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小脑后窝肿块的不常见病因:杜氏神经纤维瘤病。

An unusual cause of posterior fossa mass: Lhermitte-Duclos disease.

机构信息

Department of Pediatric Oncology, Hacettepe University Institute of Oncology, 06100 Ankara, Turkey.

出版信息

J Neurol Sci. 2010 Mar 15;290(1-2):138-41. doi: 10.1016/j.jns.2009.12.010. Epub 2010 Jan 8.

Abstract

Lhermitte-Duclos disease (LDD) (dysplastic cerebellar gangliocytoma) is a rare disorder of unknown pathogenesis, presenting with signs and symptoms resulting from obstruction of cerebrospinal fluid flow and mass effect in the posterior fossa. Magnetic resonance imaging is the diagnostic modality of choice allowing preoperative diagnosis with characteristic findings. Surgery is the choice of treatment. The typical histopathological findings of LDD are characterized by widening of the molecular layer, absence of the Purkinje cell layer and hypertrophy in the granule cell layer. Herein we report an adolescent girl with LDD diagnosed preoperatively by the conventional and advanced MR techniques.

摘要

Lhermitte-Duclos 病(LDD)(小脑神经节细胞瘤发育不良)是一种病因不明的罕见疾病,表现为脑脊液流动受阻和后颅窝占位效应引起的体征和症状。磁共振成像(MRI)是首选的诊断方法,具有特征性表现可做出术前诊断。手术是治疗的选择。LDD 的典型组织病理学表现为分子层增宽、浦肯野细胞层缺失和颗粒细胞层肥大。本文报告了一例经常规和高级 MRI 技术术前诊断为 LDD 的青少年女性病例。

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