Annals of Clinical Neurophysiology

The Korean Society of Clinical Neurophysiology (대한임상신경생리학회)
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Extensive inflammatory reaction in facioscapulohumeral muscular dystrophy

  • Choi, Jae-Hwan (Department of Neurology, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital) ;
  • Park, Young-Eun (Department of Neurology, Biomedical Research Center, Pusan National University Hospital) ;
  • Shin, Jin-Hong (Department of Neurology, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital) ;
  • Lee, Chang-Hoon (Department of Pathology, Biomedical Research Center, Pusan National University Hospital) ;
  • Kim, Dae-Seong (Department of Neurology, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital)
  • Received : 2017.04.12
  • Accepted : 2017.06.05
  • Published : 2017.07.31
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Abstract

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.

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References

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