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特发性脊髓疝:病例报告及文献综述

Idiopathic spinal cord herniation: case report and review of the literature.

作者信息

Sasani Mehdi, Ozer Ali F, Vural Metin, Sarioglu Ali C

机构信息

Neurosurgery Department, VKF American Hospital, Istanbul, Turkey.

出版信息

J Spinal Cord Med. 2009;32(1):86-94. doi: 10.1080/10790268.2009.11760757.

Abstract

BACKGROUND

Idiopathic spinal cord herniation (ISCH) is a rare cause of progressive myelopathy frequently present in Brown-Séquard syndrome. Preoperative diagnosis can be made with magnetic resonance imaging (MRI). Many surgical techniques have been applied by various authors and are usually reversible by surgical treatment.

METHODS

Case report and review of the literature.

FINDINGS

A 45-year-old woman with Brown-Séquard syndrome underwent thoracic MRI, which revealed transdural spinal cord herniation at T8 vertebral body level. During surgery the spinal cord was reduced and the ventral dural defect was restorated primarily and reinforced with a thin layer of subdermal fat. The dural defect was then closed with interrupted stitches.

RESULTS

Although neurologic status improved postoperatively, postsurgical MRI demonstrated swelling and abnormal T2-signal intensity in the reduced spinal cord. Review of the English language literature revealed 100 ISCH cases.

CONCLUSIONS

ISCH is a rare clinical entity that should be considered in differential diagnosis of Brown-Séquard syndrome, especially among women in their fifth decade of life. Outcome for patients who initially had Brown-Séquard syndrome was significantly better than for patients who presented with spastic paralysis. Although progression of neurologic deficits can be very slow, reduction of the spinal cord and repair of the defect are crucial in stopping or reversing the deterioration.

摘要

背景

特发性脊髓疝(ISCH)是导致进行性脊髓病的罕见病因,常表现为布朗 - 塞卡尔综合征。术前可通过磁共振成像(MRI)做出诊断。许多作者应用了多种手术技术,且手术治疗通常可逆转病情。

方法

病例报告及文献回顾。

结果

一名患有布朗 - 塞卡尔综合征的45岁女性接受了胸椎MRI检查,结果显示在T8椎体水平存在经硬膜脊髓疝。手术过程中,脊髓复位,腹侧硬膜缺损首先进行修复,并用一层薄的皮下脂肪加固。然后用间断缝线关闭硬膜缺损。

结果

尽管术后神经功能状态有所改善,但术后MRI显示复位后的脊髓肿胀且T2信号强度异常。对英文文献的回顾发现了100例ISCH病例。

结论

ISCH是一种罕见的临床病症,在布朗 - 塞卡尔综合征的鉴别诊断中应予以考虑,尤其是在50岁左右的女性中。最初表现为布朗 - 塞卡尔综合征的患者的预后明显优于表现为痉挛性瘫痪的患者。尽管神经功能缺损的进展可能非常缓慢,但脊髓复位和缺损修复对于阻止或逆转病情恶化至关重要。

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