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Ann Clin Neurophysiol > Volume 19(2); 2017 > Article
Ann Clin Neurophysiol. 2017; 19(2): 141-144.
Published online July 24, 2017.
doi: https://doi.org/10.14253/acn.2017.19.2.141
Extensive inflammatory reaction in facioscapulohumeral muscular dystrophy
Jae-Hwan Choi1, Young-Eun Park2, Jin-Hong Shin1, Chang-Hoon Lee3, and Dae-Seong Kim1
1Department of Neurology, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, Korea
2Department of Neurology, Biomedical Research Center, Pusan National University Hospital, Busan, Korea
3Department of Pathology, Biomedical Research Center, Pusan National University Hospital, Busan, Korea
Corresponding Author: Dae-Seong Kim ,Tel: +82-55-360-2450 , Fax: +82-55-360-2152 , Email: dskim@pusan.ac.kr
Received April 12, 2017   Revised: May 27, 2017    Accepted June 5, 2017
Copyright © 2017 The Korean Society of Clinical Neurophysiology
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
In facioscapulohumeral muscular dystrophy (FSHD), prominent inflammatory cellular infiltrates mimicking inflammatory myopathies are often observed in muscle biopsies. We report extensive inflammatory changes in a 16-year-old girl who was genetically confirmed as to have FSHD. Immunohistochemical staining revealed that this could be clearly distinguished from inflammatory myopathies, both in terms of cell subsets and the expression of antigenic targets. Our observations strongly suggest that the inflammatory cellular infiltrates in FSHD differ from those observed in inflammatory myopathies.
Key words: Facioscapulohumeral muscular dystrophy; Inflammation; Immunohistochemistry
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