Abstracts

Clinical, MRI, and EEG Characteristics and Seizure Outcome in a Series of 7 Patients Diagnosed with Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia and Epilepsy

Abstract number : 2.421
Submission category : 9. Surgery / 9C. All Ages
Year : 2023
Submission ID : 1499
Source : www.aesnet.org
Presentation date : 12/3/2023 12:00:00 AM
Published date :

Authors :
Presenting Author: Ivan Tapia Del Rio, MD – HMG Hospital, Coyoacan

Jesus Cienfuego Meza, MD – Resident, Neuropathology, National Neurology and Neurosurgery Institute; Oscar Ruben Contreras Vazquez, Student – Intern, Vascular and Skull base Surgery, National Neurology and Neurosurgery Institute, Mexico City; Carlos Francisco Hurtado Delgado, MD – Resident, Internal Medicine, Hospital Angeles del Carmen, Guadalajara Mexico; Juan Jesus Rodriguez Uranga, MD – Attending Neurologist, Epilepsy, Centro Neurologia Avanzada, Sevilla; Erika Brust Mascher, MD – Attending Physicians, Epilepsy Surgery, HMG Hospital, Coyoacan; Eduardo Sevilla Amador, Student – Student, School of Medicine, Universidad Marista de Mérida; Miroslava Ortiz Cuevas, Student – Medical – Universidad de Guanajuato; Mario Arturo Alonso Vanegas, Acad. MD FAES – Head Professor and Surgeon, Epilepsy Surgery, HMG Hospital, coyoacan

Rationale:
Mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE) is an emerging, highly epileptogenic pathological entity in drug-resistant epilepsy. While defining its clinical features remains an ongoing process, research has demonstrated that patients with MOGHE tend to have lesions primarily impacting the frontal lobe. Still, it’s important to stipulate that this may not always be the case. Despite a scarcity of reported cases in literature, knowledge about this entity continues to expand. Consequently, additional clinical aspects are being evaluated for their correlation with MOGHE. The following study presents a case series of seven patients who underwent epilepsy surgery with MOGHE as the neuropathological diagnosis in their resected tissue. This could serve as valuable knowledge for physicians concerning the growing importance of MOGHE in surgical epilepsy candidates and offer a more comprehensive understanding of these patients.

Methods:
We conducted a comprehensive descriptive analysis of clinical characteristics, EEG patterns, seizure semiology, radiological findings, surgical outcomes, and neuropathological details in a cohort of five Mexican patients and two Spanish patients diagnosed with MOGHE.

Results:
Our study demonstrated a higher prevalence of males (five males) compared to females (two females). The cohort comprised four adults and three children who underwent surgery. The average age at the time of surgery was 18.5 years, ranging from 4-42 years. The onset of seizures occurred at an average age of seven years, with a range of two to 16 years. In terms of localization, the epileptic zone was predominantly in the frontal lobe, with two cases on the left side and four on the right side. Additionally, one case presented with multilobar epilepsy involving the left fronto-temporal region. All patients had a pre surgical diagnosis of focal cortical dysplasia and an epilepsy surgical Engel 1A (Table 1) The seizure classification is presented in Table 2. MRI findings are similar to FCD IIB, cortical thickening, poor distinction between the grey- white matter junction specifically in T2 and FLAIR sequences and presented in Image 2. MRI finding in MOGHE have been classified by Hartlieb et al. 

Conclusions:
MOGHE represents a unique clinical- pathological entity characterized by subtle visible alterations on MRI scans. Despite its clinical resemblance to FCD IIA and radiological similarity to FCD IIB, MOGHE stands apart as a distinct anatomopathological entity. Surgical outcomes tend to be highly favorable regardless of the size of the lesion. Intraoperative ECoG plays a crucial role in identifying and delineating the Epileptogenic Zone (EZ), thereby facilitating optimal resection for improved post-surgical outcomes. When faced with a case of drug-resistant frontal epilepsy accompanied by early-onset seizures and positive MRI findings, it is prudent to consider the possibility of MOGHE.

Funding: None

Surgery