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High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome—A Comparative Study

Background: High-resolution nerve ultrasound (HRUS) has been proven to be a valuable tool in the diagnosis of immune-mediated neuropathies, such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes)...

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Autores principales: Dörner, Marc, Ceanga, Mihai, Schreiber, Frank, Stahl, Jan-Hendrik, Kronlage, Cornelius, Wittlinger, Julia, Kramer, Magdalena, Willikens, Sophia, Schreiber, Stefanie, Grimm, Alexander, Winter, Natalie
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7915164/
https://www.ncbi.nlm.nih.gov/pubmed/33572067
http://dx.doi.org/10.3390/diagnostics11020264
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author Dörner, Marc
Ceanga, Mihai
Schreiber, Frank
Stahl, Jan-Hendrik
Kronlage, Cornelius
Wittlinger, Julia
Kramer, Magdalena
Willikens, Sophia
Schreiber, Stefanie
Grimm, Alexander
Winter, Natalie
author_facet Dörner, Marc
Ceanga, Mihai
Schreiber, Frank
Stahl, Jan-Hendrik
Kronlage, Cornelius
Wittlinger, Julia
Kramer, Magdalena
Willikens, Sophia
Schreiber, Stefanie
Grimm, Alexander
Winter, Natalie
author_sort Dörner, Marc
collection PubMed
description Background: High-resolution nerve ultrasound (HRUS) has been proven to be a valuable tool in the diagnosis of immune-mediated neuropathies, such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is an important differential diagnosis of CIDP. Until now, there have been no studies that could identify specific HRUS abnormalities in POEMS syndrome patients. Thus, the aim of this study was to assess possible changes and compare findings with CIDP patients. Methods: We retrospectively analyzed HRUS findings in three POEMS syndrome and ten CIDP patients by evaluating cross-sectional nerve area (CSA), echogenicity and additionally calculating ultrasound pattern scores (UPSA, UPSB, UPSC and UPSS) and homogeneity scores (HS). Results: CIDP patients showed greater CSA enlargement and higher UPSS (median 14 vs. 11), UPSA (median 11.5 vs. 8) and HS (median 5 vs. 3) compared with POEMS syndrome patients. However, every POEMS syndrome patient illustrated enlarged nerves exceeding reference values, which were not restricted to entrapment sites. In CIDP and POEMS syndrome, heterogeneous enlargement patterns could be identified, such as inhomogeneous, homogeneous and regional nerve enlargement. HRUS in CIDP patients visualized both increased and decreased echointensity, while POEMS syndrome patients pictured hypoechoic nerves with hyperechoic intraneural connective tissue. Discussion: This is the first study to demonstrate HRUS abnormalities in POEMS syndrome outside of common entrapment sites. Although nerve enlargement was more prominent in CIDP, POEMS syndrome patients revealed distinct echogenicity patterns, which might aid in its differentiation from CIDP. Future studies should consider HRUS and its possible role in determining diagnosis, prognosis and treatment response in POEMS syndrome.
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spelling pubmed-79151642021-03-01 High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome—A Comparative Study Dörner, Marc Ceanga, Mihai Schreiber, Frank Stahl, Jan-Hendrik Kronlage, Cornelius Wittlinger, Julia Kramer, Magdalena Willikens, Sophia Schreiber, Stefanie Grimm, Alexander Winter, Natalie Diagnostics (Basel) Article Background: High-resolution nerve ultrasound (HRUS) has been proven to be a valuable tool in the diagnosis of immune-mediated neuropathies, such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is an important differential diagnosis of CIDP. Until now, there have been no studies that could identify specific HRUS abnormalities in POEMS syndrome patients. Thus, the aim of this study was to assess possible changes and compare findings with CIDP patients. Methods: We retrospectively analyzed HRUS findings in three POEMS syndrome and ten CIDP patients by evaluating cross-sectional nerve area (CSA), echogenicity and additionally calculating ultrasound pattern scores (UPSA, UPSB, UPSC and UPSS) and homogeneity scores (HS). Results: CIDP patients showed greater CSA enlargement and higher UPSS (median 14 vs. 11), UPSA (median 11.5 vs. 8) and HS (median 5 vs. 3) compared with POEMS syndrome patients. However, every POEMS syndrome patient illustrated enlarged nerves exceeding reference values, which were not restricted to entrapment sites. In CIDP and POEMS syndrome, heterogeneous enlargement patterns could be identified, such as inhomogeneous, homogeneous and regional nerve enlargement. HRUS in CIDP patients visualized both increased and decreased echointensity, while POEMS syndrome patients pictured hypoechoic nerves with hyperechoic intraneural connective tissue. Discussion: This is the first study to demonstrate HRUS abnormalities in POEMS syndrome outside of common entrapment sites. Although nerve enlargement was more prominent in CIDP, POEMS syndrome patients revealed distinct echogenicity patterns, which might aid in its differentiation from CIDP. Future studies should consider HRUS and its possible role in determining diagnosis, prognosis and treatment response in POEMS syndrome. MDPI 2021-02-09 /pmc/articles/PMC7915164/ /pubmed/33572067 http://dx.doi.org/10.3390/diagnostics11020264 Text en © 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Dörner, Marc
Ceanga, Mihai
Schreiber, Frank
Stahl, Jan-Hendrik
Kronlage, Cornelius
Wittlinger, Julia
Kramer, Magdalena
Willikens, Sophia
Schreiber, Stefanie
Grimm, Alexander
Winter, Natalie
High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome—A Comparative Study
title High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome—A Comparative Study
title_full High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome—A Comparative Study
title_fullStr High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome—A Comparative Study
title_full_unstemmed High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome—A Comparative Study
title_short High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome—A Comparative Study
title_sort high-resolution nerve ultrasound abnormalities in poems syndrome—a comparative study
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7915164/
https://www.ncbi.nlm.nih.gov/pubmed/33572067
http://dx.doi.org/10.3390/diagnostics11020264
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