Ortiz Torres Michael, Vicenty-Padilla Juan C, Cay-Martinez Karla C, Labat Eduardo J, Vigo-Prieto Juan
Neurological Surgery, University of Missouri School of Medicine, Columbia, USA.
Neurological Surgery, Brigham and Women's Hospital, Harvard Medical School, Boston, USA.
Cureus. 2020 Oct 23;12(10):e11109. doi: 10.7759/cureus.11109.
Hypertrophic olivary degeneration (HOD) is a rare type of neuronal degeneration seen after interruption of the dentato-rubro-olivary tract also known as the Guillain-Mollaret triangle (GMT). It is associated with hypertrophic changes of the inferior olive. Commonly reported in adults, this lesion presents with ataxia and oculopalatal myoclonus. Up to date, few cases have been published in the literature that refer to pediatric cases. This diagnosis is particularly important in the setting of brainstem tumor surgery as it should not be confused for tumor recurrence or metastasis, in turn avoiding unwarranted surgical intervention. We present the case of a 15-year-old male who underwent resection of a left superior cerebellar peduncle (SCP) pilocytic astrocytoma. On follow-up, magnetic resonance imaging (MRI) demonstrated evidence of mild residual tumor as well as progressive engorgement of the inferior olivary nucleus (ION). The patient was clinically asymptomatic and has since been observed expectantly without any issues. We were able to pinpoint the most probable location of injury in our patient's GMT. HOD remains a somewhat obscure entity. Its presentation may be early and not accompanied by significant neurologic findings, in contrast to what has been previously reported. Particularly in neoplastic cases, it may represent a diagnostic challenge and could be easily confused for tumor recurrence. A multidisciplinary approach for this entity, as with other pathologies, is of particular importance. Its proper recognition will result in the best outcomes for the patient.
肥大性橄榄核变性(HOD)是一种罕见的神经元变性类型,见于齿状红核橄榄束(也称为 Guillain-Mollaret 三角,GMT)中断后。它与下橄榄核的肥大性改变有关。常见于成人,该病变表现为共济失调和动眼腭肌阵挛。迄今为止,文献中报道的小儿病例很少。在脑干肿瘤手术中,这一诊断尤为重要,因为不应将其与肿瘤复发或转移相混淆,从而避免不必要的手术干预。我们报告一例 15 岁男性患者,其接受了左侧小脑上脚(SCP)毛细胞型星形细胞瘤切除术。随访时,磁共振成像(MRI)显示有轻度残留肿瘤以及下橄榄核(ION)逐渐充血的证据。患者临床无症状,此后一直进行观察,未出现任何问题。我们能够确定患者 GMT 中最可能的损伤部位。HOD 仍然是一个有些模糊的实体。与之前报道的情况相反,其表现可能较早且不伴有明显的神经系统表现。特别是在肿瘤病例中,它可能是一个诊断挑战,容易与肿瘤复发相混淆。与其他病理情况一样,针对这一实体采用多学科方法尤为重要。正确识别它将为患者带来最佳治疗效果。