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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。
Purpose Hypertrophic lower olive nucleus degeneration (HOD) is a special trans-synaptic degeneration secondary to lesions in the dentate nucleus - red nucleus - lower olivary nucleus loop (DROP) region, with a unique clinical And imaging performance. This article intends to discuss the clinical features of HOD in order to enhance its understanding. Methods The clinical data of 11 HOD patients were retrospectively analyzed. Results There were 10 males and 1 females, with an average age of 53.5 years. Primary disease, including cerebral hemorrhage in 1 case, 8 cases of pontine hemorrhage, pontine infarction in 1 case, 1 case of pontine glioma. HOD clinical manifestations of dizziness, blurred vision, nystagmus, verbal ambiguity, soft palate clonus, increased muscle tone, involuntary movements, postural disorders, ataxia and other symptoms, the average later than the primary disease 3.8 months. MRI showed the lower olivary nucleus volume and T2WI high signal. When the primary lesion involves the unilateral pons covered with red nucleus, HOD occurs on the same side and the clinical symptoms appear on the contralateral side. When the primary lesion involves bilateral red nucleus or pons, the HOD and clinical symptoms appear in the double side. Baclofen, clonazepam and carbamazepine can reduce clinical symptoms. Conclusion DROP area should be alert to the occurrence of HOD after the lesion. Delayed damage is an important clue for diagnosis. Ataxia and involuntary movements are the most common clinical manifestations. MRI can help confirm the diagnosis.