| 李辉
，黄波
，徐沙贝
，朱文浩.三例脑干出血继发肥大性下橄榄核变性的临床分析并文献复习[J].神经损伤功能重建,2022,17(11):621-627 |
| 三例脑干出血继发肥大性下橄榄核变性的临床分析并文献复习 |
| Hypertrophic Olivary Degeneration Secondary to Brainstem Hemorrhage: Clinical Analysis ofThree Cases and Literature Review |
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| DOI： |
| 中文关键词: 肥大性下橄榄核变性 齿状核-红核-下橄榄核环路 磁共振 临床表现 |
| 英文关键词: hypertrophic olivary degeneration dentato-rubro-olivary pathway magnetic resonance imaging clinical manifestation |
| 基金项目:湖北省自然科学基
金（No. 2021CFB3
82） |
|
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| 中文摘要: |
| 目的：对三例肥大性下橄榄核变性（HOD）患者的临床及影像学资料进行总结分析，并对既往文献报
道的脑干出血继发HOD病例进行系统性分析。方法：对三例确诊HOD的住院患者的临床资料进行分析，
并在PubMed、EMbase数据库中检索既往脑干出血继发HOD相关病例并进行归纳总结。结果：三例患者平
均年龄57.3岁，HOD的临床症状分别延迟于原发病变4月、3月、4月出现。主要临床表现包括腭肌阵挛、眼
球震颤、头部震颤、共济失调等。原发病灶均在脑桥；在MRI上均表现为体积增大的下橄榄核及T2WI高信
号。药物治疗效果有限。既往病例共39例，男25例（65.9%），女14例（34.1%）；平均年龄50.7岁；原发病变
为海绵状血管瘤出血16例（41.0%），原发性脑干出血23例（59.0%）；临床症状迟于原发病3月至15年出现
（中位时间6月），主要临床表现包括腭肌阵挛（38.5%）、肢体或头部震颤（33.3%）和眼球震颤（30.8%）等。
治疗方式主要包括药物治疗和手术治疗，其中药物治疗的好转率46.2%；手术治疗的患者3例，均明显好
转。结论：HOD的诊断主要依赖MRI和典型临床表现，但各病例间临床特征的异质性较大。脑干出血继
发HOD患者中ION单侧受累较多，出现Holmes震颤的比例相对高，药物治疗总体效果有限。 |
| 英文摘要: |
| To summarize and analyze clinical and imaging data of three patients with hypertrophic
olivary degeneration (HOD) and to systematically analyze the previous reported cases of HOD secondary to
brainstem hemorrhage. Methods: The clinical features of 3 patients diagnosed with HOD were described.
Furthermore, previous published cases of HOD secondary to brainstem hemorrhage were searched in PUBMED
and Embase and retrospectively analyzed. Results: The average age of the 3 patients was 57.3 years. The
symptoms of HOD were detected 4, 3, and 4 months after the primary neurological events respectively. The
main clinical presentations included palatal myoclonus, nystagmus, head tremor, and ataxia. The primary lesions
were all located in the pons. MRI disclosed enlarged inferior olivary nucleus (ION) and hyperintensity on
T2-weighted images. All patients responded poorly to drug treatments. A total of 39 previous reported cases
were included in the study. The mean age was 50.7 years for 25 (65.9%) males and 14 (34.1%) females. HOD
occurred secondary to hemorrhage from cavernous hemangioma in 16 (41.0% ) cases and primary brainstem
hemorrhage in 23 (59.0%) cases. The clinical symptoms of HOD were detected a median of 6 months (range, 3
months～15 years) after the primary neurological insult. The most frequent clinical manifestations were palatine
myoclonus (38.5%), limb or head tremors (33.3%), and nystagmus (30.8%). Treatments included pharmacotherapy and surgery. Symptoms in 6/13 (46.2% ) cases improved after medication, and marked improvement was
observed in all 3 patients who underwent surgical treatment. Conclusion: The diagnosis of HOD mainly
depends on MRI and typical clinical manifestations, whereas the clinical features of HOD vary greatly among
cases. Unilateral ION involvement and Holmes tremor is more frequently detected in HOD secondary to
brainstem hemorrhage, and the effect of medical treatment is overall limited. |
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