该研究得到了北京大学第一医院 IRB 的批准，并获得了所有参与者的书面知情同意书。
注：纳入 2017 年 1 月 1 日至 2022 年 3 月 1 日在北京大学第一医院儿科癫痫中心接受额叶断线术的所有顽固性癫痫患儿并进行分析。纳入符合 MOGHE 临床和放射学标准且 MRI 提示致癫痫病灶局限于额叶的患者。MOGHE 的组织病理学诊断标准之前已经描述过6.进行精学、发作期和发作间期脑电图 （EEG） 、磁共振成像 （MRI） 、发作间期 18氟脱氧葡萄糖正电子发射断层扫描 （PET） 和发育评估进行术前评估。7 岁以上的儿童通过 Wechsler Intelligence Scale-IV 进行评估，7 岁以下的儿童通过 Griffith Developmental Assessment Scale 进行评估。通过 ILAE 分类评估癫痫的结局，ILAE 1 的患者被认为无癫痫发作8。符号学分为 3 组 （局灶性、痉挛/全身性癫痫发作和混合型）。发作间期脑电图分为局灶性 （区域放电） 、多灶性 （几个单侧半球区域放电） 和弥漫性 （对侧受累）。发作期脑电图分为局灶性（区域性发作）、弥漫性（单侧半球发作或非定位性脑电图）和局灶性/弥漫性（2 种模式共存）9。根据 Hartlieb 等人 7 对 MOGHE 的分型，8 例患者的 MRI 结果也被分为 1 型（图 1A、B）或 2 型。 图 1 显示了患者 8 额部断开的代表性术中照片和术前和术后图像。
1. 定位
<ol><li>使用标准技术对患者进行全身麻醉。</li>
	<li>根据解剖标志定位外侧裂、冠状缝和中央沟，作为绘制手术切口线的参考。在中线外 1.0 厘米处和冠状缝合线前 2-3 厘米处做一个“7”形的皮肤切口。确保切口的后缘包括中央前回并向下延伸到翼状区域（图 1D）</li>
	<li>将患者仰卧，头部转向对侧。在肩膀下方放置一个肩垫以帮助头部转动。</li>
	<li>通过无菌准备皮肤和覆盖手术区域，在整个手术过程中保持无菌条件。</li>
</ol>2. 开仓
<ol><li>切开头皮和肌肉。小心将肌肉分层切割。</li>
	<li>用手术刀将头皮和骨膜分开。 注意：保留骨膜可减少术后皮下水肿。</li>
	<li>使用直径为 1 cm 的高速钻头钻三个 1 cm 的孔，然后用 1 mm 宽的铣刀铣削骨瓣。 注意：需要充分暴露蝶骨嵴，以促进额基的后续断开。</li>
	<li>使用骨蜡、明胶海绵和双极烧灼术实现止血。</li>
	<li>在骨缘钻 4-5 个 1 毫米的孔并悬吊硬脑膜以避免硬膜外血肿。</li>
	<li>在距离骨缘 0.5 cm 的地方切开硬脑膜。暴露中央前回、额下回的后部和外侧裂的起点。（图 1E）</li>
</ol>3. 额部断开程序
<ol><li>在手术前使用立体定向系统软件进行手术计划。将 3D T1、天赋加权图像和 PET 数据导入软件中，用于配准和 3D 重建10。 注意：中央前沟通过 3D 共配准描绘。未进行中央回的术中标测（图 1C）。</li>
	<li>选择术前影像学检查异常最严重的区域进行组织学检查。</li>
	<li>切除额下回的后部，以完全暴露岛叶的 1 和 2 短回。 注意：在疑似额部 MOGHE 的 5 岁以下儿童中，不考虑保留语音区域，以最大限度地扩大致癫痫区的断开程度。</li>
	<li>断开额叶基部和沿前岛叶到蝶骨嵴的纤维，然后到中线。 注意：大脑前动脉和嗅三角是断开后边界的标志。直回位于嗅神经内侧，额叶基底的断开需要到达中线软脑膜。对侧脑组织必须保存完好。</li>
	<li>沿着中央前沟解剖灰质和白质。中线断开的边界是脑镰，向下到扣带回，相应地断开它。<ol><li>当沿中央前沟进行额内断开时，稍微向前断开沟底部下方的白质，以使锥体束完整。</li>
		<li>另一方面，由于 MOGHE 是灰白质边界模糊的白质病变，因此请进行足够深的切除/断开，以实现最高的无癫痫发作机会。</li>
	</ol></li>
	<li>切开胼胝体，打开同侧脑室，沿脑室顶部至前角完全解剖弓状纤维，然后与额基断开线相遇。 注意：到目前为止，整个前侧脑室是完全开放的。</li>
	<li>将前胼胝体与侧脑室内的前连合断开，其中额基底骨断开的后部达到胼胝体断开的水平。</li>
	<li>暴露大脑前动脉，这可能是胼胝体断开的解剖标志。最后，用抽吸术切除大脑前动脉后面的末端旁回和后回直肌。 注意：额基底骨断开应完全断开，包括末端旁回和后回直肌;否则，癫痫发作的结果将受到很大影响。额叶断开通常伴有根据术前评估切除 1 或/和 2 个岛叶短回，从而有助于更全面地切除致癫痫区。断开连接后的照片和术后 MRI 如图 1 所示。应积极考虑切除 1 和 2 个短岛叶回，尤其是术前 MRI 和 PET 提示异常时。</li>
</ol>4. 关闭
<ol><li>用 4-0 可吸收缝合线缝合硬脑膜，以实现紧密闭合。</li>
	<li>放置硬膜外引流管 2 天。</li>
	<li>使用 3 个可吸收的颅骨锁固定骨骼。</li>
	<li>用 4-0 根皮下缝合线闭合皮下层。</li>
	<li>用吻合器缝合皮肤。</li>
</ol>

MOGHE 和癫痫：额叶断开手术的结果 

<ol>
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Y., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Clinical+characteristics+and+surgical+outcomes+in+children+with+mild+malformation+of+cortical+development+and+oligodendroglial+hyperplasia+in+epilepsy.">Clinical characteristics and surgical outcomes in children with mild malformation of cortical development and oligodendroglial hyperplasia in epilepsy.</a> Epilepsia Open. 8 (3), 898-911 (2023).</li><li>Hartlieb, T., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Age-related+MR+characteristics+in+mild+malformation+of+cortical+development+with+oligodendroglial+hyperplasia+and+epilepsy+(MOGHE).">Age-related MR characteristics in mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE).</a> Epilepsy Behav. 91, 68-74 (2019).</li><li>Wieser, H. G., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Proposal+for+a+new+classification+of+outcome+with+respect+to+epileptic+seizures+following+epilepsy+surgery.">Proposal for a new classification of outcome with respect to epileptic seizures following epilepsy surgery.</a> Epilepsia. 42 (2), 282-286 (2001).</li><li>Wang, Y., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Disconnection+surgery+in+pediatric+epilepsy:+A+single+center's+experience+with+185+cases.">Disconnection surgery in pediatric epilepsy: A single center's experience with 185 cases.</a> Neurosurgery. 93 (6), 1251-1258 (2023).</li><li>Wang, Y., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Seizure+and+cognitive+outcomes+of+posterior+quadrantic+disconnection:+A+series+of+12+pediatric+patients.">Seizure and cognitive outcomes of posterior quadrantic disconnection: A series of 12 pediatric patients.</a> Br J Neurosurg. 34 (6), 677-682 (2020).</li><li>Gaballa, A., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Clinical+characteristics+and+postoperative+seizure+outcome+in+patients+with+mild+malformation+of+cortical+development+and+oligodendroglial+hyperplasia.">Clinical characteristics and postoperative seizure outcome in patients with mild malformation of cortical development and oligodendroglial hyperplasia.</a> Epilepsia. 62 (12), 2920-2931 (2021).</li><li>Barba, C., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Clinical+features,+neuropathology,+and+surgical+outcome+in+patients+with+refractory+epilepsy+and+brain+somatic+variants+in+the+slc35a2+gene.">Clinical features, neuropathology, and surgical outcome in patients with refractory epilepsy and brain somatic variants in the slc35a2 gene.</a> Neurology. 100 (5), e528-e542 (2023).</li><li>Garganis, K., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=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.</a> Epileptic Disord. 25 (3), 343-359 (2023).</li><li>Willard, A., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Seizure+outcome+after+surgery+for+mri-diagnosed+focal+cortical+dysplasia:+A+systematic+review+and+meta-analysis.">Seizure outcome after surgery for mri-diagnosed focal cortical dysplasia: A systematic review and meta-analysis.</a> Neurology. 98 (3), e236-e248 (2022).</li><li>Kalbhenn, T., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Operative+posterior+disconnection+in+epilepsy+surgery:+Experience+with+29+patients.">Operative posterior disconnection in epilepsy surgery: Experience with 29 patients.</a> Epilepsia. 60 (9), 1973-1983 (2019).</li><li>Devlin, A. M., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Clinical+outcomes+of+hemispherectomy+for+epilepsy+in+childhood+and+adolescence.">Clinical outcomes of hemispherectomy for epilepsy in childhood and adolescence.</a> Brain. 126 (Pt 3), 556-566 (2003).</li><li>Marras, C. E., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Hemispherotomy+and+functional+hemispherectomy:+Indications+and+outcome.">Hemispherotomy and functional hemispherectomy: Indications and outcome.</a> Epilepsy Res. 89 (1), 104-112 (2010).</li><li>Castagno, S., et al. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Seizure+outcomes+of+large+volume+temporo-parieto-occipital+and+frontal+surgery+in+children+with+drug-resistant+epilepsy.">Seizure outcomes of large volume temporo-parieto-occipital and frontal surgery in children with drug-resistant epilepsy.</a> Epilepsy Res. 177, 106769 (2021).</li><li>Kamalboor, H., Alhindi, H., Alotaibi, F., Althubaiti, I., Alkhateeb, M. <a target="_blank" href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed&cmd=Search&doptcmdl=Citation&defaultField=Title+Word&term=Frontal+disconnection+surgery+for+drug-resistant+epilepsy:+Outcome+in+a+series+of+16+patients.">Frontal disconnection surgery for drug-resistant epilepsy: Outcome in a series of 16 patients.</a> Epilepsia Open. 5 (3), 475-486 (2020).</li></ol>

作者没有什么可披露的。

MOGHE 是一种主要位于 GM/WM 边界的新白质实体，但没有像 FCD2 那样表现出皮质层杂乱无章。非典型的符号学和广泛的脑电图使得使用传统的解剖电临床评估方法很难确定致癫痫区的位置，这给手术决策带来了困难。以前的研究报告称，MOGHE 患者在广泛切除后取得了良好的结果 6,11，但缺乏详细信息。然而，有文献报道，广泛额叶切除术?...

皮质发育畸形 （MCD） 是幼儿耐药性癫痫和发育迟缓的重要原因。MCD 的类型很多，其中 FCD 是最常见的1 型。根据 2022 年最新更新的 FCD 分类，MOGHE 被描述和分类为以白质为主的病变，相比之下，近皮质局限性 FCD2 主要被描述为增殖性少突胶质细胞增生伴癫痫 （POGHE）3，并于 2017 年首次提出4。MOGHE 的定义是标本 2,4,5 中白质和深皮层中异位神经元的增加超过 2200 个 Olig2 免疫反应细胞/mm2。
临床上，MOGHE 最常见于额叶，癫痫发作痉挛是最常见的初始癫痫发作类型6。发作间期脑电图模式通常是多灶性的或在幼儿中广泛存在6。在年幼的儿童中，MRI 显示灰白质交界处的层状信号增加。在年龄较大的儿童中，观察到皮层下 T2/FLAIR 信号减少和灰白质转变模糊7。在临床实践中，准确描述 MOGHE 非常困难。因此，传统的术前评估通过综合解剖-电-临床评估来确定致癫痫区范围的概念和策略难以实施。在此，分析了额叶中 MOGHE 的儿科队列，以扩展 MOGHE 手术治疗知识。

<table border="1" fo:keep-together.within-page="1" fo:keep-with-next.within-page="always">
	<tbody>
		<tr>
			<td>Absorbable cranial bone lock</td>
			<td>Braun Inc.</td>
			<td>FF016</td>
			<td></td>
		</tr>
		<tr>
			<td>Drainage</td>
			<td>Branden Inc.</td>
			<td>Fr12</td>
			<td></td>
		</tr>
		<tr>
			<td>High-speed drill</td>
			<td>Stryker</td>
			<td>5400-050-000</td>
			<td></td>
		</tr>
		<tr>
			<td>Microscope</td>
			<td>Leica Inc.</td>
			<td>M525F40</td>
			<td></td>
		</tr>
		<tr>
			<td>NIHON KOHDAN EEG system</td>
			<td>NIHON KOHDAN Inc.</td>
			<td>EEG-1200C</td>
			<td>EEG&#160;</td>
		</tr>
		<tr>
			<td>Philips PET-CT system</td>
			<td>Philips Inc.</td>
			<td>Gemini GXL</td>
			<td>PET-CT</td>
		</tr>
		<tr>
			<td>Sinovation Stereotactic system</td>
			<td>Sinovation Inc.</td>
			<td>SR1</td>
			<td>3D construction</td>
		</tr>
	</tbody>
</table>

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

皮质发育畸形是幼儿耐药性癫痫的重要原因。癫痫中少突胶质细胞增生的轻度皮质发育畸形 （MOGHE） 已被添加到最后一个局灶性皮质发育不良 （FCD） 分类中，通常累及额叶。癫痫发作时的符号学以非偏侧性婴儿痉挛症为主;畸形的边界通常难以通过磁共振成像 （MRI） 和正电子发射断层扫描 （PET） 确定，并且脑电图 （EEG） 结果通常很普遍。因此，传统的术前评估通过综合解剖-电-临床方法确定致癫痫区范围的理念和策略难以实施。 
额叶断开术是治疗癫痫的一种有效的手术方法，但相关报道较少。回顾性研究了 8 例经组织病理证实的 MOGHE 患儿。MOGHE 位于所有患者的额叶，并进行额叶断开。在断开连接手术中使用岛周入路，分为几个手术步骤：额下回部分切除术、额基底和额内断开以及前体胼胝切开术。 
1 例患者表现为短期术后言语障碍，而 1 例患者表现出短暂的术后肢体无力。未观察到长期术后并发症。术后 2 年，75% 的患者无癫痫发作，其中一半患者认知能力得到改善。这一发现表明，额叶断开术是治疗 MOGHE 的一种有效且安全的外科手术，而不是额叶的广泛切除。

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.

作者聚焦：研究 MOGHE 患者额叶断开后的临床特征和癫痫结果

额叶断开治疗额叶少突胶质细胞增生症 （MOGHE） 皮质发育轻度畸形

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.

根据纳入标准，共有 8 名符合条件的患者被纳入分析。该小组由 8 名男孩组成。发病年龄为 4-28 个月 （中位 6 个月），手术年龄为 17-135 个月 （中位 38 个月）。癫痫持续时间为 13 至 121 个月 （中位数为 32 个月）。4 例患者的符号为癫痫性痉挛，3 例为局灶性癫痫发作，1 例为混合性癫痫发作。发作间期 EEG 显示 3 例局灶性，3 例患者多灶性，2 例患者弥漫性。发作期脑电图显示 5 例患者为局灶性，...

在这里，我们提出了一个方案来描述 8 例额叶断开后额叶少突胶质细胞增生 （MOGHE） 轻度皮质发育畸形患者的癫痫结果和并发症。该程序的特点是简单、用户友好和术后并发症较少。

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

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

428 次观看.  Peking University First Hospital. 我们研究了 MOGHE 的术后癫痫结局。由于 MOGHE 的临床特点，传统的理念和术前评估确定致癫痫区程度的策略难以实施。我们尝试分析位于额叶的 MOGHE 额叶断开后的癫痫结果和并发症。对于 MOGHE 患者，切除范围应相对较大。MRI 可分为两种类型。1 型皮髓交界处层状 T2 和 FLAIR 信号增加，2 型由于相邻白质信号增加，皮髓分化减少。额叶断开是...