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Chiari type I malformation with cervicothoracic syringomyelia subterfuge as flail arm syndrome

Chiari type I malformation with cervicothoracic syringomyelia although very common in clinical practice usually in children can progress slowly and mimic muscular dystrophies in adulthood. We present a rare adult case of Chiari type I malformation with cervicothoracic syringomyelia subterfuge as Fla...

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Autores principales: Lan, Zhi Gang, Richard, Seidu A., Liu, Jiagang, You, Chao
Formato: Online Artículo Texto
Lenguaje:English
Publicado: PAGEPress Publications, Pavia, Italy 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5641841/
https://www.ncbi.nlm.nih.gov/pubmed/29071044
http://dx.doi.org/10.4081/ni.2017.7336
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author Lan, Zhi Gang
Richard, Seidu A.
Liu, Jiagang
You, Chao
author_facet Lan, Zhi Gang
Richard, Seidu A.
Liu, Jiagang
You, Chao
author_sort Lan, Zhi Gang
collection PubMed
description Chiari type I malformation with cervicothoracic syringomyelia although very common in clinical practice usually in children can progress slowly and mimic muscular dystrophies in adulthood. We present a rare adult case of Chiari type I malformation with cervicothoracic syringomyelia subterfuge as Flail arm syndrome. A 44-year-old man was diagnosed with congenital type I Chiari malformation with cervicothoracic syringomyelia about 21 years ago without surgery. His health status deteriorated over the years until 21 days prior to presentation when he had severe pain in the right knee. In his upper limbs, he had bilateral corresponding severe weakness of 0/5 proximal strength and 0/5 strength in his distal muscles. Magnetic resonance imaging (MRI) revealed an enlargement of the spinal cord from C1-C4 level with a mass that appeared hypo-dense on T1 and hyperdense on T2. Syringomyelia is a potentially serious neurologic condition that can mimic other neuromuscular disorders. Early detection and diagnosis with MRI is crucial to avoid irreversible neurological complications. We suggest that whether asymptomatic or symptomatic, decompressive surgery should be carried out to allow for free flow of cerebrospinal fluid thereby improving the quality of life for the patient.
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spelling pubmed-56418412017-10-25 Chiari type I malformation with cervicothoracic syringomyelia subterfuge as flail arm syndrome Lan, Zhi Gang Richard, Seidu A. Liu, Jiagang You, Chao Neurol Int Case Report Chiari type I malformation with cervicothoracic syringomyelia although very common in clinical practice usually in children can progress slowly and mimic muscular dystrophies in adulthood. We present a rare adult case of Chiari type I malformation with cervicothoracic syringomyelia subterfuge as Flail arm syndrome. A 44-year-old man was diagnosed with congenital type I Chiari malformation with cervicothoracic syringomyelia about 21 years ago without surgery. His health status deteriorated over the years until 21 days prior to presentation when he had severe pain in the right knee. In his upper limbs, he had bilateral corresponding severe weakness of 0/5 proximal strength and 0/5 strength in his distal muscles. Magnetic resonance imaging (MRI) revealed an enlargement of the spinal cord from C1-C4 level with a mass that appeared hypo-dense on T1 and hyperdense on T2. Syringomyelia is a potentially serious neurologic condition that can mimic other neuromuscular disorders. Early detection and diagnosis with MRI is crucial to avoid irreversible neurological complications. We suggest that whether asymptomatic or symptomatic, decompressive surgery should be carried out to allow for free flow of cerebrospinal fluid thereby improving the quality of life for the patient. PAGEPress Publications, Pavia, Italy 2017-10-02 /pmc/articles/PMC5641841/ /pubmed/29071044 http://dx.doi.org/10.4081/ni.2017.7336 Text en ©Copyright E.C. Mader Jr et al., 2017 http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Lan, Zhi Gang
Richard, Seidu A.
Liu, Jiagang
You, Chao
Chiari type I malformation with cervicothoracic syringomyelia subterfuge as flail arm syndrome
title Chiari type I malformation with cervicothoracic syringomyelia subterfuge as flail arm syndrome
title_full Chiari type I malformation with cervicothoracic syringomyelia subterfuge as flail arm syndrome
title_fullStr Chiari type I malformation with cervicothoracic syringomyelia subterfuge as flail arm syndrome
title_full_unstemmed Chiari type I malformation with cervicothoracic syringomyelia subterfuge as flail arm syndrome
title_short Chiari type I malformation with cervicothoracic syringomyelia subterfuge as flail arm syndrome
title_sort chiari type i malformation with cervicothoracic syringomyelia subterfuge as flail arm syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5641841/
https://www.ncbi.nlm.nih.gov/pubmed/29071044
http://dx.doi.org/10.4081/ni.2017.7336
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