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I型Chiari畸形合并颈胸段脊髓空洞症伪装成双臂肌萎缩:一例报告

Chiari malformation type I with cervicothoracic syringomyelia masquerading as bibrachial amyotrophy: a case report.

作者信息

Mora Jeffrey R, Rison Richard A, Beydoun Said R

机构信息

Keck School of Medicine, Neuromuscular Division, University of Southern California, 1520 San Pablo Street, Suite 3000, Los Angeles, CA, 90033, USA.

Department of Neurology, University of Southern California Keck School of Medicine, Los Angeles County Medical Center, 1520 San Pablo Street, Suite 3000, Los Angeles, CA, 90033, USA.

出版信息

J Med Case Rep. 2015 Jan 27;9:11. doi: 10.1186/1752-1947-9-11.

DOI:10.1186/1752-1947-9-11
PMID:25622641
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4417345/
Abstract

INTRODUCTION

Clinical presentation of syringomyelia can mimic a variety of neuromuscular disorders. A misdiagnosis can result in progressive pressure on the spinal cord, causing the development of severe irreversible neurologic deficits.

CASE PRESENTATION

We report the very unusual case of a 50-year-old Latino man who developed severe distal muscle atrophy and bulbar dysfunction as a result of Chiari malformation type I with chronic cervicothoracic syringomyelia.

CONCLUSION

Syringomyelia is a potentially serious neurologic condition with symptoms that can mimic other neuromuscular disorders. Severe untreated cases can result in irreversible spinal cord injury. Prompt diagnosis with magnetic resonance imaging is important in both establishing diagnosis and directing further surgical management.

摘要

引言

脊髓空洞症的临床表现可能类似于多种神经肌肉疾病。误诊可能导致脊髓受到渐进性压迫,从而引发严重的不可逆神经功能缺损。

病例报告

我们报告了一例非常罕见的病例,一名50岁的拉丁裔男性因I型Chiari畸形合并慢性颈胸段脊髓空洞症而出现严重的远端肌肉萎缩和延髓功能障碍。

结论

脊髓空洞症是一种潜在的严重神经系统疾病,其症状可能类似于其他神经肌肉疾病。未经治疗的严重病例可导致不可逆的脊髓损伤。磁共振成像的及时诊断对于确立诊断和指导进一步的手术治疗都很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/268c/4417345/ad3a80c02dc8/13256_2014_3147_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/268c/4417345/2074fadf21a1/13256_2014_3147_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/268c/4417345/19517b89ca1a/13256_2014_3147_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/268c/4417345/961d992ca898/13256_2014_3147_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/268c/4417345/435bad9ec5ab/13256_2014_3147_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/268c/4417345/ad3a80c02dc8/13256_2014_3147_Fig5_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/268c/4417345/2074fadf21a1/13256_2014_3147_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/268c/4417345/19517b89ca1a/13256_2014_3147_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/268c/4417345/961d992ca898/13256_2014_3147_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/268c/4417345/435bad9ec5ab/13256_2014_3147_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/268c/4417345/ad3a80c02dc8/13256_2014_3147_Fig5_HTML.jpg

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Evid Based Spine Care J. 2013 Oct;4(2):116-25. doi: 10.1055/s-0033-1357362.
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Pathogenesis of syringomyelia associated with Chiari type 1 malformation: review of evidences and proposal of a new hypothesis.与 Chiari Ⅰ 型畸形相关的脊髓空洞症的发病机制:证据回顾与新假说的提出。
Neurosurg Rev. 2010 Jul;33(3):271-84; discussion 284-5. doi: 10.1007/s10143-010-0266-5. Epub 2010 Jun 8.
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Defining the line between hydromyelia and syringomyelia. A differentiation is possible based on electrophysiological and magnetic resonance imaging studies.
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The natural history of the Chiari Type I anomaly.Chiari I型畸形的自然病史。
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