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The Pathogenesis of Chiari Malformation and Syringomyelia: A Case Report and Systematic Review of Current Theories

We report a case of a 42-year-old female presenting with left axillary pain radiating down the arm and weakness in the ipsilateral hand. Specialist examinations of neurological and musculoskeletal systems were insignificant. Magnetic resonance imaging (MRI) of the whole spine and brain revealed cere...

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Autores principales: Sivakumaran, Priya, Ashwood, Neil, Kamal, Muhammad, Jayakumar, Nithish
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10656570/
https://www.ncbi.nlm.nih.gov/pubmed/38022024
http://dx.doi.org/10.7759/cureus.47301
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author Sivakumaran, Priya
Ashwood, Neil
Kamal, Muhammad
Jayakumar, Nithish
author_facet Sivakumaran, Priya
Ashwood, Neil
Kamal, Muhammad
Jayakumar, Nithish
author_sort Sivakumaran, Priya
collection PubMed
description We report a case of a 42-year-old female presenting with left axillary pain radiating down the arm and weakness in the ipsilateral hand. Specialist examinations of neurological and musculoskeletal systems were insignificant. Magnetic resonance imaging (MRI) of the whole spine and brain revealed cerebellar tonsillar herniation of 9-10mm indicating a Chiari type 1 malformation and a large tubular T2 high-intensity lesion in the cervical cord, extending from the C2/3-disc level down to C6/C7 as well as a similar but smaller lesion behind the bodies of C7 and T1. Both lesions were consistent with syringomyelia. Surgical intervention was deemed inappropriate, and she was treated with three months of physiotherapy. Regular follow-up for two years showed gradual symptom resolution, syrinx shrinkage, and no further complications arising secondary to Chiari type 1 malformation. Chiari malformation is an anatomical anomaly of the cranio-cervical junction. It is often incidentally found on MRI, but although asymptomatic in the population, complications associated with the condition such as syringomyelia are a common initial presentation. The relationship between Chiari malformation, particularly Chiari type 1 malformation, and syringomyelia is close with the majority of patients often presenting with idiopathic syringomyelia also found to have a Chiari type 1 malformation. Considerable discussion about the pathogenic mechanisms for syringomyelia development in Chiari malformation is recognized and advancing continually. 
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spelling pubmed-106565702023-10-19 The Pathogenesis of Chiari Malformation and Syringomyelia: A Case Report and Systematic Review of Current Theories Sivakumaran, Priya Ashwood, Neil Kamal, Muhammad Jayakumar, Nithish Cureus Neurology We report a case of a 42-year-old female presenting with left axillary pain radiating down the arm and weakness in the ipsilateral hand. Specialist examinations of neurological and musculoskeletal systems were insignificant. Magnetic resonance imaging (MRI) of the whole spine and brain revealed cerebellar tonsillar herniation of 9-10mm indicating a Chiari type 1 malformation and a large tubular T2 high-intensity lesion in the cervical cord, extending from the C2/3-disc level down to C6/C7 as well as a similar but smaller lesion behind the bodies of C7 and T1. Both lesions were consistent with syringomyelia. Surgical intervention was deemed inappropriate, and she was treated with three months of physiotherapy. Regular follow-up for two years showed gradual symptom resolution, syrinx shrinkage, and no further complications arising secondary to Chiari type 1 malformation. Chiari malformation is an anatomical anomaly of the cranio-cervical junction. It is often incidentally found on MRI, but although asymptomatic in the population, complications associated with the condition such as syringomyelia are a common initial presentation. The relationship between Chiari malformation, particularly Chiari type 1 malformation, and syringomyelia is close with the majority of patients often presenting with idiopathic syringomyelia also found to have a Chiari type 1 malformation. Considerable discussion about the pathogenic mechanisms for syringomyelia development in Chiari malformation is recognized and advancing continually.  Cureus 2023-10-19 /pmc/articles/PMC10656570/ /pubmed/38022024 http://dx.doi.org/10.7759/cureus.47301 Text en Copyright © 2023, Sivakumaran et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Neurology
Sivakumaran, Priya
Ashwood, Neil
Kamal, Muhammad
Jayakumar, Nithish
The Pathogenesis of Chiari Malformation and Syringomyelia: A Case Report and Systematic Review of Current Theories
title The Pathogenesis of Chiari Malformation and Syringomyelia: A Case Report and Systematic Review of Current Theories
title_full The Pathogenesis of Chiari Malformation and Syringomyelia: A Case Report and Systematic Review of Current Theories
title_fullStr The Pathogenesis of Chiari Malformation and Syringomyelia: A Case Report and Systematic Review of Current Theories
title_full_unstemmed The Pathogenesis of Chiari Malformation and Syringomyelia: A Case Report and Systematic Review of Current Theories
title_short The Pathogenesis of Chiari Malformation and Syringomyelia: A Case Report and Systematic Review of Current Theories
title_sort pathogenesis of chiari malformation and syringomyelia: a case report and systematic review of current theories
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10656570/
https://www.ncbi.nlm.nih.gov/pubmed/38022024
http://dx.doi.org/10.7759/cureus.47301
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