马罗-拉米综合征中因颈段硬脊膜弥漫性增厚导致的脊髓病：一例报告

Myelopathy due to diffuse thickening of the cervical dura mater in Maroteaux-Lamy syndrome: report of a case.

作者信息

Tamaki N, Kojima N, Tanimoto M, Suyama T, Matsumoto S

机构信息

Department of Neurosurgery, Kobe University School of Medicine, Japan.

出版信息

Neurosurgery. 1987 Sep;21(3):416-9. doi: 10.1227/00006123-198709000-00027.

DOI:10.1227/00006123-198709000-00027

PMID:3118237

Abstract

A rare case of diffuse thickening of the cervical dura mater leading to cord compression in Maroteaux-Lamy syndrome (mucopolysaccharidosis VI) is reported. Spinal computed tomographic scans after intrathecal metrizamide injection and magnetic resonance images are shown. This condition presents as cervical myelopathy. Early decompression can be beneficial for patients with this infrequent metabolic disorder.

摘要

本文报告了一例罕见的马罗-拉米综合征（黏多糖贮积症VI型）患者，其颈段硬脊膜弥漫性增厚导致脊髓受压。文中展示了鞘内注射甲泛葡胺后的脊髓计算机断层扫描图像及磁共振成像。这种情况表现为颈髓病。早期减压对患有这种罕见代谢紊乱疾病的患者可能有益。