Department of Neurology, Shengjing Hospital of China Medical University, No. 36 Sanhao Street, Heping District, Shenyang, Liaoning, 110004, P. R. China.
J Integr Neurosci. 2020 Sep 30;19(3):507-511. doi: 10.31083/j.jin.2020.03.1238.
Transsynaptic degeneration in the cerebellum and brainstem may give rise to a rare neurological condition with various clinical manifestations, namely hypertrophic olivary degeneration. The classical manifestations of hypertrophic olivary degeneration comprise myoclonus, palatal tremor, ataxia, and ocular symptoms. Any lesions interrupting the dentate-rubro-olivary pathway, referred to as the anatomic Guillain-Mollaret triangle, contribute to the broad aetiologies of hypertrophic olivary degeneration. The clinical diagnosis depends primarily on the associated symptoms and the characteristic magnetic resonance imaging findings. Concerning treatment and prognosis, there are no widely accepted guidelines. Here, we identified 11 cases of hypertrophic olivary degeneration secondary to brainstem infarction from 1964 to the present. Combined with two of our cases, the clinical and imaging findings of 13 patients with hypertrophic olivary degeneration secondary to brainstem infarction were studied. A meta-analysis of case studies gives the correlation coefficient between infraction location and time to develop hypertrophic olivary degeneration as 0.217 ( = 0.393, > 0.05). At the significance level of < 0.05, there was no significant correlation between information location and time to develop hyperophic olivary degeneration. The χ between infraction location and magnetic resonance imaging findings of hypertrophic olivary degeneration was 8.750 ( = 0.364, > 0.05). At the significance level of < 0.05, there was no significant correlation between infraction location and magnetic resonance imaging findings of hypertrophic olivary degeneration. Conclusion based on the analysis of available data suggests that when newly developed or progressive worsening motor symptoms are presented in patients with previous brainstem infarction, a diagnosis of hypertrophic olivary degeneration should be investigated.
小脑和脑干的跨突触变性可能导致一种罕见的具有多种临床表现的神经疾病，即橄榄体肥大性变性。橄榄体肥大性变性的典型表现包括肌阵挛、腭震颤、共济失调和眼部症状。任何中断齿状核-红核-橄榄体通路的病变，称为解剖学的Guillain-Mollaret 三角，都可能导致橄榄体肥大性变性的广泛病因。临床诊断主要取决于相关症状和特征性磁共振成像发现。关于治疗和预后，目前尚无广泛接受的指南。在此，我们从 1964 年至今共发现 11 例继发于脑干梗死的橄榄体肥大性变性病例。结合我们的 2 例病例，研究了 13 例继发于脑干梗死的橄榄体肥大性变性患者的临床和影像学表现。对病例研究的荟萃分析得出梗死部位与发生橄榄体肥大性变性的时间之间的相关系数为 0.217（ = 0.393，> 0.05）。在显著性水平 < 0.05 时，梗死部位与发生橄榄体肥大性变性的时间之间没有显著相关性。橄榄体肥大性变性的梗死部位与磁共振成像发现之间的 χ 为 8.750（ = 0.364，> 0.05）。在显著性水平 < 0.05 时，梗死部位与磁共振成像发现之间没有显著相关性。根据对现有数据的分析得出的结论表明，当先前患有脑干梗死的患者出现新出现或进行性加重的运动症状时，应考虑诊断为橄榄体肥大性变性。
