肥大性下橄榄核变性的临床特点:六例病例并文献复习  

Hypertrophic olivary degeneration:retrospective study on six cases and literature review

作　　者：陈玲 龚光明[1] 吴军 李珠 秦洁[1] Chen Ling;Gong Guangming;Wu Jun;Li Zhu;Qin Jie(Department of Neurology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China;Department of Immunology,College of Basic Medical Sciences,Zhengzhou University,Zhengzhou 450001,China)

机构地区：[1]郑州大学第一附属医院神经内科,450052 [2]郑州大学基础医学院免疫学系,450001

出　　处：《中国实用医刊》2020年第3期1-5,共5页Chinese Journal of Practical Medicine

基　　金：国家自然科学基金(81870919);河南省自然科学基金(182300410352)。

摘　　要：目的研究肥大性下橄榄核变性(HOD)的临床特点。方法回顾性分析郑州大学第一附属医院神经内三科2013年1月至2018年12月诊治的6例HOD患者的临床资料并进行1次电话随访。结果6例HOD患者中,4例为单侧HOD,2例为双侧HOD。表现为共济失调5例,肢体震颤3例,眼球震颤2例,腭肌震颤1例,肢体疼痛1例,全身肌阵挛1例,复视1例。磁共振成像(MRI)均表现为T2WI高信号、T1WI稍低信号或等信号,3例磁敏感加权成像(SWI)红核均未见明显的信号降低,1例液体衰减反转恢复(FLAIR)上高信号,1例磁共振增强扫描未发现下橄榄核强化。结论HOD表现除典型症状外,还有不典型症状;MRI能清晰显示下橄榄核的继发性变性,SWI未显示出红核变性;药物治疗肢体震颤效果不佳;总体预后不佳,部分患者肢体震颤症状可随时间有所改善。Objective To study the clinical characteristics of hypertrophic olivary degeneration (HOD).Methods Clinical data from 6 HOD patients, diagnosed and treated in Department of Neurology of the First Affiliated Hospital of Zhengzhou University from January 2013 to December 2018, were retrospectively analyzed, and patients were followed up once by telephone.Results There were 4 cases of unilateral HOD and 2 cases of bilateral HOD. Clinical manifestations were ataxia in 5 patients, limb tremor in 3 patients, bilateral eyeball tremor in 2 patients, bilateral palatal muscle tremor in 1 patient, limb pain in 1 patient, systemic myoclonus in 1 patient, diplopia in 1 patient. Magnetic resonance imaging (MRI) showed high signal on T2 weighted imaging, slightly low signal or isointensity signal on T1 weighted imaging. Susceptibility weighted imaging (SWI) were performed on 3 patients, and no significant increase in the density of the red nucleus was found. One patient presented hyperintensity on fluid-attenuated inversion recovery (FLAIR). One patient underwent enhanced MRI, but no enhancement in the inferior olivary nucleus was found.Conclusions In addition to typical symptoms, HOD also presents atypical symptoms. MRI can clearly show the secondary degeneration of olive nucleus, and SWI can not show the red degeneration. The effect of medicine on limb tremor is not good. The overall prognosis is poor, but limbs tremor symptoms of some patients can be improved with time.

关 键 词：肥大性下橄榄核变性 磁共振成像 磁敏感加权成像 

分 类 号：R742[医药卫生—神经病学与精神病学]