肥大性下橄榄核变性两例并文献复习  

Hypertrophic olivary degeneration:two cases and literatures review

作　　者：方世元 孔凡一 毛晨晖[1] Fang Shiyuan;Kong Fanyi;Mao Chenhui(Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医院神经科,100730 [2]中国医学科学院北京协和医学院

出　　处：《北京医学》2025年第2期97-101,共5页Beijing Medical Journal

基　　金：北京协和医院中央高水平医院临床科研专项(2022-PUMCH-A-254);中国医学科学院医学与健康科技创新工程(2021-I2M-1-020);科技创新2030-“脑科学与类脑研究”重大项目(2021ZD0201106)。

摘　　要：目的探讨肥大性下橄榄核变性(hypertrophic olivary degeneration,HOD)的病因、临床表现及影像特点。方法回顾性选取2024年1~6月中国医学科学院北京协和医院神经科HOD患者,分析其临床及影像资料,并复习相关文献。结果共纳入2例患者,均为中年男性,在脑桥卒中的7~8个月后新发复视、构音不清、走路不稳,查体可见复杂眼震、腭肌阵挛、肢体不自主震颤。影像学可见下橄榄核体积增大伴T2高信号。2例患者经托吡酯和(或)苯海索治疗数周至数月,症状无改善。结论HOD是继发于格莫三角(guillain-mollaret triangle,GMT)损害的迟发性病理改变。对于GMT结构存在梗死、出血、手术等病史的患者,应警惕HOD。常用药物包括托吡酯、卡马西平、左乙拉西坦、氯硝西泮、苯海索等,但总体疗效尚不确切。Objective To explore the etiology,clinical manifestations and imaging characteristics of hypertrophic olivary degeneration(HOD).Methods The HOD patients in Peking Union Medical College Hospital,Chinese Academy of Medical Sciences from January to June,2024 were selected,and the clinical and radiological data were analyzed retrospectively.Results A total of two cases were included,who were middle-aged men,and developed diplopia,dysarthria,and ataxia seven to eight months after pontine stroke.Physical examination revealed complex nystagmus,palatal myoclonus,and involuntary limb tremors.Imaging showed that enlargement of the inferior olive nuclei with T2 signal hyperintensity.Both patients were treated with topiramate and/or benztropine for several weeks to months,with no significant improvement.Conclusions HOD is a delayed pathological change secondary to guillain-mollaret triangle(GMT).Clinicians should be cautious of this condition in patients with a history of infarction,hemorrhage,or surgery affecting the structures of the GMT.Common treatments include topiramate,carbamazepine,levetiracetam,clonazepam,and benztropine.However,overall efficacy remains uncertain.

关 键 词：肥大性下橄榄核变性 跨突触变性 格莫三角 

分 类 号：R741[医药卫生—神经病学与精神病学]