Department of Neurology, Beijing Aerospace Central Hospital, Peking University Aerospace Clinical College, Beijing 100049, People's Republic of China.
Department of Respiratory Medicine, Beijing Aerospace Central Hospital, Peking University Aerospace Clinical College, Beijing 100049, People's Republic of China.
Brain Res. 2019 Sep 1;1718:53-63. doi: 10.1016/j.brainres.2019.04.024. Epub 2019 Apr 23.
Hypertrophic olivary degeneration (HOD) is a rare form of trans-synaptic degeneration characterized by hypertrophy of the inferior olivary nucleus situated in the olivary body, part of the medulla oblongata, representing a major source of input to the cerebellum. HOD typically results from focal lesions interrupting connections from the inferior olive within the dentato-rubro-olivary pathway (DROP), a region also known as the Guillain-Mollaret triangle (GMT) (red nucleus, inferior olivary nucleus, and contralateral dentate nucleus). Clinically, HOD presents classically as palatal tremor and can include dentatorubral tremor and/or ocular myoclonus. Oppenheim first described the enlargement of the ION in his post-mortem study. Since then, a limited number of cases have been reported in the literatures. Thus, we intended to describe the definition, pathophysiology, clinical features, radiological features, diagnosis, and current management of HOD. We provide a comprehensive review of HOD focusing on etiology. The present review may lead to a better understanding of HOD clinical characteristic with the goal of contributing to existing knowledge of this rare disease.
橄榄体脑桥小脑萎缩（HOD）是一种罕见的跨突触变性形式，其特征是位于延髓橄榄体中的下橄榄核肥大，下橄榄核是小脑的主要传入源之一。HOD 通常由局灶性病变引起，中断了齿状核红核橄榄束（DROP）内下橄榄核的连接，该区域也称为 Guillain-Mollaret 三角（GMT）（红核、下橄榄核和对侧齿状核）。临床上，HOD 表现为典型的腭震颤，可包括齿状核红核震颤和/或眼肌阵挛。Oppenheim 首先在他的尸检研究中描述了 ION 的增大。此后，文献中报道了有限数量的病例。因此，我们旨在描述 HOD 的定义、病理生理学、临床特征、影像学特征、诊断和当前的治疗方法。我们提供了对 HOD 的全面综述，重点关注病因。本综述可能有助于更好地了解 HOD 的临床特征，目的是为这一罕见疾病的现有知识做出贡献。
