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Lhermitte-Duclos病与小脑神经节细胞瘤——一位视力逐渐丧失患者的偶然发现

Lhermitte-Duclos Disease and Cerebellar Gangliocytoma-An Incidental Finding in a Patient with Gradual Vision Loss.

作者信息

Badakere Akshay, Chaugule Pratik, Rath Soveeta Souravee

机构信息

LV Prasad Eye Institute, KAR Campus, Hyderabad, India.

出版信息

Neuroophthalmology. 2017 Mar 15;41(3):159-160. doi: 10.1080/01658107.2017.1291685. eCollection 2017 Jun.

DOI:10.1080/01658107.2017.1291685
PMID:28512508
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5417168/
Abstract

A 50-year-old male patient presented to the neuro-ophthalmology clinic with chief complaints of gradual decrease in vision in both eyes, more in the left eye, for 6 years. On general examination, the patient had a hemiplegic gait. His presenting acuity was 20/50 in the right eye and 20/320 in the left eye, not improving further. He had dense posterior subcapsular cataracts in both eyes, and fundus examination revealed pale discs. Humphrey visual field tests 30-2 revealed a vertical nasal midline defect in the right eye and grossly depressed fields in the left eye. Keeping in mind the above findings, the authors requested for a magnetic resonance imaging (MRI) of the brain. The brain MRI shows a large infarct in the right parieto-occipital lobe and a small circumscribed lesion in the left cerebellum. The radiologist opined that it could possibly be a gangliocytoma of the cerebellum, and a possible diagnosis of Lhermitte-Duclos syndrome was made.

摘要

一名50岁男性患者因双眼视力逐渐下降(左眼更明显)6年就诊于神经眼科门诊。全身检查时,患者有偏瘫步态。其就诊时右眼视力为20/50,左眼视力为20/320,且无进一步改善。双眼均有致密的后囊下白内障,眼底检查显示视盘苍白。Humphrey视野检查30-2显示右眼有垂直性鼻侧中线缺损,左眼视野严重压低。考虑到上述检查结果,作者要求对脑部进行磁共振成像(MRI)检查。脑部MRI显示右侧顶枕叶有一大面积梗死灶,左侧小脑有一小的局限性病变。放射科医生认为可能是小脑神经节细胞瘤,并做出了可能为Lhermitte-Duclos综合征的诊断。

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本文引用的文献

1
An unusual cause of posterior fossa mass: Lhermitte-Duclos disease.小脑后窝肿块的不常见病因:杜氏神经纤维瘤病。
J Neurol Sci. 2010 Mar 15;290(1-2):138-41. doi: 10.1016/j.jns.2009.12.010. Epub 2010 Jan 8.
2
Lhermitte-Duclos disease and Cowden's syndrome. Report of two cases.Lhermitte-Duclos病与考登综合征。两例报告。
Neurochirurgie. 2006 Nov;52(5):407-14. doi: 10.1016/s0028-3770(06)71239-5.
3
Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm?勒米特-迪克洛病(发育异常性小脑神经节细胞瘤):是一种畸形、错构瘤还是肿瘤?
Acta Neurol Scand. 2002 Mar;105(3):137-45. doi: 10.1034/j.1600-0404.2002.1r127.x.