轻度皮质发育畸形伴少突胶质细胞增生及癫痫（MOGHE）—一个新的临床组织病理学实体_Journal of Epilepsy

Authors：

 王薇薇 , 吴逊

北京大学第一医院神经内科（北京 100034）;

Corresponding author：

王薇薇, Email: bxtong37@163.com

Keywords：

癫痫; 磁共振成像; 少突胶质细胞; 异位神经元

DOI：

10.7507/2096-0247.202111008

Video：

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2002年Burger等首先报道癫痫患者的大脑标本中有少突胶质细胞增生。2013年Coras等认为是一个新的临床病理学实体称之增殖性少突胶质细胞伴癫痫（Proliferative oligodendroglial hyperlasia in epilepsy，POGHE）。2017年Schurr等详细研究其病理学后确认为轻度皮质发育畸形伴少突胶质细胞增生及癫痫（Mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy，MOGHE）。迄今国外文献报道92例，国内尚无报道及介绍，本文对92例进行分析并复习文献。均为儿童药物难治性部分发作，发作年龄≤15岁者96.4%，≤10岁者83.1%。临床表现多种多样。电临床多定位于额叶（81.5%），少数在颞顶或颞枕区。磁共振成像异常类似于局灶性皮质发育不良（Focal cortical dysplasia，FCD），尤其是FCDⅡa。均为药物难治性癫痫，并做外科切除性治疗。组织病理学均有不同于FCD的特点，即灰白质交界处有簇状或层状少突胶质细胞增生及异位神经元。但皮质分层无异常。

Citation： 王薇薇, 吴逊. 轻度皮质发育畸形伴少突胶质细胞增生及癫痫（MOGHE）—一个新的临床组织病理学实体. Journal of Epilepsy, 2022, 8(2): 142-145. doi: 10.7507/2096-0247.202111008 Copy

1.	Bonduelle T, Hartlieb T, Baldassari S, et al. Frequent SLC35A2 brain mosacicism in mild malfarmation of cortical derelopment with oligodendroglial hyperplasia in epilepsy(MOGHE). Acta Neuropathologica Communications, 2021, 9(3): 1-13.
2.	Coello VC. Mortia-Sherman M, Yasuda C, et al. Magnetic resonance imaging findings and Clinical characteristics in mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy in a predominantly adult cohort. Epilepsia, 2021, 62(6): 1429-1442.
3.	Burger PC, Scheithauer BW, Vaget FS. Sungical pathology of the nervous system and its covering. 4the, ed. New York, Churchill Livinston, 2002.
4.	Hamilton BE, Neobit GM. MR imaging identification of oligodendroglial hyperplasia. Am J Neuroradial, 2009, 30(7): 1412-1413.
5.	Coras R, Schurr J. Pieper T, et al. Proliferative oligodendroglial hyperplasia in epilepsy (POGHE): A novel diagnostic entity. Epilepsia, 2013, 54(Suppl 3): 318.
6.	Woermann FG, Bien CG. Schulz R. et al. MR features of proliferative oligodendroglial hyperplasia in epilepsy (POGHE)——a novel diagnostic entity. Epilepsia, 2014, 55(suppl 2): 13.
7.	Shurr J, Coras R, Rössler K, et al. Mild malformation of cortical development with oligodendroglial hyperplasia in frontal lobe epilepsy: a new clinico-pathological entity. Brain Pathology, 2017, 27(1): 26-35.
8.	Hartlieb T, Winkler P, Coras R, et al. Age-related MR characteristics in mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE). Epilepsy & Behavion, 2019, 91(1): 68-74.
9.	Garganis K, Kokkinos V, Zountsas B, et al. Temporal lobe “plus”epilepsy associated with oligodendroglial hyperplasia (MOGHE). Acta Neural Scand, 2019, 140(4): 296-300.
10.	Di Giacomo R, Deleo F, Garbell R, et al. Mild malformatiom of cortical development with oligodendroglial hyperplasia(MOGHE): neurophysiological fingerprints of a new pathological entity. Clin Neurophysiol, 2021, l32(1): 154-156.
11.	Lin Y, Xu X, Aung T, et al. Basal temporo-occipital mild malformation of cortical development with oligodendroglial hyperplasia: a multimodal investigation turning non-lesion to lesion epilepsy. Clin Neurophysial, 2020, 131(12): 2826-2828.
12.	Bronen RA, Spencer DD, Fulbright RK. Cerebrospinal fluid cleft with Cortical dimple: MR imaging marker for forcal Cortical dysgenesis. Neuroradiology, 2000, 214(3): 657-663.
13.	邓馨, 王薇薇, 吴逊. 限局性皮质发育障碍分型、诊断和治疗. 癫痫杂志, 2016, 2(3): 234-241.
14.	Blümcke I, Thom M, Aronica E, et al. The Clinicopathologic spectum of forcal cortical dysplasias: a consensus classification proposed by an and hoc Task Force of lLAE Diagnostic Methods Commission. Epilepsia, 2011, 52(1): 158-174.

1. Bonduelle T, Hartlieb T, Baldassari S, et al. Frequent SLC35A2 brain mosacicism in mild malfarmation of cortical derelopment with oligodendroglial hyperplasia in epilepsy(MOGHE). Acta Neuropathologica Communications, 2021, 9(3): 1-13.
2. Coello VC. Mortia-Sherman M, Yasuda C, et al. Magnetic resonance imaging findings and Clinical characteristics in mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy in a predominantly adult cohort. Epilepsia, 2021, 62(6): 1429-1442.
3. Burger PC, Scheithauer BW, Vaget FS. Sungical pathology of the nervous system and its covering. 4the, ed. New York, Churchill Livinston, 2002.
4. Hamilton BE, Neobit GM. MR imaging identification of oligodendroglial hyperplasia. Am J Neuroradial, 2009, 30(7): 1412-1413.
5. Coras R, Schurr J. Pieper T, et al. Proliferative oligodendroglial hyperplasia in epilepsy (POGHE): A novel diagnostic entity. Epilepsia, 2013, 54(Suppl 3): 318.
6. Woermann FG, Bien CG. Schulz R. et al. MR features of proliferative oligodendroglial hyperplasia in epilepsy (POGHE)——a novel diagnostic entity. Epilepsia, 2014, 55(suppl 2): 13.
7. Shurr J, Coras R, Rössler K, et al. Mild malformation of cortical development with oligodendroglial hyperplasia in frontal lobe epilepsy: a new clinico-pathological entity. Brain Pathology, 2017, 27(1): 26-35.
8. Hartlieb T, Winkler P, Coras R, et al. Age-related MR characteristics in mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE). Epilepsy & Behavion, 2019, 91(1): 68-74.
9. Garganis K, Kokkinos V, Zountsas B, et al. Temporal lobe “plus”epilepsy associated with oligodendroglial hyperplasia (MOGHE). Acta Neural Scand, 2019, 140(4): 296-300.
10. Di Giacomo R, Deleo F, Garbell R, et al. Mild malformatiom of cortical development with oligodendroglial hyperplasia(MOGHE): neurophysiological fingerprints of a new pathological entity. Clin Neurophysiol, 2021, l32(1): 154-156.
11. Lin Y, Xu X, Aung T, et al. Basal temporo-occipital mild malformation of cortical development with oligodendroglial hyperplasia: a multimodal investigation turning non-lesion to lesion epilepsy. Clin Neurophysial, 2020, 131(12): 2826-2828.
12. Bronen RA, Spencer DD, Fulbright RK. Cerebrospinal fluid cleft with Cortical dimple: MR imaging marker for forcal Cortical dysgenesis. Neuroradiology, 2000, 214(3): 657-663.
13. 邓馨, 王薇薇, 吴逊. 限局性皮质发育障碍分型、诊断和治疗. 癫痫杂志, 2016, 2(3): 234-241.
14. Blümcke I, Thom M, Aronica E, et al. The Clinicopathologic spectum of forcal cortical dysplasias: a consensus classification proposed by an and hoc Task Force of lLAE Diagnostic Methods Commission. Epilepsia, 2011, 52(1): 158-174.

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