scholarly journals A case of mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE)

2021 ◽  
Vol 12 (3) ◽  
pp. 93-100
Author(s):  
V. S. Khalilov ◽  
A. N. Kislyakov ◽  
T. V. Basalay ◽  
A. V. Levov ◽  
A. A. Kholin
Keyword(s):  
White Matter ◽  
Frontal Lobe ◽  
Focal Cortical Dysplasia ◽  
Epileptiform Activity ◽  
Cortical Development ◽  
Dynamic Mri ◽  
Subtotal Resection ◽  
Pharmacoresistant Epilepsy ◽  
Malformation Of Cortical Development ◽  
The Right

Recently, in the scientist community of specialists dealing with structural epilepsy, it has been noticed an increasing interest in a special form of cortical development disorder not to be included in the ILAE Classification of the epilepsies the 2017 revision. It is so-called mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE). There are a number of publications devoted to the neuroimaging features of MOGHE, which are possible to distinguish from other epileptogenic substrates in comparisons with clinical/anamnestic data and dynamic observation. Our paper describes the case of a patient under 6 years suffering from pharmacoresistant epilepsy with histologically confirmed MOGHE, and having undergone the procedure of epileptic surgery. MRI showed an increased intensity of the T2/FLAIR signal from the white matter in combination with signs of laminar hyperintensivity, regional sulcation disturbance, smoothness of gray-white matter demarcation in the right frontal lobe. A signal intensification from the white matter with the formation similarity of the «transmantl» sign and further pronounced smoothness of the gray-white matter demarcation was observed on dynamic MRI. These changes were estimated as focal cortical dysplasia. Pre-surgical examination revealed a correlation of epileptiform activity with MRI changes. The subtotal resection of the right frontal lobe and the morphological conclusion established the presence of MOGHE was performed.

scholarly journals Epileptic Patient with Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia and Epilepsy (MOGHE): A Case Report and Review of the Literature

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
A. Verentzioti ◽  
I. Blumcke ◽  
A. Alexoudi ◽  
P. Patrikelis ◽  
A. Siatouni ◽  
...  
Keyword(s):  
Case Report ◽  
White Matter ◽  
Frontal Lobe ◽  
Epileptic Patient ◽  
Focal Epilepsy ◽  
Cortical Development ◽  
Resected Specimen ◽  
Frontal Lobe Epilepsy ◽  
Review Of The Literature ◽  
Malformation Of Cortical Development

Introduction. There is an emerging interest in the literature about MOGHE (Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia and Epilepsy). We report the case of an epileptic patient with MOGHE. Case Report. A 33-year-old male patient was suffering from refractory focal epilepsy since adolescence. MRI demonstrated increased T2/FLAIR signal intensity of right frontal lobe. Presurgical evaluation led to definition of epileptogenic network in a specific area of right frontal lobe. The resected specimen revealed MOGHE. Discussion. MOGHE appears to be a brain entity which shares some unique histopathological features. Review of the literature is in accordance with our patient’s findings. The major neuropathological finding consists of areas with blurred gray-white matter boundaries due to heterotopic neurons in white matter and increased numbers of subcortical oligodendroglial cells with increased proliferation. MR abnormalities are present in T2/FLAIR sequences. It concerns patients with refractory frontal lobe epilepsy and appears to associate with unfavourable postsurgical outcome in seizure control. Conclusion. More cases are needed in order to establish more data about this distinct entity in frontal lobe epilepsy. This could be valuable knowledge to patients and doctors concerning expectations or management of undesirable outcome in frontal lobe epilepsy surgery.

Pathologic Features of Dysplasia and Accompanying Alterations Observed in Surgical Specimens from Patients with Intractable Epilepsy

2004 ◽  
Vol 19 (3) ◽  
pp. 341-350 ◽  
Author(s):  
Akiyoshi Kakita ◽  
Shigeki Kameyama ◽  
Shintaro Hayashi ◽  
Hiroshi Masuda ◽  
Hitoshi Takahashi
Keyword(s):  
White Matter ◽  
Focal Cortical Dysplasia ◽  
Molecular Layer ◽  
Cortical Development ◽  
Neurofibrillary Tangle ◽  
Intractable Epilepsy ◽  
Clinical Findings ◽  
Cortical Layer ◽  
Pathologic Features ◽  
Cortical Dysplasias

Malformations caused by abnormalities of cortical development, or cortical dysplasias, were examined in surgical specimens from 108 patients with medically intractable epilepsy to determine the scope of histopathologic changes. The relevance of the clinical findings was also evaluated. Various types and degrees of dysplastic features were observed in various combinations, including architectural abnormalities, an increased number of neurons in the molecular layer and/or cortical layer II, neuronal clustering, an increased number of satellite oligodendrocytes, abnormal gyration, single and/or aggregates of heterotopic neurons in the white matter, and the appearance of cytologically abnormal cells, such as giant or dysmorphic neurons and balloon cells. In the temporal lobe specimens, microdysgenesis (corresponding to mild malformations caused by abnormalities of cortical development and type IA/B focal cortical dysplasias) was more frequently observed than Taylor-type focal cortical dysplasia (type IIA/B), whereas in the frontal lobe specimens, the frequency of occurrence of both types was even. The ages at seizure onset and surgery of patients with the latter type were significantly lower than those of patients with the former. On the other hand, prominent astrocytosis in the cortex and white matter was evident in all cases, and many corpora amylacea and neurofibrillary tangle—like inclusions were observed in a subset of cases. An ultrastructural investigation revealed dilatation of the postsynaptic dendritic spines and shafts in the cortex and features indicating the occurrence in the white matter of demyelination followed by remyelination. Thus, with regard to the epileptogenic lesions, although dysplastic changes constitute the pathogenetic basis, the overlapping subsequent degenerative processes involving synapses, dendrites, and axons might contribute to the development of epileptogenic processes. Astrocytes might also actively participate in the development of the pathogenesis of epilepsy. ( J Child Neurol 2005;20:341—350).

scholarly journals Periinsular anterior quadrantotomy: technical note

2018 ◽  
Vol 21 (2) ◽  
pp. 124-132 ◽  
Author(s):  
Giulia Cossu ◽  
Sebastien Lebon ◽  
Margitta Seeck ◽  
Etienne Pralong ◽  
Mahmoud Messerer ◽  
...  
Keyword(s):  
Frontal Lobe ◽  
Refractory Epilepsy ◽  
Focal Cortical Dysplasia ◽  
Seizure Outcome ◽  
Illustrative Case ◽  
Neural Pathways ◽  
Functional Neuroanatomy ◽  
The Right ◽  
Epileptogenic Foci

Refractory frontal lobe epilepsy has been traditionally treated through a frontal lobectomy. A disconnective technique may allow similar seizure outcomes while avoiding the complications associated with large brain resections. The aim of this study was to describe a new technique of selective disconnection of the frontal lobe that can be performed in cases of refractory epilepsy due to epileptogenic foci involving 1 frontal lobe (anterior to the motor cortex), with preservation of motor function. In addition to the description of the technique, an illustrative case is also presented.This disconnective procedure is divided into 4 steps: the suprainsular window, the anterior callosotomy, the intrafrontal disconnection, and the frontobasal disconnection. The functional neuroanatomy is analyzed in detail for each step of the surgery. It is important to perform cortical and subcortical electrophysiological mapping to guide this disconnective procedure and identify eloquent cortices and intact neural pathways.The authors describe the case of a 9-year-old boy who presented with refractory epilepsy due to epileptogenic foci localized to the right frontal lobe. MRI confirmed the presence of a focal cortical dysplasia of the right frontal lobe. A periinsular anterior quadrant disconnection (quadrantotomy) was performed. The postoperative period was uneventful, and the patient was in Engel seizure outcome Class I at the 3-year follow-up. A significant cognitive gain was observed during follow-up.Periinsular anterior quadrantotomy may thus represent a safe technique to efficiently treat refractory epilepsy when epileptogenic foci are localized to 1 frontal lobe while preserving residual motor functions.

scholarly journals Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Frontal Lobe Epilepsy: A New Clinico-Pathological Entity

2016 ◽  
Vol 27 (1) ◽  
pp. 26-35 ◽  
Author(s):  
Johannes Schurr ◽  
Roland Coras ◽  
Karl Rössler ◽  
Tom Pieper ◽  
Manfred Kudernatsch ◽  
...  
Keyword(s):  
Frontal Lobe ◽  
Cortical Development ◽  
Frontal Lobe Epilepsy ◽  
Malformation Of Cortical Development

scholarly journals Frontal dermoid cyst coexisting with suprasellar craniopharyngioma: a spectrum of ectodermally derived epithelial-lined cystic lesions?

2016 ◽  
Vol 41 (6) ◽  
pp. E16 ◽  
Author(s):  
Hussam Abou-Al-Shaar ◽  
Muhammad M. Abd-El-Barr ◽  
Hasan A. Zaidi ◽  
Eleanor Russell-Goldman ◽  
Rebecca D. Folkerth ◽  
...  
Keyword(s):  
Frontal Lobe ◽  
Dermoid Cyst ◽  
Histopathological Examination ◽  
Third Ventricle ◽  
Residual Tumor ◽  
Subtotal Resection ◽  
Cystic Lesions ◽  
Frontal Lesion ◽  
The Third ◽  
The Right

There is a wide group of lesions that may exist in the sellar and suprasellar regions. Embryologically, there is varying evidence that many of these entities may in fact represent a continuum of pathology deriving from a common ectodermal origin. The authors report a case of a concomitant suprasellar craniopharyngioma invading the third ventricle with a concurrent frontal lobe cystic dermoid tumor. A 21-year-old man presented to the authors' service with a 3-day history of worsening headache, nausea, vomiting, and blurry vision. Magnetic resonance imaging depicted a right frontal lobe lesion associated with a separate suprasellar cystic lesion invading the third ventricle. The patient underwent a right pterional craniotomy for resection of both lesions. Gross-total resection of the right frontal lesion was achieved, and subtotal resection of the suprasellar lesion was accomplished with some residual tumor adherent to the walls of the third ventricle. Histopathological examination of the resected right frontal lesion documented a diagnosis of dermoid cyst and, for the suprasellar lesion, a diagnosis of adamantinomatous craniopharyngioma. The occurrence of craniopharyngioma with dermoid cyst has not been reported in the literature before. Such an association might indeed suggest the previously reported hypothesis that these lesions represent a spectrum of ectodermally derived epithelial-lined cystic lesions.

scholarly journals Contralateral insular epileptogenic hub causing seizure relapse after opercular focal cortical dysplasia surgery and response to radiofrequency thermocoagulation: illustrative case

2021 ◽  
Vol 2 (5) ◽  
Author(s):  
Pushkaran Jayapaul ◽  
Shameer Aslam ◽  
Bindhu Mangalath Rajamma ◽  
Siby Gopinath ◽  
Ashok Pillai
Keyword(s):  
Focal Cortical Dysplasia ◽  
Positron Emission Tomography Imaging ◽  
Cortical Dysplasia ◽  
Illustrative Case ◽  
Epileptogenic Zone ◽  
Pharmacoresistant Epilepsy ◽  
Novel Technique ◽  
Positron Emission ◽  
Seizure Relapse ◽  
The Right

BACKGROUND The reevaluation and management of seizure relapse following resective surgery in magnetic resonance imaging (MRI)-negative pharmacoresistant epilepsy remains a significant challenge. OBSERVATIONS A 25-year-old right-handed male with medically refractory epilepsy presented with nonlocalizing electroencephalography (EEG) and MRI. Stereo-EEG (SEEG) implantation based on semiology and positron emission tomography imaging revealed a left frontal opercular focus with rapid bilateral insular ictal synchrony. The initial epileptogenic zone was resected and pathologically proven to be type 2A focal cortical dysplasia (FCD). Seizure relapse after 9 months was eventually reinvestigated, and repeat SEEG revealed a secondary epileptogenic focus in the contralateral insula. A novel technique of volumetric stereotactic radiofrequency ablation (vRFA) was utilized for the right insular focus, following which, the patient remains seizure-free for 20 months. He suffered a transient bilateral opercular syndrome following the second intervention that eventually resolved. LESSONS The authors present clinical evidence to suggest epileptogenic nodes distant from the primary focus as a mechanism for seizure relapse following FCD surgery and the importance of bilateral insular SEEG coverage. The authors also describe a novel technique of minimally invasive vRFA that allows ablation of a larger volume of cerebral cortex when compared to conventional bedside SEEG electrode thermocoagulation.

scholarly journals Cognitive functioning after epilepsy surgery in children with mild malformation of cortical development and focal cortical dysplasia

2019 ◽  
Vol 94 ◽  
pp. 209-215 ◽  
Author(s):  
Tim J. Veersema ◽  
Monique M.J. van Schooneveld ◽  
Cyrille H. Ferrier ◽  
Pieter van Eijsden ◽  
Peter H. Gosselaar ◽  
...  
Keyword(s):  
Cognitive Functioning ◽  
Epilepsy Surgery ◽  
Focal Cortical Dysplasia ◽  
Cortical Development ◽  
Cortical Dysplasia ◽  
Malformation Of Cortical Development

Surgery for Nontumoral Insular Epilepsy Explored by Stereoelectroencephalography

Neurosurgery ◽  
2016 ◽  
Vol 79 (4) ◽  
pp. 578-588 ◽  
Author(s):  
Guillaume Gras-Combe ◽  
Lorella Minotti ◽  
Dominique Hoffmann ◽  
Alexandre Krainik ◽  
Philippe Kahane ◽  
...  
Keyword(s):  
Focal Cortical Dysplasia ◽  
Intractable Epilepsy ◽  
Insular Cortex ◽  
Cavernous Angioma ◽  
Cortical Dysplasia ◽  
Electrode Placement ◽  
Seizure Freedom ◽  
Epileptogenic Zone ◽  
Pharmacoresistant Epilepsy ◽  
The Right

Abstract BACKGROUND Hidden by the perisylvian operculi, insular cortex has long been underexplored in the context of epilepsy surgery. Recent studies advocated stereoelectroencephalography (SEEG) as a reliable tool to explore insular cortex and its involvement in intractable epilepsy and suggested that insular seizures could be an underestimated entity. However, the results of insular resection to treat pharmacoresistant epilepsy are rarely reported. OBJECTIVE We report 6 consecutive cases of right insular resection performed based on anatomoelectroclinical correlations provided by SEEG. METHODS Six right-handed patients (3 male, 3 female) with drug-resistant epilepsy underwent comprehensive presurgical evaluation. Based on video electroencephalographic recordings, they all underwent SEEG evaluation with bilateral (n = 4) or unilateral right (n = 2) insular depth electrode placement. All patients had both orthogonal and oblique (1 anterior, 1 posterior) insular electrodes (n = 4-6 electrodes). Preoperative magnetic resonance imaging findings were normal in 4 patients, 1 patient had right insular focal cortical dysplasia, and 1 patient had a right opercular postoperative scar (cavernous angioma). All patients underwent right partial insular corticectomy via the subpial transopercular approach. RESULTS Intracerebral recordings demonstrated an epileptogenic zone confined to the right insula in all patients. After selective insular resection, 5 of 6 patients were seizure free (Engel class I) with a mean follow-up of 36.2 months (range, 18-68 months). Histological findings revealed focal cortical dysplasia in 5 patients and a gliosis scar in 1 patient. All patients had minor transient neurological deficit (eg, facial paresis, dysarthria). CONCLUSION Insular resection based on SEEG findings can be performed safely with a significant chance of seizure freedom.

scholarly journals Diffusion tensor imaging correlates of hippocampal sclerosis and anterior temporal lobe T2 signal changes in pharmacoresistant epilepsy

2014 ◽  
Vol 01 (01) ◽  
pp. 001-007 ◽  
Author(s):  
Kevin Spitler ◽  
Francis Tirol ◽  
Itzhak Fried ◽  
Jerome Engel ◽  
Noriko Salamon
Keyword(s):  
Diffusion Tensor Imaging ◽  
White Matter ◽  
Temporal Lobe ◽  
Diffusion Tensor ◽  
Hippocampal Sclerosis ◽  
Anterior Temporal Lobe ◽  
Pharmacoresistant Epilepsy ◽  
Fiber Tracts ◽  
White Matter Fiber Tracts ◽  
The Right

AbstractBackground and purpose Our goal was to determine fiber tract integrity in hippocampal sclerosis (HS) using diffusion tensor imaging (DTI) and to correlate white matter damage with other pathology in this disease.Methods Twenty-six patients and eight controls were studied with DTI tractography for 8 pairs of white matter fiber tracts and 2 commissural tracts. Fractional anisotropy (FA) of the fiber tracts was compared with controls. The FA of select fiber tracts was also compared with change in T2 signal in the anterior temporal lobe (ATC), and the performance on neuropsychological tests.Results In comparison with controls, subjects with left sided hippocampal sclerosis (L-HS) had 3 ipsilateral fiber tracts with decreased FA. The FA of fiber tracts was similar in right sided HS (R-HS) to controls. The ipsilateral inferior longitudinal fasciculus had a decrease in FA that correlated with the ATC (T2 signal change). The right superior longitudinal fasciculus had a decrease in FA proportional to lower performance on tests of memory and language.Conclusion The subjects with L-HS had more extensive structural abnormalities involving white matter tracts, both ipsilateral and contralateral. In contrast, subjects with R-HS had limited changes in white matter integrity. Pathology of white matter appears to be involved in deficits associated with HS, including ATC and cognitive performance.

Sign in / Sign up

Export Citation Format

Share Document