Department of Neurology, SCTIMST, Trivandrum, India.
Department of Imaging Sciences and Interventional Radiology, SCTIMST, Trivandrum, India.
J Spinal Cord Med. 2021 May;44(3):437-441. doi: 10.1080/10790268.2019.1579986. Epub 2019 Mar 14.
Idiopathic spinal cord herniation (ISCH) remains an obscure, under recognised but potentially treatable entity among the etiologies of compressive myelopathy. Here, we present a retrospective case series of eight patients of ISCH with their clinical features, imaging findings and long-term follow up data along with a relevant review of the literature. Mean age at presentation was 36 years with the duration of symptoms ranging from two months to more than thirty years. Contrary to existing data, only two patients in our series had the classical presentation of Brown-Sequard syndrome ( = 2). The clinical spectrum of the presentation was broad with pure spastic quadriparesis ( = 3), girdle sensation ( = 1) and radiculopathy ( = 1), the latter presentation has been unreported for ISCH. The typical radiological picture was seen in all patients. All except one patient was managed conservatively. In the one patient who underwent bilateral dentate ligament excision, there was mild functional improvement on long-term follow-up despite a definite worsening of the radiological picture. Five of the patients had a long-term follow-up with no clinically or functionally significant worsening. The current study highlights the wide clinical spectrum and relatively stable natural course of ISCH and reports on hitherto undescribed presentation as radiculopathy.
特发性脊髓脊膜膨出(ISCH)仍然是一种病因不明、认识不足但可能治疗的压迫性脊髓病。在此,我们回顾性分析了 8 例 ISCH 患者的临床特征、影像学表现和长期随访资料,并对文献进行了综述。患者的平均发病年龄为 36 岁,症状持续时间从 2 个月到 30 多年不等。与现有数据不同,我们的系列研究中只有 2 例患者出现典型的 Brown-Sequard 综合征(2/8,25%)。临床表现的范围很广,包括单纯痉挛性四肢瘫痪(3/8,37.5%)、束带感(1/8,12.5%)和神经根病(1/8,12.5%),后一种表现以前未报道过。所有患者均表现出典型的影像学特征。除 1 例患者外,其余患者均接受保守治疗。在接受双侧齿状韧带切除术的 1 例患者中,尽管影像学表现明显恶化,但在长期随访中仍有轻度的功能改善。5 例患者进行了长期随访,无明显的临床或功能恶化。本研究强调了 ISCH 的广泛临床表现和相对稳定的自然病程,并报告了以前未描述的神经根病表现。