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颅内和脊髓海绵状血管畸形并存预示着侵袭性临床表现。

Coexistence of Intracranial and Spinal Cord Cavernous Malformations Predict Aggressive Clinical Presentation.

作者信息

Ren Jian, Hong Tao, He Chuan, Sun Liyong, Li Xiaoyu, Ma Yongjie, Yu Jiaxing, Ling Feng, Zhang Hongqi

机构信息

Department of Neurosurgery, China International Neuroscience Institute, Xuanwu Hospital, Capital Medical University, Beijing, China.

出版信息

Front Neurol. 2019 Jun 13;10:618. doi: 10.3389/fneur.2019.00618. eCollection 2019.

DOI:10.3389/fneur.2019.00618
PMID:31249549
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6584750/
Abstract

Patients with spinal cord cavernous malformations (CMs) are at increased risk for multiple neuraxis CMs. Few studies focused on the natural history of patients with coexistence of intracranial and spinal cord CMs. Forty patients who underwent both intracranial and spinal MR imaging from a single center were reviewed retrospectively. American Spinal Injury Association (ASIA) impairment scale was used to evaluate neurological and disability status. The median age of the 40 patients was 40.6 years old (range 9-69), and the male-to-female ratio was 2.1:1. The mean size of the intramedullary lesions was 10.1 ± 5.8 mm (range, 3.0-34.0 mm). Six patients (15%) harbored at least one intracranial lesion. Five of the 6 patients (83.3%) suffered aggressive clinical presentations with severe neurological and disability status; in patients with sporadic spinal CMs, the proportion was 26.5% (9 of 34). Coexistence of intracranial and spinal cord CMs is more likely to follow an aggressive course ( = 0.031, OR = 19.547, 95% CI = 1.322-289.123). In the postoperative long-term follow up, the unfavorable rate of patients with associated intracranial CMs was significantly higher than that of patients with sporadic spinal cord CMs ( = 0.049). The prevalence of associated intracranial CMs in patients with spinal cord CMs was 15%. Coexistence of intracranial and spinal cord CMs is more likely to follow an aggressive course. This study highlights the necessity of intracranial MR imaging for patients with spinal cord CMs to benefit to the predicting prognosis and selection of treatment strategies.

摘要

患有脊髓海绵状血管畸形(CMs)的患者发生多发性神经轴CMs的风险增加。很少有研究关注颅内和脊髓CMs共存患者的自然病史。对来自单一中心的40例接受颅内和脊髓磁共振成像检查的患者进行了回顾性研究。采用美国脊髓损伤协会(ASIA)损伤量表评估神经功能和残疾状况。40例患者的中位年龄为40.6岁(范围9 - 69岁),男女比例为2.1:1。髓内病变的平均大小为10.1±5.8mm(范围3.0 - 34.0mm)。6例患者(15%)至少有1个颅内病变。这6例患者中有5例(83.3%)出现侵袭性临床表现,伴有严重的神经功能和残疾状况;在散发性脊髓CMs患者中,这一比例为26.5%(34例中的9例)。颅内和脊髓CMs共存更有可能呈现侵袭性病程(P = 0.031,OR = 19.547,95%CI = 1.322 - 289.123)。在术后长期随访中,伴有颅内CMs患者的不良率显著高于散发性脊髓CMs患者(P = 0.049)。脊髓CMs患者中伴有颅内CMs的患病率为15%。颅内和脊髓CMs共存更有可能呈现侵袭性病程。本研究强调了对脊髓CMs患者进行颅内磁共振成像检查对于预测预后和选择治疗策略的必要性。

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本文引用的文献

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Familial Cerebral Cavernous Malformations.家族性脑海绵状血管畸形
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Cranial Cavernous Malformations: Natural History and Treatment.颅内海绵状血管畸形:自然病史与治疗
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