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小脑发育异常性神经节细胞瘤(勒米特-迪克洛病)。

Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease).

作者信息

Prestor Borut

机构信息

Department of Neurosurgery, University Hospital Center, Zaloska 7, Ljubljana, Slovenia.

出版信息

J Clin Neurosci. 2006 Oct;13(8):877-81. doi: 10.1016/j.jocn.2005.07.018. Epub 2006 Aug 24.

Abstract

Dysplastic gangliocytoma of the cerebellum is a rare disorder requiring surgical resection. During surgical exploration, the margin with normal cerebellar tissue is often not distinct. We present here a case in which we surgically treated a patient with Lhermitte-Duclos disease, and discuss radical resection of the tumour. The patient was first admitted at the age of 34 years. A MRI scan revealed a large left cerebellar mass typical of dysplastic gangliocytoma. Postoperatively the patient developed severe cerebellar syndrome with only partial recovery after 4 years. MRI revealed no impairment of cerebrospinal fluid circulation; the mass in the left cerebellum had been removed, and no recurrent disease was visible. Clinical problems after gross total or complete removal of Lhermitte-Duclos lesions have not been reported in the literature, but herein we stress the importance of extreme caution in removing these lesions in cerebellar areas that have no distinct border between the tumour and normal tissue.

摘要

小脑发育异常性神经节细胞瘤是一种需要手术切除的罕见疾病。在手术探查过程中,与正常小脑组织的边界通常不清晰。我们在此介绍一例手术治疗Lhermitte-Duclos病患者的病例,并讨论肿瘤的根治性切除。该患者首次入院时34岁。磁共振成像(MRI)扫描显示左侧小脑有一个典型的发育异常性神经节细胞瘤大肿块。术后患者出现严重的小脑综合征,4年后仅部分恢复。MRI显示脑脊液循环无损害;左侧小脑的肿块已被切除,未见复发疾病。文献中尚未报道完全或彻底切除Lhermitte-Duclos病变后的临床问题,但在此我们强调,在肿瘤与正常组织无明显边界的小脑区域切除这些病变时要格外谨慎。

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