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孤立性脊髓结节病:一种神秘的脊髓内病理——病例报告及文献综述

Isolated spinal neurosarcoidosis: An enigmatic intramedullary spinal cord pathology-case report and review of the literature.

作者信息

Kasliwal Manish K, Harbhajanka Aparna, Nag Sukriti, O'Toole John E

机构信息

Department of Neurosurgery, RUSH University Medical Center, Chicago, Illinois, USA.

Department of Pathology, RUSH University Medical Center, Chicago, Illinois, USA.

出版信息

J Craniovertebr Junction Spine. 2013 Jul;4(2):76-81. doi: 10.4103/0974-8237.128536.

DOI:10.4103/0974-8237.128536
PMID:24744567
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3980561/
Abstract

Isolated spinal cord neurosarcoidosis (NS) in the absence of systemic disease or intracranial involvement is exceptionally rare. Adjunctive laboratory tests though useful may not be reliable and the absence of any pathognomonic radiological features makes the diagnosis difficult. As spinal cord NS may be a presenting feature of systemic sarcoidosis which may be occult on routine workup, (18)F-fluorodeoxyglucose-positron emission tomography (FDG-PET) may be of value in unraveling this systemic involvement avoiding biopsying the spinal cord. A case of truly isolated NS is described with review of literature on this enigmatic pathology. Long segment intramedullary signal changes with focal parenchymal along with dural/meningeal enhancement in the absence of significant cervical stenosis in a young patient of northern European or African-American decent is very suggestive of NS and although may be presumably treated with steroids; there should be a low threshold for spinal cord biopsy especially in the absence of response to steroids to confirm isolated spinal cord NS in a patient with clinical neurological deterioration.

摘要

孤立性脊髓结节病(NS),在无全身性疾病或颅内受累的情况下极为罕见。辅助实验室检查虽有用但可能不可靠,且缺乏任何特征性影像学表现使得诊断困难。由于脊髓NS可能是全身性结节病的首发表现,而全身性结节病在常规检查中可能隐匿,(18)F - 氟脱氧葡萄糖 - 正电子发射断层扫描(FDG - PET)对于揭示这种全身性受累情况可能有价值,从而避免对脊髓进行活检。本文描述了一例真正的孤立性NS病例,并回顾了关于这种神秘病理学的文献。在一名北欧或非裔美国血统的年轻患者中,出现长节段髓内信号改变伴局灶性实质改变以及硬脊膜/脑膜强化,且无明显颈椎管狭窄,这非常提示NS,尽管可能推测用类固醇治疗;对于脊髓活检应保持较低阈值,特别是在对类固醇无反应且患者临床神经功能恶化以确诊孤立性脊髓NS的情况下。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7587/3980561/e6368ae24c1c/JCVJS-4-76-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7587/3980561/a8c7e94b5742/JCVJS-4-76-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7587/3980561/345216d7247e/JCVJS-4-76-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7587/3980561/91ceafd56a1a/JCVJS-4-76-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7587/3980561/5a39b6cbc47d/JCVJS-4-76-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7587/3980561/e6368ae24c1c/JCVJS-4-76-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7587/3980561/a8c7e94b5742/JCVJS-4-76-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7587/3980561/345216d7247e/JCVJS-4-76-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7587/3980561/91ceafd56a1a/JCVJS-4-76-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7587/3980561/5a39b6cbc47d/JCVJS-4-76-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7587/3980561/e6368ae24c1c/JCVJS-4-76-g005.jpg

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