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

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. 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). Cross-sectional multicenter study of a cohort of 49 patients with SBH (female n = 30, 61%), recruited from 23 Italian centers. 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). 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. Copyright © 2023 Elsevier Inc. All rights reserved.

Citation

Irene Toldo, Francesco Brunello, Paola Cavasin, Margherita Nosadini, Stefano Sartori, Anna Chiara Frigo, Roberto Mai, Veronica Pelliccia, Maria Margherita Mancardi, Pasquale Striano, Marisavina Severino, Federico Zara, Romana Rizzi, Susanna Casellato, Gabriella Di Rosa, Mario Mastrangelo, Alberto Spalice, Mauro Budetta, Luca De Palma, Renzo Guerrini, Dario Pruna, Duccio Maria Cordelli, Vito Sofia, Amanda Papa, Valentina Chiesa, Francesca Ragona, Pasquale Parisi, Alfredo D'Aniello, Pierangelo Veggiotti, Filippo Dainese, Lucio Giordano, Laura Licchetta, Paolo Tinuper, Giuseppe D'Orsi, Matteo Cassina, Renzo Manara. Extended Glasgow Outcome Scale to Evaluate the Functional Impairment of Patients With Subcortical Band Heterotopia: A Multicentric Cross-sectional Study. Pediatric neurology. 2023 Apr;141:58-64

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PMID: 36773408

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