【摘要】 目的肥大性下橄榄核变性(HOD)是由原发于齿状核-红核-下橄榄核环路(DROP)区病变所继发的一种特殊的跨突触变性,具有较为独特的临床和影像学表现。本文拟对HOD的临床特点进行探讨,以提高对其的认识。方法回顾性分析11例HOD患者的临床资料。结果男性10例,女性1例,平均年龄53.5岁。原发病包括中脑出血1例,脑桥出血8例,脑桥梗塞1例,脑桥胶质瘤1例。HOD临床表现为头晕、视物不清、眼震、言语含混、软腭阵挛、肌张力增高、不自主运动、姿势异常、共济失调等症候组合,平均晚于原发病3.8个月出现。MRI上表现为下橄榄核体积增大和T2WI高信号。当原发病变累及单侧脑桥被盖或红核时,HOD发生在同侧,临床症状出现在对侧;当原发病变累及双侧红核或脑桥被盖时,HOD和临床症状出现在双侧。巴氯芬、氯硝安定和卡马西平可以减轻临床症状。结论 DROP区病变后要警惕HOD的发生,延迟损害是诊断的重要线索,共济失调和不自主运动是最常见的临床表现,MRI检查可以帮助确诊。更多还原

【Abstract】 Objective Hypertrophic olivary degeneration(HOD) is a unique transsynaptic degeneration with special clinical manifestations,occurringdelayed damage after the primary damage to the dentato-rubro-olivary pathway(DROP).This paper aims to investigate the clinical and neuroimaging characteristics of HOD.Methods We retrospectively analyzed the clinical data of 11 patients with HOD.Results Of the 11 patients,10 were male and 1 female,with an average age of 53.5 years old.Primary diseases included midbrain hemorrhage(n=1),pontine hemorrhage(n=8),pontine infarction(n=1) and pontine gliomas(n=1).The onset of HOD symptoms,which included dizziness,nystagmus,palatal myoclonus,hypermyotonia,involuntary movement,abnormal posture,ataxia,was meanly 3.8 months later than that of the primary diseases.Enlargement and increased T2 signal intensity of the inferior olive distant from the primary damage were MRI features of HOD.When the primary lesion only affected the unilateral central tegmental tract or red nucleus,HOD was ipsilateral and symptoms occurred in the contralateral side.If the lesion involved both the central tegmental tracts or superior cerebellar pedunculus or red nuclei,HOD and symptoms were bilateral.Baclofen,clonazepam and carbamazepine could be used to alleviatethe clinical symptoms.Conclusion We should be vigilant about the occurrence of HOD secondary to lesions involved the DROP,in which delayed symptoms could be vital clues.ataxia and involuntary movement are the most common clinical manifestation,and brain MRI is of great help for further diagnosis.更多还原