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

Ling Chen, Guangming Gong, Jun-fu Wu, Zhu Li, J. Qin
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Abstract

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. Key words: Hypertrophic olivary degeneration; Magnetic resonance imaging; Susceptibility weighted imaging
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增生性橄榄变性:6例回顾性分析并文献复习
目的探讨肥厚性橄榄变性(HOD)的临床特点。方法回顾性分析2013年1月至2018年12月郑州大学第一附属医院神经内科诊治的6例HOD患者的临床资料,并对患者进行1次电话随访。结果单侧HOD 4例,双侧HOD 2例。临床表现为共济失调5例,肢体震颤3例,双侧眼球震颤2例,双侧腭肌震颤1例,肢体疼痛1例,全身肌颤1例,复视1例。磁共振成像(MRI) T2加权成像呈高信号,T1加权成像呈微低信号或等强信号。3例患者行敏感性加权成像(SWI),未见红核密度明显增加。1例患者在液体衰减反转恢复(FLAIR)中表现为高强度。1例患者行MRI增强检查,但下橄榄核未见强化。结论HOD除表现出典型症状外,还表现出非典型症状。MRI能清楚显示橄榄核继发性变性,SWI不能显示红色变性。药物治疗肢体震颤效果不佳。整体预后较差,但部分患者肢体震颤症状可随时间改善。关键词:增生性橄榄变性;磁共振成像;磁化率加权成像
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