Post-Operative Seizure Freedom Need not be Elusive in Mild Oligodendroglial Hyperplasia and Epilepsy (MOGHE)

Chatterjee, A. K.; Mundlamuri, Ravindranadh Chowdary; Asranna, Ajay; Kenchaiah, Raghavendra; Bhargava, G K; Mariyappa, N.; Mhatre, Radhika; Kulanthaivelu, Karthik; Mangalore, Sandhya; Nagaraj, Chandana; Bharath, Rose Dawn; Saini, Jitender; Sadashiva, Nishanth; Arivazhagan, Arimappamagan; Rao, Malla Bhaskara; Mahadevan, Anita; Sinha, Sanjib

doi:10.4103/aian.aian_1155_20

Cited by 2 publications

(2 citation statements)

References 9 publications

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“…The patients had had a relatively normal developmental trajectory and manifested hyperkinetictype seizures with distal stereotypies and semivoluntarynaturalistic, repetitive automatisms, corresponding to "group 3" hyperkinetic frontal lobe seizures as described by Bonini et al 16 In their study, stereo-EEG investigations had shown this semiological pattern to be associated with an epileptogenic network involving the rostral prefrontal, ventrolateral, and rostral cingulate cortices. 16 Previous studies on Frontal Lobe MOGHE report mainly "spasms," tonic, and only rarely "hyperkinetic" seizures, 4,5,8 the last ones usually with agitated/phrenetic automatisms (corresponding to Bonini group 4, and indicative of a more anterior, basal frontal epileptogenic network).…”

Section: Discussionmentioning

confidence: 99%

“…Mild Malformation with Oligodendroglial Hyperplasia (MOGHE) has been recently described, as a distinct clinicopathologic entity in adult and pediatric epilepsy patients, most of them with Frontal Lobe Epilepsy [1][2][3][4][5][6][7][8] as well as with "Temporal plus" 9 occipitotemporal, 10,11 and Epileptic Encephalopathy 7 electroclinical phenotypes. Although MRI features in some cases suggest FCD IIa type, 3 on histopathologic grounds MOGHE is an entity distinct from FCD, its cardinal features consist of normal cortical architecture, increased oligodendrocyte numbers and proliferative activity in the deep cortical layers and superficial white matter, heterotopic neurons in the white matter, as well as cortical-white matter blurring and hypomyelinated white matter areas.…”

Section: Introductionmentioning

confidence: 99%

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Frontal lobe epilepsy and mild malformation with oligodendroglial hyperplasia: Further observations on electroclinical and imaging phenotypes, and surgical perspectives

Garganis

Gkiatis

Maletić

et al. 2023

Epileptic Disorders

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Objective Mild malformation with oligodendroglial hyperplasia (MOGHE) is a recently described clinicopathologic entity, associated with drug‐resistant epilepsy and extensive epileptogenic networks. Knowledge is accumulating about particular electroclinical phenotypes, correlations with imaging, and potential prognostic significance for surgical outcomes. The study adds relevant information by documenting the presence of a hyperkinetic frontal lobe seizure phenotype in adolescents and an epileptic encephalopathy phenotype in young children. Methods Five cases were subjected to a structured presurgical evaluation protocol, including EEG‐FMRI, chronic and acute invasive EEG, subjected to frontal lobe surgery with postoperative follow‐up between 15 months and 7 years. Results In the two adult cases, surface EEG demonstrated lateralized widespread frontal lobe epileptogenicity and hyperkinetic semiological features. MRI demonstrated cortical white matter blurring and deeper white matter abnormalities. EEG‐FMRI suggested concordant frontal lobe involvement. iEEG demonstrated a widespread frontal lobe epilepsy network. The three young children demonstrated a diffuse epileptic encephalopathy phenotype, with nonlocalizing, nonlateralizing surface EEG, and “spasms” as the main seizure type. MRI demonstrated extensive frontal lobe subcortical gray and white matter abnormalities, consistent with MOGHE literature for this age, while EEG‐FMRI, in 2/3, demonstrated concordant frontal lobe involvement. They did not undergo chronic iEEG, and the resection was assisted by acute intraoperative ECoG. All cases were subjected to extensive frontal lobectomies with Engel class IA (2/5), IB (1/5), and IIB (2/5) outcomes. Significance The study confirms the presence of frontal lobe epilepsy and epileptic encephalopathy phenotypes, in accordance with epilepsy phenotypes already described in MOGHE literature. Presurgical evaluation studies, including EEG‐FMRI, can provide strong lateralizing and localizing evidence of the epileptogenic networks involved. All responded favorably to extensive frontal lobe resections, despite widespread epileptic activity recorded by surface and intracranial EEG pre‐ and postoperatively; an epileptic encephalopathy phenotype, in the first years of life, should not discourage such a resection.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Frontal lobe epilepsy and mild malformation with oligodendroglial hyperplasia: Further observations on electroclinical and imaging phenotypes, and surgical perspectives

Garganis

Gkiatis

Maletić

et al. 2023

Epileptic Disorders

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Clinical characteristics and multimodal imaging can help diagnosing and treating mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy

Dimova,

Metodiev,

Todorov

et al. 2024

Epileptic Disorders

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ObjectiveMild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE) is a recently described, histopathologically and molecularly defined (SLC35A2‐mutated) type of cortical malformation. Although increasingly recognized, the diagnosis of MOGHE remains a challenge. We present the characteristics of the first six patients diagnosed in Bulgaria, with the aim to facilitate identification, proper presurgical evaluation, and surgical treatment approach in this disease.MethodsRevision of histopathological specimens of 202 patients operated on for drug‐resistant focal epilepsy identified four cases with MOGHE. Another two were suggested, based on clinical characteristics and subsequently, were histologically confirmed. Sanger SLC35A2 sequencing on paraffin‐embedded or fresh‐frozen brain tissue was performed. Analysis of seizure types, neuropsychological profiles, electroencephalographic (EEG), imaging features and epilepsy surgery outcomes was done.ResultsThree out of the six cases (50%) harbored pathogenic SLC35A2 mutations. One patient had a heterozygous somatic variant with uncertain significance. Clinical characteristics included epilepsy onset in infancy (in 100% under 3 years of age), multiple seizure types, and moderate or severe intellectual/developmental delay. Epileptic spasms with hypsarrhythmia on EEG were the initial seizure type in five patients. The subsequent seizure types resembled those in Lennox–Gastaut syndrome. The majority of the patients (n = 4) presented prominent and persisting autistic features. Magnetic resonance imaging (MRI) showed multilobar (n = 6) and bilateral (n = 3) lesions, affecting the frontal lobes (n = 5; bilaterally in three) and characterized by increased signal on T2/fluid‐attenuated inversion recovery (FLAIR). Voxel‐based morphometric MRI post‐processing and positron emission tomography helped determining the localization and extent of the lesions and presumed epileptogenic zones. After surgery, four patients (66.7%) were seizure‐free ≥2 years. Interestingly, all seizure‐free patients carried somatic SLC35A2‐alterations.SignificanceEpileptic spasms, early prominent neuropsychological disturbances, MRI‐T2/FLAIR hyperintense lesions with cortico‐subcortical blurring, frequently multilobar and especially frontal, can preoperatively help to suspect MOGHE. Epilepsy surgery is still the only successful treatment option in MOGHE.

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Post-Operative Seizure Freedom Need not be Elusive in Mild Oligodendroglial Hyperplasia and Epilepsy (MOGHE)

Cited by 2 publications

References 9 publications

Frontal lobe epilepsy and mild malformation with oligodendroglial hyperplasia: Further observations on electroclinical and imaging phenotypes, and surgical perspectives

Frontal lobe epilepsy and mild malformation with oligodendroglial hyperplasia: Further observations on electroclinical and imaging phenotypes, and surgical perspectives

Clinical characteristics and multimodal imaging can help diagnosing and treating mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy

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