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May 2019; 6 (3) ArticleOpen Access

Myalgia with the presence of pathologic EMG correlates with perimysial inflammatory infiltrates

Kirsten Johannssen, Nicholas Schwab, Carsten P. Wessig, Karlheinz Reiners, Heinz Wiendl, Claudia Sommer
First published March 11, 2019, DOI: https://doi.org/10.1212/NXI.0000000000000549
Kirsten Johannssen
From the Department of Neurology (K.J., N.S., C.P.W., K.R., H.W., C.S.), University of Würzburg; Department of Neurology (N.S., H.W.), University of Münster, Münster, Germany; and Department of Neurology (K.J.), University of Erlangen, Germany.
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Nicholas Schwab
From the Department of Neurology (K.J., N.S., C.P.W., K.R., H.W., C.S.), University of Würzburg; Department of Neurology (N.S., H.W.), University of Münster, Münster, Germany; and Department of Neurology (K.J.), University of Erlangen, Germany.
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Carsten P. Wessig
From the Department of Neurology (K.J., N.S., C.P.W., K.R., H.W., C.S.), University of Würzburg; Department of Neurology (N.S., H.W.), University of Münster, Münster, Germany; and Department of Neurology (K.J.), University of Erlangen, Germany.
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Karlheinz Reiners
From the Department of Neurology (K.J., N.S., C.P.W., K.R., H.W., C.S.), University of Würzburg; Department of Neurology (N.S., H.W.), University of Münster, Münster, Germany; and Department of Neurology (K.J.), University of Erlangen, Germany.
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Heinz Wiendl
From the Department of Neurology (K.J., N.S., C.P.W., K.R., H.W., C.S.), University of Würzburg; Department of Neurology (N.S., H.W.), University of Münster, Münster, Germany; and Department of Neurology (K.J.), University of Erlangen, Germany.
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Claudia Sommer
From the Department of Neurology (K.J., N.S., C.P.W., K.R., H.W., C.S.), University of Würzburg; Department of Neurology (N.S., H.W.), University of Münster, Münster, Germany; and Department of Neurology (K.J.), University of Erlangen, Germany.
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Myalgia with the presence of pathologic EMG correlates with perimysial inflammatory infiltrates
Kirsten Johannssen, Nicholas Schwab, Carsten P. Wessig, Karlheinz Reiners, Heinz Wiendl, Claudia Sommer
Neurol Neuroimmunol Neuroinflamm May 2019, 6 (3) e549; DOI: 10.1212/NXI.0000000000000549

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Abstract

Objective We aimed to define normal numbers of inflammatory cells in muscle biopsies and to identify the predictive value of isolated muscle pain and increased creatine kinase regarding the diagnosis of myositis.

Methods We analyzed muscle biopsies of 71 patients using immunostains for CD3+, CD4+, CD8+, CD68+, major histocompatibility complex class I, perforin, and myeloid-related protein (MRP) 8. Patients were categorized as follows—group 1: myalgia without further clinical or laboratory abnormalities (n = 24); group 2: asymptomatic elevation of creatine kinase (hyperCKemia, n = 26); group 3: myalgia and pathologic EMG findings (n = 9); and group 4: otherwise healthy controls who had malignant hyperthermia susceptibility testing (n = 12).

Results In the normal muscle biopsy specimens from group 4, mean endomysial macrophage (CD68+) density was 21.7 ± 5.6/mm2, and perimysial density was 13.0 ± 5.6/mm2. Numbers of T-lymphocytes (CD3+) were 5 ± 3.5 endomysially and 2.2 ± 3.9/mm2 perimysially. This was not different from groups 1 and 2. Only group 3 patients had increased mean numbers of perimysial macrophages (24.1 ± 6.3/mm2; p = 0.0005), CD3+ (7.6 ± 4.9/mm2; p = 0.0056), and CD8+ T-lymphocytes (5.4 ± 3.1/mm2; p = 0.0008) and displayed the activation marker MRP8 in all cases. Although inflammatory cells were increased in the perimysium in group 3, histology did not fulfill the criteria for dermatomyositis, polymyositis, or inclusion body myositis.

Conclusions Normal muscle contains a considerable number of macrophages and T-lymphocytes. Muscle biopsy is likely to detect inflammatory changes in patients with myalgia or hyperCKemia only if pathologic EMG findings are present.

Glossary

ATPase=
adenosine triphosphatase;
H&E=
hematoxylin and eosin;
hyperCKemia=
elevation of serum creatine kinase;
MH=
malignant hyperthermia;
MHC=
major histocompatibility complex;
MRP=
myoloid-related protein

Footnotes

  • Funding information and disclosures are provided at the end of the article. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/NN.

  • The Article Processing Charge was funded by the University Hospital Würzburg.

  • Received July 2, 2017.
  • Accepted in final form January 8, 2019.
  • Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

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