The study was approved by the IRB of Peking University First Hospital, and written informed consent was obtained from all participants.
NOTE: All children with intractable epilepsy who underwent frontal disconnection at the Pediatric Epilepsy Center of Peking University First Hospital from January 1, 2017, to March 1, 2022, were included and analyzed. Those who met the clinical and radiological criteria of MOGHE and whose MRI suggested that the epileptogenic lesion was confined to the frontal ...

MOGHE and Epilepsy: Outcomes of Frontal Disconnection Surgery 

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MOGHE is a new white matter entity predominantly at the GM/WM boundaries but shows no cortical layer disorganization as is typical for FCDs2. The untypical semiology and extensive EEG make it very difficult to determine the location of the epileptogenic zone using traditional methods of anatomo-electro-clinical evaluation, which creates difficulties in surgical decision-making. Previous studies reported that patients with MOGHE achieved good outcomes after wide resection6<s...

Malformation of cortical development (MCD) is an important cause of drug-resistant epilepsy and developmental delay in young children. There are many types of MCD, among which FCD is the most common type1. According to the latest updated FCD classification in 2022, MOGHE is described and classified as a predominantly white matter lesion in contrast to juxta-cortical localized FCD2 that was primarily described as proliferative&#8195;oligodendroglial&#8195;hyperplasia&#8195;with&#8195;epilepsy&#8195;(POGHE) in 20133 and first proposed in 20174. MOGHE is defined by an increase in heterotopic neurons in the white matter and deep cortical layers above 2200 Olig2-immunoreactive cells/mm2 in specimen2,4,5.
Clinically, MOGHE is most commonly observed in the frontal lobe, and epileptic spasms are the most common initial seizure type6. The interictal EEG pattern was usually multifocal or widespread in young children6. In younger children, MRI showed an increased laminar signal at the gray-white matter junction. In older children, reduced subcortical T2/FLAIR signals and blurring at the gray-white matter transition were observed7. An accurate delineation of MOGHE is very difficult in clinical practice. Therefore, the traditional concept and strategy of preoperative evaluation to determine the extent of the epileptogenic zone by comprehensive anatomo-electro-clinical evaluation is difficult to implement. Herein, a pediatric cohort with MOGHE in the frontal lobe was analyzed to extend the knowledge of surgical treatment for MOGHE.

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			<td>NIHON KOHDAN EEG system</td>
			<td>NIHON KOHDAN Inc.</td>
			<td>EEG-1200C</td>
			<td>EEG&#160;</td>
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			<td>Philips PET-CT system</td>
			<td>Philips Inc.</td>
			<td>Gemini GXL</td>
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</table>

frontal disconnection for treating mild malformation of cortical development with oligodendroglial hyperplasia in epilepsy (moghe) in the frontal lobe

Malformation of cortical development is an important cause of drug-resistant epilepsy in young children. Mild malformation of cortical development with oligodendroglial hyperplasia in epilepsy (MOGHE) has been added to the last focal cortical dysplasia (FCD) classification and commonly involves the frontal lobe. The semiology at the onset of epilepsy is dominated by non-lateralizing infantile spasm; the boundaries of the malformation are usually difficult to determine by magnetic resonance imaging (MRI) and positron emission tomography (PET), and electroencephalography (EEG) findings are often widespread. Therefore, the traditional concept and strategy of preoperative evaluation to determine the extent of the epileptogenic zone by comprehensive anatomo-electro-clinical methods are difficult to implement. 
Frontal disconnection is an effective surgical method for the treatment of epilepsy, but there are few related reports. A total of 8 children with histo-pathologically confirmed MOGHE were retrospectively studied. MOGHE was located in the frontal lobe in all patients, and frontal disconnection was performed. The periinsular approach was used in the disconnective procedures, divided into several surgical steps: the partial inferior frontal gyrus resection, the frontobasal and intrafrontal disconnection, and the anterior corpus callosotomy. 
One patient presented with a short-term postoperative speech disorder, while another patient exhibited transient postoperative limb weakness. No long-term postoperative complications were observed. At 2 years after surgery, 75% of patients were seizure-free, with cognitive improvement in half of them. This finding suggested that frontal disconnection is an effective and safe surgical procedure for the treatment of MOGHE instead of extensive resection in the frontal lobe.

Author Spotlight: Studying Clinical Characters and Epilepsy Outcomes After Frontal Disconnection in Patients with MOGHE

Frontal Disconnection for Treating Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Epilepsy (MOGHE) in the Frontal Lobe

We studied the postoperative epilepsy outcome for MOGHE. Due to MOGHE's clinical characteristics, the traditional concept and the strategy of preoperative evaluation to determine the extent of epileptogenic zone are difficult to implement. We try to analyze the epilepsy outcome and the complications after frontal lobe disconnection of MOGHE located in the frontal lobe.For patients with MOGHE, the resection extent should be relatively large. MRI can be divided into two types. Type 1 with an increased laminar T2 and FLAIR signal at the corticomedullary junction, and Type 2 with reduced corticomedullary differentiation because of the increased signal of the adjacent white matter.Frontal disconnection is an effective way to treat epilepsy with MOGHE in the frontal lobe. The procedure is categorized by its simplicity, user-friendliness, and fewer postoperative complications.

According to the inclusion criteria, a total of 8 eligible patients were included in the analysis. The group consisted of 8 boys. The age of onset was 4-28 months (median 6 months), and the age of surgery ranged from 17-135 months (median 38 months). The duration of epilepsy ranged from 13 to 121 months (median 32 months). The semiology of 4 patients was epileptic spasms, 3 had focal seizures, and 1 had mixed seizures. Interictal EEG indicated focal in 3 patients, multifocal in 3 patients, and diffuse in 2 patients. Icta...

Here, we present a protocol to describe the epilepsy outcome and complications of 8 patients with mild malformation of cortical development with oligodendroglial hyperplasia in epilepsy (MOGHE) in the frontal lobe after frontal disconnection. The procedure is characterized by its simplicity, user-friendliness, and fewer postoperative complications.

Malformation of cortical development is an important cause of drug-resistant epilepsy in young children. Mild malformation of cortical development with ...

frontal-disconnection-for-treating-mild-malformation-cortical

Research

JoVE Journal

Medicine

Efficacy of Frontal Disconnection in Treating Epilepsy Associated with Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Epilepsy (MOGHE)

To begin, administer general anesthesia to the patient before the procedure. Locate the Sylvian fissure, coronal suture, and central sulcus according to anatomical landmarks. Draw a seven-shaped skin incision one centimeter off the midline and two to three centimeters anterior to the coronal suture.Ensure the posterior margin of the incision includes the precentral gyrus and extends down to the pterygoid region. Position the patient supine with the head turned to the opposite side and place a shoulder pad under the shoulder to help the head turn. After draping the surgical area, incise the scalp and muscles taking care to cut the muscles in layers and separate the scalp and periosteum with a scalpel.Using a high-speed drill of one-centimeter diameter, drill three one-centimeter holes and mill down the bone flap with a one-millimeter-wide milling cutter. Use bone wax, gelatin sponge, and bipolar cautery to achieve hemostasis. Next, drill four to five holes of one millimeter in the bone margin and suspend the dura to avoid epidural hematoma.Cut open the dura 0.5 centimeters away from the bone margin. Expose the precentral gyrus, the posterior part of the inferior frontal gyrus, and the beginning of the lateral fissure. Use the stereotactic system software for surgical planning before surgery.Import the 3D T1 flare-weighted images and PET data into the software for registration and 3D reconstruction. Select the area with the most severe abnormality on preoperative imaging for histological examination. Next, resect the posterior part of the inferior frontal gyrus to fully expose the one and two short gyri of the insula.Disconnect the base of the frontal lobe and the fibers along the anterior insula to the sphenoid ridge and then to the midline. Then, perform interfrontal disconnection along the precentral sulcus followed by the white matter under the bottom of the sulcus slightly forward to keep the pyramidal tract intact. Conduct a deep resection to ensure the highest chance of seizure freedom due to MOGHE being a white-matter lesion with blurred gray-white matter boundaries, afterward disconnecting at the midline boundary, which is the cerebral falx, and continue down to the cingulate gyrus.Next, incise the corpus callosum to open the ipsilateral ventricles. Dissect the arcuate fibers completely along the top of the ventricles to the anterior horn meeting the frontal base disconnection line. Afterward, disconnect the anterior corpus callosum to the anterior commissure within the lateral ventricles.Expose the anterior cerebral artery using it as an anatomical landmark for corpus callosum disconnection. Ensure the posterior portion of the frontobasal disconnection reaches the level of the callosal disconnection. Resect the paraterminal gyrus and posterior gyrus rectus behind the anterior cerebral artery with suction.Expose the anterior cerebral artery. Ensure the frontobasal disconnection is complete, including the paraterminal gyrus and posterior gyrus rectus. Consider resecting the one or two short insular gyri based on preoperative evaluation.Suture the dura with 4-0 absorbable sutures to achieve a tight closure and place an epidural drain for two days. Use three absorbable cranial bone locks to fix the bone. Close the subcutaneous layer with 4-0 subcuticular sutures, and suture the skin with a stapler.After an average of two years of follow-up post surgery, six patients were seizure-free with a seizure-free rate of 75%Developmental assessment performed three months to one year after surgery revealed that four patients experienced cognitive improvement.

289 Views.  Peking University First Hospital.  Here, we present a protocol to describe the epilepsy outcome and complications of 8 patients with mild malformation of cortical development with oligodendroglial hyperplasia in epilepsy (MOGHE) in the frontal lobe after frontal disconnection. The procedure is characterized by its simplicity, user-friendliness, and fewer postoperative complications.

MOGHE and Epilepsy: Outcomes of Frontal Disconnection Surgery

概要