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Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case

BACKGROUND: Hirayama disease (HD), also known as juvenile spinal muscular atrophy, is a rare condition in which flexion of the cervical neck causes compression and ischemic changes to the anterior horns of the spinal cord. Here the authors presented the first reported case of HD in North America tha...

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Detalles Bibliográficos
Autores principales: Singh, Rohin, Hudson, Miles, Meyer, Jenna H., Neal, Matthew T., Patel, Naresh P.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Association of Neurological Surgeons 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9379630/
https://www.ncbi.nlm.nih.gov/pubmed/36130535
http://dx.doi.org/10.3171/CASE21697
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author Singh, Rohin
Hudson, Miles
Meyer, Jenna H.
Neal, Matthew T.
Patel, Naresh P.
author_facet Singh, Rohin
Hudson, Miles
Meyer, Jenna H.
Neal, Matthew T.
Patel, Naresh P.
author_sort Singh, Rohin
collection PubMed
description BACKGROUND: Hirayama disease (HD), also known as juvenile spinal muscular atrophy, is a rare condition in which flexion of the cervical neck causes compression and ischemic changes to the anterior horns of the spinal cord. Here the authors presented the first reported case of HD in North America that was successfully treated via surgical intervention. OBSERVATIONS: The patient was a 15-year boy with insidious onset upper limb weakness and atrophy. His findings were a classic presentation of HD although his complex history and relative rarity of the disease caused him to remain undiagnosed for months. After conservative management via cervical collar failed, the patient was successfully treated via C5-C7 anterior cervical discectomy and fusion. The patient’s symptoms stabilized by the 3-month follow-up. LESSONS: The diagnosis of HD is easy to miss because of the lack of reporting and widespread knowledge of this condition in North America. Thus, when presented with a case of insidious onset limb weakness in a juvenile patient, HD should be placed on the differential list and verified with cervical flexion magnetic resonance imaging. Additionally, surgical intervention should be considered a safe and effective option for HD when conservative methods have failed.
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spelling pubmed-93796302022-10-04 Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case Singh, Rohin Hudson, Miles Meyer, Jenna H. Neal, Matthew T. Patel, Naresh P. J Neurosurg Case Lessons Case Lesson BACKGROUND: Hirayama disease (HD), also known as juvenile spinal muscular atrophy, is a rare condition in which flexion of the cervical neck causes compression and ischemic changes to the anterior horns of the spinal cord. Here the authors presented the first reported case of HD in North America that was successfully treated via surgical intervention. OBSERVATIONS: The patient was a 15-year boy with insidious onset upper limb weakness and atrophy. His findings were a classic presentation of HD although his complex history and relative rarity of the disease caused him to remain undiagnosed for months. After conservative management via cervical collar failed, the patient was successfully treated via C5-C7 anterior cervical discectomy and fusion. The patient’s symptoms stabilized by the 3-month follow-up. LESSONS: The diagnosis of HD is easy to miss because of the lack of reporting and widespread knowledge of this condition in North America. Thus, when presented with a case of insidious onset limb weakness in a juvenile patient, HD should be placed on the differential list and verified with cervical flexion magnetic resonance imaging. Additionally, surgical intervention should be considered a safe and effective option for HD when conservative methods have failed. American Association of Neurological Surgeons 2022-03-07 /pmc/articles/PMC9379630/ /pubmed/36130535 http://dx.doi.org/10.3171/CASE21697 Text en © 2022 The authors https://creativecommons.org/licenses/by-nc-nd/4.0/CC BY-NC-ND 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) ).
spellingShingle Case Lesson
Singh, Rohin
Hudson, Miles
Meyer, Jenna H.
Neal, Matthew T.
Patel, Naresh P.
Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case
title Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case
title_full Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case
title_fullStr Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case
title_full_unstemmed Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case
title_short Surgical treatment of spinal cord compression due to Hirayama disease: illustrative case
title_sort surgical treatment of spinal cord compression due to hirayama disease: illustrative case
topic Case Lesson
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9379630/
https://www.ncbi.nlm.nih.gov/pubmed/36130535
http://dx.doi.org/10.3171/CASE21697
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