Magnetic resonance imaging findings and clinical characteristics in mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy in a predominantly adult cohort

Autor(es)

Mendes Coelho, Vanessa C. ^[1] ; Morita-Sherman, Marcia ^{[1, 2]} ; Yasuda, Clarissa L. ^[1] ; Alvim, Marina M. K. ^[1] ; Amorim, Barbara Juarez ^[3] ; Tedeschi, Helder ^[1] ; Ghizoni, Enrico ^{[1, 4]} ; Rogerio, Fabio ; Cendes, Fernando ^[1]

Afiliação do(s) autor(es)

^[1] Univ Estadual Campinas, Dept Neurol, Campinas, SP - Brazil

^[2] Cleveland Clin Fdn, Epilepsy Ctr, 9500 Euclid Ave, Cleveland, OH 44195 - USA

^[3] Univ Estadual Campinas, Div Nucl Med, Dept Radiol, Campinas - Brazil

^[4] Univ Estadual Campinas, Dept Pathol, Campinas - Brazil

• Tipo de documento: Artigo Científico
• Fonte: Epilepsia; v. 62, n. 6 APR 2021.
• Citações Web of Science: 0

Resumo

Objective We aimed to better characterize the magnetic resonance imaging (MRI) findings of mild malformation of cortical development with oligodendroglial hyperplasia (MOGHE), a rare clinicopathological entity associated with pharmacoresistance recently described in patients with frontal lobe epilepsy. Methods We studied 12 patients who underwent epilepsy surgery and whose surgical specimens showed histopathological findings of MOGHE, characterized by preserved cortical lamination, blurred gray-white matter interface due to increased number of oligodendrocytes, and heterotopic neurons in the white matter. The age at MRI evaluation ranged from 11 to 58 years, except for one 4.5-year-old patient. Results Following a detailed MRI analysis using an in-house protocol, we found abnormalities in all cases. The lesion was circumscribed in the frontal lobe in six (50%) and in the temporal lobe in three (25%) patients. In the remaining three patients (25%), the lesion was multilobar (frontotemporal and temporoparieto-occipital). Cortical thickening was mild in all patients, except in the 4.5-year-old patient, who had pronounced cortical thickening and white matter blurring. We also identified cortical/subcortical hyperintense T2/fluid-attenuated inversion recovery signal associated with gray/white matter blurring in all but one patient. When present, cleft cortical dimple, and deep sulci aided in localizing the lesion. Overall, the MRI findings were like those in focal cortical dysplasia (FCD) Type IIa. Surgical outcome was excellent in five patients (Engel Class I in 25% and II in 17%). The remaining seven patients (58%) had worthwhile seizure reduction (Engle Class III). Incomplete lesion resection was significantly associated with worse outcomes. Significance MRI findings associated with MOGHE are similar to those described in FCD Type IIa. Although more frequent in the frontal lobe, MOGHE also occurred in the temporal lobe or involved multiple lobes. Multilobar or extensive MOGHE MRI lesions are associated with less favorable surgical outcomes. Because this is a rare condition, multicenter studies are necessary to characterize MOGHE further. (AU)

• Processo FAPESP: 19/08259-0 - Análise do transcriptoma do tecido cerebral de indivíduos epilépticos com displasia cortical focal ou com malformação leve do desenvolvimento cortical com hiperplasia oligodendroglial (MOGHE)
• Beneficiário: Fábio Rogério
• Modalidade de apoio: Auxílio à Pesquisa - Regular
• Processo FAPESP: 13/07559-3 - Instituto Brasileiro de Neurociência e Neurotecnologia - BRAINN
• Beneficiário: Fernando Cendes
• Modalidade de apoio: Auxílio à Pesquisa - Centros de Pesquisa, Inovação e Difusão - CEPIDs