특발성 척수 탈출증 = Idiopathic Spinal Cord Herniation

다국어 초록 (Multilingual Abstract)

Study Design: Literature review.
Objectives: The aim of this study was to provide insight into idiopathic spinal cord herniation (ISCH) in terms of clinical presentation, pathophysiology, diagnosis, classification, and treatment.
Summary of Literature Review: ISCH is a rare disorder characterized by anterior displacement of the spinal cord through a ventral dural defect. It has increasingly been recognized and described over the past 10 years.
Materials and Methods: Review of the English-language literature on ISCH.
Results: ISCH occurs in middle-aged adults with a female preponderance. The most common clinical presentation is Brown- Sequard syndrome, which can progress to spastic paraparesis. Its pathophysiology is unknown. However, some authors proposed that inflammation may play an important role in the emergence of a dural defect. Magnetic resonance imaging typically shows an anterior kink of the thoracic spinal cord with an obliteration of the ventral subarachnoid space and the widened dorsal subarachnoid space.
Surgery is generally recommended for patients with motor deficits or progressive neurological symptoms. The posterior approach has been used because it allows wide exposure of the spinal cord. The surgical treatment of ISCH consists of spinal cord reduction from the ventral dural defect, which can be managed with enlargement, direct repair, or duraplasty (dural repair with a patch). In recent years, duraplasty has been used more frequently than enlargement of the dural defect.
Conclusions: ISCH causing thoracic myelopathy could be safely treated with surgical management. The possibility of this disease should be kept in mind when treating patients with progressive myelopathy.

국문 초록 (Abstract)

연구계획: 문헌 고찰목적: 특발성 척수 탈출증의 임상 양상, 병태 생리, 진단, 분류 및 치료에 대해 알아보고자 하였다.
선행문헌 요약: 특발성 척수 탈출증은 척수가 전방의 경막 결손을 통하여 빠져 나가는 질환으로 비교적 드물게 발생하지만, 최근 10년 동안 빠르게 인식되면서 많은 증례가 보고되고 있다.
대상 및 방법: 영문으로 보고된 특발성 척수 탈출증에 대한 문헌을 조사하여 고찰하였다.
결과: 특발성 척수 탈출증은 주로 성인 중년층에서 발생하며 여자에서 호발된다. 가장 흔한 임상 양상은 Brown-Sequard 증후군이며, 이것이 악화되어강직성 부전 마비로 나타날 수도 있다. 정확한 발생 기전을 알려져 있지 않지만, 염증이 경막 결손을 형성하는 데 중요한 역할을 한다는 주장은 설득력이있다. 자기 공명 영상에서 흉추부 척수가 전방으로 전위되어 있으면서 척수 전방에 지주막하 공간이 소실되어 있고, 척수 후방의 지주막하 공간은 넓어진소견을 보인다. 수술은 일반적으로 근력 약화가 있거나 신경학적 증상이 진행하는 경우에 필요하다. 광범위하게 척수를 노출시킬 수 있는 후방 수술이 이용된다. 수술은 빠져 나간 척수를 경막내로 이동시킨 다음, 경막 결손에 대해서는 경막 결손을 더 크게 하는 방법, 직접 봉합하는 방법, 패취를 이용한 경막성형술을 시행하는 방법이 있다. 최근에는 경막성형술이 더 자주 사용되어 보고되고 있다.
결론: 특발성 척수 탈출증은 흉추 척수증을 유발할 수 있으나 수술로 비교적 안전하게 치료될 수 있다. 진행성 척수증을 가진 환자를 진료할 때 이 질환을반드시 유념하여야 한다.


약칭 제목: 특발성 척수 탈출증

참고문헌 (Reference)

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