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Late Presentation of Hirayama Disease With “Snake Eye Sign”: A Case Report

Hirayama disease, also called non-progressive juvenile muscular atrophy of distal upper limbs, is a type of cervical myelopathy associated with flexion movements of the neck. It is a type of benign motor neuron disease seen typically in young males in the age group of 15 to 25. The disease has an in...

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Autores principales: Mishra, Sarvesh C, Singh, Vivek, Singh, Anil K, Sharma, Srishti, Tyagi, Isha
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8865601/
https://www.ncbi.nlm.nih.gov/pubmed/35223326
http://dx.doi.org/10.7759/cureus.21557
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author Mishra, Sarvesh C
Singh, Vivek
Singh, Anil K
Sharma, Srishti
Tyagi, Isha
author_facet Mishra, Sarvesh C
Singh, Vivek
Singh, Anil K
Sharma, Srishti
Tyagi, Isha
author_sort Mishra, Sarvesh C
collection PubMed
description Hirayama disease, also called non-progressive juvenile muscular atrophy of distal upper limbs, is a type of cervical myelopathy associated with flexion movements of the neck. It is a type of benign motor neuron disease seen typically in young males in the age group of 15 to 25. The disease has an insidious onset with a stationary stage following a progressive phase. It is also called monomelic amyotrophy with patients usually presenting with insidious onset unilateral upper limb weakness and muscle wasting. A bilateral and asymmetrical presentation can be seen very rarely. A middle-aged male patient presented with bilateral asymmetrical upper limb weakness, muscle wasting involving forearm and hand muscles. Neurological examination showed bilateral upper limb weakness and muscle wasting involving forearm and hand muscles, with a classical pattern of muscle wasting in bilateral forearm muscles called oblique amyotrophy. A clinical diagnosis of Hirayama disease was made and the patient was sent to the radiology department for Magnetic Resonance Imaging of the cervical spine in flexion and neutral positions. The imaging findings were consistent with the clinical diagnosis of Hirayama disease with the presence of an abnormal “snake eye appearance”. The electrophysiological assessment done including the electromyography and nerve conduction studies were also consistent with the clinical diagnosis. “Snake eye appearance” on MRI in patients with Hirayama disease is associated with unfavorable outcomes and represents cervical myelopathy involving the anterior horn cells.
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spelling pubmed-88656012022-02-26 Late Presentation of Hirayama Disease With “Snake Eye Sign”: A Case Report Mishra, Sarvesh C Singh, Vivek Singh, Anil K Sharma, Srishti Tyagi, Isha Cureus Neurology Hirayama disease, also called non-progressive juvenile muscular atrophy of distal upper limbs, is a type of cervical myelopathy associated with flexion movements of the neck. It is a type of benign motor neuron disease seen typically in young males in the age group of 15 to 25. The disease has an insidious onset with a stationary stage following a progressive phase. It is also called monomelic amyotrophy with patients usually presenting with insidious onset unilateral upper limb weakness and muscle wasting. A bilateral and asymmetrical presentation can be seen very rarely. A middle-aged male patient presented with bilateral asymmetrical upper limb weakness, muscle wasting involving forearm and hand muscles. Neurological examination showed bilateral upper limb weakness and muscle wasting involving forearm and hand muscles, with a classical pattern of muscle wasting in bilateral forearm muscles called oblique amyotrophy. A clinical diagnosis of Hirayama disease was made and the patient was sent to the radiology department for Magnetic Resonance Imaging of the cervical spine in flexion and neutral positions. The imaging findings were consistent with the clinical diagnosis of Hirayama disease with the presence of an abnormal “snake eye appearance”. The electrophysiological assessment done including the electromyography and nerve conduction studies were also consistent with the clinical diagnosis. “Snake eye appearance” on MRI in patients with Hirayama disease is associated with unfavorable outcomes and represents cervical myelopathy involving the anterior horn cells. Cureus 2022-01-24 /pmc/articles/PMC8865601/ /pubmed/35223326 http://dx.doi.org/10.7759/cureus.21557 Text en Copyright © 2022, Mishra 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
Mishra, Sarvesh C
Singh, Vivek
Singh, Anil K
Sharma, Srishti
Tyagi, Isha
Late Presentation of Hirayama Disease With “Snake Eye Sign”: A Case Report
title Late Presentation of Hirayama Disease With “Snake Eye Sign”: A Case Report
title_full Late Presentation of Hirayama Disease With “Snake Eye Sign”: A Case Report
title_fullStr Late Presentation of Hirayama Disease With “Snake Eye Sign”: A Case Report
title_full_unstemmed Late Presentation of Hirayama Disease With “Snake Eye Sign”: A Case Report
title_short Late Presentation of Hirayama Disease With “Snake Eye Sign”: A Case Report
title_sort late presentation of hirayama disease with “snake eye sign”: a case report
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8865601/
https://www.ncbi.nlm.nih.gov/pubmed/35223326
http://dx.doi.org/10.7759/cureus.21557
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