Lee J D, Ho Y S, Chen S T, Lin T K, Ro L S, Lee T H
Department of Neurology, Chang Gung Memorial Hospital, Taipei, Taiwan.
J Formos Med Assoc. 1998 Sep;97(9):649-52.
We report a case of Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum), an uncommon disorder of uncertain pathogenesis characterized by disarrangement of the normal cerebellar laminar cytoarchitecture. A 40-year-old man was admitted because of vomiting and syncope of a few days' duration, and a 2-month history of intermittent headaches and unsteady gait. A computed tomographic scan of the patient's head showed obstructive hydrocephalus due to displacement of the fourth ventricle by a large, nonenhancing cerebellar mass. The magnetic resonance images of the brain also revealed a space-occupying lesion within the right cerebellum with unusual septation. After surgery, the histologic examination confirmed the diagnosis of Lhermitte-Duclos disease. This is the first report of Lhermitte-Duclos disease in Taiwan.
我们报告一例Lhermitte-Duclos病（小脑发育异常性神经节细胞瘤），这是一种发病机制不明的罕见疾病，其特征是正常小脑层状细胞结构紊乱。一名40岁男性因持续数天的呕吐和晕厥入院，并有2个月间歇性头痛和步态不稳的病史。患者头部计算机断层扫描显示，一个巨大的、无强化的小脑肿块使第四脑室移位，导致梗阻性脑积水。脑部磁共振成像还显示右小脑内有一个占位性病变，伴有异常分隔。手术后，组织学检查确诊为Lhermitte-Duclos病。这是台湾首例Lhermitte-Duclos病报告。
