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Hirayama disease: the importance of flexion imaging
Hirayama disease is a rare cervical myelopathy characterised by asymmetrical upper limb weakness and muscle atrophy in the forearm and hand. MRI of the cervical spine plays an essential role in diagnosis, however, the characteristic findings are often only seen when the patient is imaged with the ne...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The British Institute of Radiology.
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8803229/ https://www.ncbi.nlm.nih.gov/pubmed/35136641 http://dx.doi.org/10.1259/bjrcr.20210105 |
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author | Kusel, Kieran Warne, Richard Lakshmanan, Rahul Mason, Michael Bynevelt, Michael Shah, Snehal |
author_facet | Kusel, Kieran Warne, Richard Lakshmanan, Rahul Mason, Michael Bynevelt, Michael Shah, Snehal |
author_sort | Kusel, Kieran |
collection | PubMed |
description | Hirayama disease is a rare cervical myelopathy characterised by asymmetrical upper limb weakness and muscle atrophy in the forearm and hand. MRI of the cervical spine plays an essential role in diagnosis, however, the characteristic findings are often only seen when the patient is imaged with the neck in flexion. We present a case of a 15-year-old male who presented with left forearm and hand weakness with muscle wasting. An MRI of the cervical spine with the neck in a neutral position demonstrated atrophy of the spinal cord with intrinsic signal abnormality between C5 and C7. Further imaging with the patient’s neck in flexion demonstrated the hallmark features of Hirayama disease. There was anterior displacement of the thecal sac and spinal cord, and an enlarged, crescent-shaped dorsal epidural space which enhanced following i.v. gadolinium administration. The atrophic segment of cord contacted the posterior vertebral bodies when the neck was in full flexion. This case highlights the importance of imaging patients suspected of having this entity with the neck in full flexion in order to make a diagnosis. |
format | Online Article Text |
id | pubmed-8803229 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | The British Institute of Radiology. |
record_format | MEDLINE/PubMed |
spelling | pubmed-88032292022-02-07 Hirayama disease: the importance of flexion imaging Kusel, Kieran Warne, Richard Lakshmanan, Rahul Mason, Michael Bynevelt, Michael Shah, Snehal BJR Case Rep Case Report Hirayama disease is a rare cervical myelopathy characterised by asymmetrical upper limb weakness and muscle atrophy in the forearm and hand. MRI of the cervical spine plays an essential role in diagnosis, however, the characteristic findings are often only seen when the patient is imaged with the neck in flexion. We present a case of a 15-year-old male who presented with left forearm and hand weakness with muscle wasting. An MRI of the cervical spine with the neck in a neutral position demonstrated atrophy of the spinal cord with intrinsic signal abnormality between C5 and C7. Further imaging with the patient’s neck in flexion demonstrated the hallmark features of Hirayama disease. There was anterior displacement of the thecal sac and spinal cord, and an enlarged, crescent-shaped dorsal epidural space which enhanced following i.v. gadolinium administration. The atrophic segment of cord contacted the posterior vertebral bodies when the neck was in full flexion. This case highlights the importance of imaging patients suspected of having this entity with the neck in full flexion in order to make a diagnosis. The British Institute of Radiology. 2021-09-10 /pmc/articles/PMC8803229/ /pubmed/35136641 http://dx.doi.org/10.1259/bjrcr.20210105 Text en © 2022 The Authors. Published by the British Institute of Radiology https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Case Report Kusel, Kieran Warne, Richard Lakshmanan, Rahul Mason, Michael Bynevelt, Michael Shah, Snehal Hirayama disease: the importance of flexion imaging |
title | Hirayama disease: the importance of flexion imaging |
title_full | Hirayama disease: the importance of flexion imaging |
title_fullStr | Hirayama disease: the importance of flexion imaging |
title_full_unstemmed | Hirayama disease: the importance of flexion imaging |
title_short | Hirayama disease: the importance of flexion imaging |
title_sort | hirayama disease: the importance of flexion imaging |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8803229/ https://www.ncbi.nlm.nih.gov/pubmed/35136641 http://dx.doi.org/10.1259/bjrcr.20210105 |
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