neurology · 2023

Extended Glasgow Outcome Scale to Evaluate the Functional Impairment of Patients With Subcortical Band Heterotopia: A Multicentric Cross-sectional Study.

Многоцентровое исследованиеСтатьяНазвание и аннотация: Перевод в очереди
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Toldo Irene, Brunello Francesco, Cavasin Paola, Nosadini Margherita, Sartori Stefano, Frigo Anna Chiara, Mai Roberto, Pelliccia Veronica, Mancardi Maria Margherita, Striano Pasquale, Severino Marisavina, Zara Federico, Rizzi Romana, Casellato Susanna, Di Rosa Gabriella, Mastrangelo Mario, Spalice Alberto, Budetta Mauro, De Palma Luca, Guerrini Renzo, Pruna Dario, Cordelli Duccio Maria, Sofia Vito, Papa Amanda, Chiesa Valentina, Ragona Francesca, Parisi Pasquale, D'Aniello Alfredo, Veggiotti Pierangelo, Dainese Filippo, Giordano Lucio, Licchetta Laura, Tinuper Paolo, D'Orsi Giuseppe, Cassina Matteo, Manara Renzo

Аннотация

BACKGROUND: Subcortical band heterotopia (SBH) is a rare malformation of the cortical development characterized by a heterotopic band of gray matter between cortex and ventricles. The clinical presentation typically includes intellectual disability and epilepsy.

PURPOSE: To evaluate if the Extended Glasgow Outcome Scale-pediatric version (EGOS-ped) is a feasible tool for evaluating the functional disability of patients with (SBH).

METHOD: Cross-sectional multicenter study of a cohort of 49 patients with SBH (female n = 30, 61%), recruited from 23 Italian centers.

RESULTS: Thirty-nine of 49 (80%) cases showed high functional disability at EGOS-ped assessment. In the poor result subgroup (EGOS-ped >3) motor deficit, language impairment, and lower intelligence quotient were more frequent (P < 0.001, P = 0.02, and P = 0.01, respectively); the age at epilepsy onset was remarkably lower (P < 0.001); and the prevalence of epileptic encephalopathy (West syndrome or Lennox-Gastaut-like encephalopathy) was higher (P = 0.04). The thickness and the extension of the heterotopic band were associated with EGOS-ped score (P < 0.01 and P = 0.02). Pachygyria was found exclusively among patients with poor outcome (P < 0.01).

CONCLUSIONS: The EGOS-ped proved to be a reliable tool for stratifying the functional disability of patients with SBH. According to this score, patients could be dichotomized: group 1 (80%) is characterized by a poor overall functionality with early epilepsy onset, thick heterotopic band, and pachygyria, whereas group 2 (20%) is characterized by a good overall functionality with later epilepsy onset and thinner heterotopic band.

Предметные рубрики MeSH

ИП Урсолов Владлен Сергеевич

ИНН 500118482352 · ОГРНИП 314500117700049

Реквизиты и контакты