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脑干海绵状血管畸形中的肥大性橄榄核变性:预测因素及临床意义分析

Hypertrophic Olivary Degeneration in Brainstem Cavernous Malformations: An Analysis of Predictors and Clinical Implications.

作者信息

Bektas Delal, Lanzino Giuseppe, Graepel Stephen, Flemming Kelly D

机构信息

Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota, USA.

Department of Neurosurgery, University Hospital Zurich, Zurich, Switzerland.

出版信息

Neurosurgery. 2025 Jul 17. doi: 10.1227/neu.0000000000003631.

Abstract

BACKGROUND AND OBJECTIVES

This study aimed to evaluate predictors of hypertrophic olivary degeneration (HOD) in patients with brainstem cerebral cavernous malformations (CCMs) and to examine its association with functional outcomes.

METHODS

A cohort of 120 patients with brainstem CCM was analyzed using prospectively collected registry data and retrospective chart reviews. Demographic, clinical, radiological, and surgical data were evaluated. Predictors of HOD and poor functional outcomes were identified through univariate and multivariate analyses.

RESULTS

The cohort included 54 females (45.0%), with a mean age at diagnosis of 43.8 years (SD = 16.5). Symptomatic hemorrhage was present in 70.8% of patients at diagnosis, and 28.3% underwent surgical intervention. Radiographic HOD was observed in 20% of patients (n = 24), with 87% of cases involving lesions within the Guillain-Mollaret triangle (GMT). Larger lesion size (odds ratio [OR] = 1.09, 95% CI: 1.01-1.19, P = .027), pontine location (OR = 19.81, P = .006), and GMT involvement (OR = 25.24, P < .001) were significantly associated with HOD. Repeated symptomatic hemorrhage and surgery, including the extent of resection, did not predict the development of HOD. Patients with HOD had worse functional outcomes at last follow-up (P = .012), with GMT involvement remaining the sole independent predictor of mRS ≥3 (OR = 3.44, P = .040).

CONCLUSION

HOD reflects trans-synaptic degeneration within the GMT and is strongly associated with lesion location and size. It serves as a radiological marker of cumulative damage to the brainstem. Preoperative imaging and risk stratification focusing on GMT involvement are critical for guiding management and counseling patients with brainstem CCM.

摘要

背景与目的

本研究旨在评估脑干脑海绵状血管畸形(CCM)患者肥厚性橄榄核变性(HOD)的预测因素,并探讨其与功能预后的关系。

方法

使用前瞻性收集的登记数据和回顾性病历审查,对120例脑干CCM患者进行队列分析。评估人口统计学、临床、放射学和手术数据。通过单因素和多因素分析确定HOD和功能预后不良的预测因素。

结果

该队列包括54名女性(45.0%),诊断时的平均年龄为43.8岁(标准差=16.5)。70.8%的患者在诊断时有症状性出血,28.3%的患者接受了手术干预。20%的患者(n=24)观察到影像学HOD,其中87%的病例累及Guillain-Mollaret三角(GMT)内的病变。较大的病变大小(比值比[OR]=1.09,95%可信区间:1.01-1.19,P=.027)、脑桥位置(OR=19.81,P=.006)和GMT累及(OR=25.24,P<.001)与HOD显著相关。反复出现症状性出血和手术,包括切除范围,不能预测HOD的发生。HOD患者在最后一次随访时功能预后较差(P=.012),GMT累及仍然是改良Rankin量表(mRS)≥3的唯一独立预测因素(OR=3.44,P=.040)。

结论

HOD反映了GMT内的跨突触变性,与病变位置和大小密切相关。它是脑干累积损伤的影像学标志。术前影像学检查和关注GMT累及的风险分层对于指导脑干CCM患者的管理和咨询至关重要。

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