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伴有半侧巨脑症的表皮痣综合征:神经病理学研究

Epidermal nevus syndrome with hemimegalencephaly: neuropathological study.

作者信息

Sakuta R, Aikawa H, Takashima S, Ryo S

机构信息

Department of Pediatrics, Nihon University School of Medicine, Tokyo, Japan.

出版信息

Brain Dev. 1991 Jul;13(4):260-5. doi: 10.1016/s0387-7604(12)80060-x.

DOI:10.1016/s0387-7604(12)80060-x
PMID:1957975
Abstract

A neuropathologic examination of a 5-year-boy with epidermal nevus syndrome and hemimegalencephaly revealed 1) left hemimegalencephaly with an increased volume of the white matter, and 2) cerebral polymicrogyria with pachygyria, heterotopic neurons and prominent astrogliosis. Golgi staining revealed hypertrophic neurons with increased dendrites and spines. The hemihypertrophy of the cerebral hemispheres seems to have contributed to an increased volume of the white matter with migration disorder, and may have been due to a disorder in the germinal cell proliferation stage.

摘要

对一名患有表皮痣综合征和半侧巨脑症的5岁男孩进行的神经病理学检查显示:1)左侧半侧巨脑症,白质体积增加;2)脑回肥厚伴多小脑回、神经元异位和明显的星形胶质细胞增生。高尔基染色显示神经元肥大,树突和棘突增加。大脑半球的半侧肥大似乎导致了白质体积增加并伴有迁移障碍,这可能是由于生发细胞增殖阶段的紊乱所致。

相似文献

1
Epidermal nevus syndrome with hemimegalencephaly: neuropathological study.伴有半侧巨脑症的表皮痣综合征:神经病理学研究
Brain Dev. 1991 Jul;13(4):260-5. doi: 10.1016/s0387-7604(12)80060-x.
2
Epidermal nevus syndrome with azygos anterior cerebral artery.
Pediatr Neurol. 2008 Oct;39(4):283-5. doi: 10.1016/j.pediatrneurol.2008.06.016.
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Epidermal nevus syndrome with hemimegalencephaly: a clinical report of a case with acanthosis nigricans-like nevi on the face and neck, hemimegalencephaly, and hemihypertrophy of the body.伴有半侧巨脑症的表皮痣综合征:1例面部和颈部出现黑棘皮病样痣、半侧巨脑症及身体半侧肥大的临床报告
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What syndrome is this? Epidermal nevus syndrome: a neurologic variant with hemimegalencephaly, facial hemihypertrophy and gyral malformation.这是什么综合征?表皮痣综合征:一种伴有半侧巨脑畸形、面部半侧肥大和脑回畸形的神经学变异型。
Pediatr Dermatol. 1998 Jan-Feb;15(1):59-61. doi: 10.1046/j.1525-1470.1998.1998015059.x.
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Developmental neural abnormalities and seizures in epidermal nevus syndrome.表皮痣综合征中的发育性神经异常与癫痫发作
Dev Med Child Neurol. 1996 Aug;38(8):716-23. doi: 10.1111/j.1469-8749.1996.tb12141.x.
6
[Transepidermal elimination of nevus cell nests (author's transl)].痣细胞巢的经表皮消除(作者译)
Dermatologica. 1974;148(1):61-4.
7
Is apoptosis involved in the development of balloon cell nevus? Suggestion from a case report.细胞凋亡是否参与气球状细胞痣的发生发展?一项病例报告提示。
J Am Acad Dermatol. 2007 Jun;56(6):1069-70. doi: 10.1016/j.jaad.2005.07.064.
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[Giant combined pigmented dysplastic nevus].
Arkh Patol. 1987;49(11):71-4.
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Epidermal nevus syndrome: a neurologic variant with hemimegalencephaly, gyral malformation, mental retardation, seizures, and facial hemihypertrophy.表皮痣综合征:一种伴有半侧巨脑症、脑回畸形、智力发育迟缓、癫痫发作和面部半侧肥大的神经学变异型。
Neurology. 1991 Feb;41(2 ( Pt 1)):266-71. doi: 10.1212/wnl.41.2_part_1.266.
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[Woolly hair nevus associated with an ipsilateral linear epidermal nevus].[羊毛状毛发痣伴同侧线状表皮痣]
Actas Dermosifiliogr. 2007 Apr;98(3):198-201.

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A profound case of linear epidermal nevus in a patient with epidermal nevus syndrome.一名患有表皮痣综合征患者的严重线状表皮痣病例。
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Cerebral manifestations, hemihypertrophy and lymphoedema of one leg in a child with epidermal nevus syndrome (Schimmelpenning-Feuerstein-Mims).患有表皮痣综合征(施密尔彭宁-费尔斯坦-米姆斯综合征)儿童的脑部表现、半身肥大及单侧腿部淋巴水肿
Pediatr Radiol. 2003 Sep;33(9):637-40. doi: 10.1007/s00247-003-0944-3. Epub 2003 Jun 12.
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Hemimegalencephaly, hemihypertrophy and vascular lesions.半侧巨脑症、半侧肥大及血管病变。
Eur J Pediatr. 1995 Feb;154(2):134-7. doi: 10.1007/BF01991917.