第347课（中枢神经）病例探析（093）—小脑发育不良性神经节细胞瘤每次大家在阅读文章前，请花两分钟看看视频吧一份公益，一份感动女性，29岁，体检意外发现平扫轴位T1WI示右侧小脑半球病灶呈条纹状相间的等、低信号或层状排列结构；轴位T2WI病灶呈条纹状相间的等、高信号；T1增强扫描，肿瘤无明显强化，肿块占位效应较轻，周围无水肿。
最后诊断：小脑发育不良性神经节细胞瘤小脑发育不良性神经节细胞瘤最早是由Lhermitte 和Duclos 在1920年报告，故又称为Lhermitte-Duclosdisease （LDD），病程进展较慢，早期多无症状，首发症状以颅内压增高为主，其次为后组颅神经麻痹，小脑症状一般相对出现较晚。
Cerebellar dysplasia ganglioma was first reported by Lhermitte and Duclos in 1920, so it is also known as lhermitte-duclosdisease (LDD). The progression of the disease is slow, and most of the early symptoms are asymptomatic. The firstsymptom is mainly intracranial pressure increase, followed by cranial nerve palsy in the posterior group.LDD典型的病理特征为大量的发育不良的皮质神经元（神经节细胞）替代了小脑的内颗粒层细胞，浦肯野细胞减少或消失，肿瘤细胞亦可侵入分子层。
分子层增厚、过度髓鞘化，肿瘤与正常组织呈分层状结构。
增生的节细胞可能起源于迁移和成熟障碍。
The typical pathological feature of LDD is that a large number of dysplastic cortical neurons (ganglion cells) replace the inner granulosa cells in the cerebellum, purkinwild cells decrease or disappear, and tumor cells may invade the molecular layer. Molecular layer thickening, excessive myelin sheath, tumor and normal tissue presented a layered structure. Proliferative ganglion cells may arise from migration and maturation disorders.发育不良性小脑神经节细胞瘤( dysplastic cerebellar gangliocytoma )是一种罕见、由发育不良的神经节细胞构成、进展缓慢的小脑肿瘤,WHOI级,属于神经元和混合性神经元神经胶质肿瘤。
中国医学影像学杂志 2019年 第27卷 第9期 中枢神经影像学•论著646小脑发育不良性神经节细胞瘤的临床特征与MRI 表现李贝贝，任翠萍*，程敬亮，李莹，肖翠萍，林晨郑州大学第一附属医院磁共振科，河南郑州 450052； *通讯作者 任翠萍 rcp810@【摘要】目的 探讨小脑发育不良性神经节细胞瘤（LDD ）的MRI 特征性表现及临床特点。
资料与方法 回顾性分析5例经手术病理证实为LDD 患者的临床资料及MRI 表现，包括肿瘤发病部位、大小、形态及信号强度，同时记录是否伴有瘤周水肿、瘤体是否强化及强化方式等。
结果 5例LDD 患者中，2例位于右侧小脑半球，1例累及右侧小脑半球及右侧桥小脑结合臂，1例位于左侧小脑半球，1例位于小脑上蚓部。
瘤体内存在呈斜形或纵行排列的条片状结构，呈“虎斑征”；与正常脑组织对比呈层状等/长T1、等/长T2信号；5例均无明显瘤周水肿；3例增强扫描病灶未见明显强化，2例呈点条状轻度强化。
结论 LDD 的MRI 表现具有典型“虎斑征”，并有明显的占位效应。
增强扫描病变不强化或轻微强化。
【关键词】神经节瘤；小脑肿瘤；磁共振成像；疾病特征；病理学，外科 【中图分类号】R739.4；R445.2 【DOI 】10.3969/j.issn.1005-5185.2019.09.002Clinical Features and MRI Manifestations of Lhermitte-Duclos DiseaseLI Beibei, REN Cuiping *, CHENG Jingliang, LI Ying, XIAO Cuiping, LIN ChenDepartment of MRI, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China; *Address Correspondence to: REN Cuiping; E-mail: rcp810@【Abstract 】Purpose To investigate the MRI manifestations and clinical features of Lhermitte-Duclos disease (LDD). Materials and Methods Clinical data and MRI manifestations of 5 patients with LDD confirmed by pathology were retrospectively analyzed, including the location, size, shape and signal intensity of the tumor, and recording whether the peri-tumoral edema, whether the tumor was strengthened and its strengthening method. Results For 5 cases with LDDs, the tumor was located in the right cerebellar hemisphere in 2 cases, in the right cerebellar hemisphere and right olivopontocerebellar brachium conjunctivum in 1 case, in the left cerebellar hemisphere in 1 case and in the superior vermis in 1 case. There were striped structures arranged in oblique line or longitudinal line in the tumor, showing “tiger spot signs”, which exhibited layered isometric T1 and isometric T2 signals compared with normal brain tissue. There was no obvious peri-tumoral edema in all 5 cases; no significant enhancement was found through enhancement scan in 3 cases, and dot-bar light enhancement was observed in 2 cases. Conclusion MRI manifestations of LDDs show typical “tiger spot signs” and a mass effect. Enhancement scan demonstrates that the lesion is not enhanced or slightly enhanced.【Key words 】Ganglioneuroma; Cerebellar neoplasms; Magnetic resonance imaging; Disease attributes; Pathology, surgical Chinese Journal of Medical Imaging, 2019, 27 (9): 646-648小脑发育不良性神经节细胞瘤（Lhermitte-Duclos disease ，LDD ），由Lhermitte 等[1]于1920年首次报道并命名。
