Rieder Carlos R M, Rebouças Ricardo Gurgel, Ferreira Marcelo Paglioli
Clínica de Distúrbios do Movimento, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brasil.
Arq Neuropsiquiatr. 2003 Jun;61(2B):473-7. doi: 10.1590/s0004-282x2003000300028. Epub 2003 Jul 28.
Hypertrophic olivary degeneration (HOD) is a rare type of neuronal degeneration involving the dento-rubro-olivary pathway and presents clinically as palatal tremor. We present a 48 year old male patient who developed Holmes' tremor and bilateral HOD five months after brainstem hemorrhage. The severe rest tremor was refractory to pharmacotherapy and botulinum toxin injections, but was markedly reduced after thalamotomy. Magnetic resonance imaging permitted visualization of HOD, which appeared as a characteristic high signal intensity in the inferior olivary nuclei on T2- and proton-density-weighted images. Enlargement of the inferior olivary nuclei was also noted. Palatal tremor was absent in that moment and appears about two months later. The delayed-onset between insult and tremor following structural lesions of the brain suggest that compensatory or secondary changes in nervous system function must contribute to tremor genesis. The literature and imaging findings of this uncommon condition are reviewed.
肥大性橄榄核变性(HOD)是一种罕见的神经元变性类型,累及齿状红核橄榄束通路,临床上表现为腭震颤。我们报告一名48岁男性患者,在脑干出血五个月后出现霍姆斯震颤和双侧HOD。严重的静止性震颤对药物治疗和肉毒杆菌毒素注射均无效,但在丘脑切开术后明显减轻。磁共振成像可显示HOD,其在T2加权像和质子密度加权像上表现为下橄榄核特征性高信号强度。还注意到下橄榄核增大。当时无腭震颤,约两个月后出现。脑部结构性病变后损伤与震颤之间的延迟发作提示,神经系统功能的代偿性或继发性改变必定促成了震颤的发生。本文对这种罕见病症的文献及影像学表现进行了综述。