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Bimelic symmetric Hirayama disease: Spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease
Background: The aim of the study was to evaluate the magnetic resonance imaging (MRI) findings in bilateral symmetrical Hirayama disease and find out MRI features which are probably more indicative of symmetrical Hirayama disease, thereby help in differentiating this entity from other motor neuron d...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Tehran University of Medical Sciences
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5673986/ https://www.ncbi.nlm.nih.gov/pubmed/29114369 |
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author | Boruah, Deb Kumar Sanyal, Shantiranjan Prakash, Arjun Achar, Sashidhar Dhingani, Dhabal D. Sarma, Binod |
author_facet | Boruah, Deb Kumar Sanyal, Shantiranjan Prakash, Arjun Achar, Sashidhar Dhingani, Dhabal D. Sarma, Binod |
author_sort | Boruah, Deb Kumar |
collection | PubMed |
description | Background: The aim of the study was to evaluate the magnetic resonance imaging (MRI) findings in bilateral symmetrical Hirayama disease and find out MRI features which are probably more indicative of symmetrical Hirayama disease, thereby help in differentiating this entity from other motor neuron disease (MND). Methods: This prospective as well as retrospective study was carried out from December 2010 to September 2016 in a tertiary care center of northeast India on 92 patients with Hirayama disease. Only 19 patients having bilateral symmetric upper limb involvement at the time of presentation were included in this study sample. Results: Nineteen patients, who constituted 20.6% of 92 patients of clinical and flexion MRI confirmed Hirayama disease were found to have bilateral symmetrical wasting and weakness of distal upper limb muscles at the time of presentation. Mean ± standard deviation (SD) age of onset of the disease process was 21.7 ± 3.8 years with mean ± SD duration of illness of 3.6 ± 1.3 years. MRI revealed lower cervical cord flattening in 13 (68.4%) patients which was symmetrical in 6 (31.6%) patients and asymmetrical in 7 (36.8%) patients. In the majority of these patients, T2-weighted images (T2WI) cervical cord hyperintensities were found extending from C5 to C6 vertebral level. Seven (36.8%) patients in our study showed bilateral symmetric T2WI hyperintensities in anterior horn cells (AHC). Conclusion: Bilateral symmetrical involvement of Hirayama disease is an uncommon presentation. Symmetrical cervical cord flattening, T2WI cord and/or bilateral AHC hyperintensities were the major MRI findings detected. Flexion MRI demonstrated similar findings in both bimelic amyotrophy and classical unilateral amyotrophy. However, flexion MRI produced some distinguishing features more typical for bilateral symmetrical Hirayama disease which help to differentiate it from other MNDs. |
format | Online Article Text |
id | pubmed-5673986 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Tehran University of Medical Sciences |
record_format | MEDLINE/PubMed |
spelling | pubmed-56739862017-11-07 Bimelic symmetric Hirayama disease: Spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease Boruah, Deb Kumar Sanyal, Shantiranjan Prakash, Arjun Achar, Sashidhar Dhingani, Dhabal D. Sarma, Binod Iran J Neurol Original Article Background: The aim of the study was to evaluate the magnetic resonance imaging (MRI) findings in bilateral symmetrical Hirayama disease and find out MRI features which are probably more indicative of symmetrical Hirayama disease, thereby help in differentiating this entity from other motor neuron disease (MND). Methods: This prospective as well as retrospective study was carried out from December 2010 to September 2016 in a tertiary care center of northeast India on 92 patients with Hirayama disease. Only 19 patients having bilateral symmetric upper limb involvement at the time of presentation were included in this study sample. Results: Nineteen patients, who constituted 20.6% of 92 patients of clinical and flexion MRI confirmed Hirayama disease were found to have bilateral symmetrical wasting and weakness of distal upper limb muscles at the time of presentation. Mean ± standard deviation (SD) age of onset of the disease process was 21.7 ± 3.8 years with mean ± SD duration of illness of 3.6 ± 1.3 years. MRI revealed lower cervical cord flattening in 13 (68.4%) patients which was symmetrical in 6 (31.6%) patients and asymmetrical in 7 (36.8%) patients. In the majority of these patients, T2-weighted images (T2WI) cervical cord hyperintensities were found extending from C5 to C6 vertebral level. Seven (36.8%) patients in our study showed bilateral symmetric T2WI hyperintensities in anterior horn cells (AHC). Conclusion: Bilateral symmetrical involvement of Hirayama disease is an uncommon presentation. Symmetrical cervical cord flattening, T2WI cord and/or bilateral AHC hyperintensities were the major MRI findings detected. Flexion MRI demonstrated similar findings in both bimelic amyotrophy and classical unilateral amyotrophy. However, flexion MRI produced some distinguishing features more typical for bilateral symmetrical Hirayama disease which help to differentiate it from other MNDs. Tehran University of Medical Sciences 2017-07-06 /pmc/articles/PMC5673986/ /pubmed/29114369 Text en Copyright © 2015 Iranian Neurological Association, and Tehran University of Medical Sciences This is an Open Access article distributed under the terms of the Creative Commons Attribution License, (http://creativecommons.org/licenses/by/3.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Original Article Boruah, Deb Kumar Sanyal, Shantiranjan Prakash, Arjun Achar, Sashidhar Dhingani, Dhabal D. Sarma, Binod Bimelic symmetric Hirayama disease: Spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease |
title | Bimelic symmetric Hirayama disease: Spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease |
title_full | Bimelic symmetric Hirayama disease: Spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease |
title_fullStr | Bimelic symmetric Hirayama disease: Spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease |
title_full_unstemmed | Bimelic symmetric Hirayama disease: Spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease |
title_short | Bimelic symmetric Hirayama disease: Spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease |
title_sort | bimelic symmetric hirayama disease: spectrum of magnetic resonance imaging findings and comparative evaluation with classical monomelic amyotrophy and other motor neuron disease |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5673986/ https://www.ncbi.nlm.nih.gov/pubmed/29114369 |
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