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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MDPI AG
2021-02-01
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| Series: | Diagnostics |
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| Online Access: | https://www.mdpi.com/2075-4418/11/2/264 |
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| author | Marc Dörner Mihai Ceanga Frank Schreiber Jan-Hendrik Stahl Cornelius Kronlage Julia Wittlinger Magdalena Kramer Sophia Willikens Stefanie Schreiber Alexander Grimm Natalie Winter |
| author_facet | Marc Dörner Mihai Ceanga Frank Schreiber Jan-Hendrik Stahl Cornelius Kronlage Julia Wittlinger Magdalena Kramer Sophia Willikens Stefanie Schreiber Alexander Grimm Natalie Winter |
| author_sort | Marc Dörner |
| collection | DOAJ |
| 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. <b><i>Discussion:</i></b> 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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| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-09T05:04:23Z |
| publishDate | 2021-02-01 |
| publisher | MDPI AG |
| record_format | Article |
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| spelling | doaj.art-bebd6bd7149b443ebc23f0b536db9ca52023-12-03T12:56:57ZengMDPI AGDiagnostics2075-44182021-02-0111226410.3390/diagnostics11020264High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome—A Comparative StudyMarc Dörner0Mihai Ceanga1Frank Schreiber2Jan-Hendrik Stahl3Cornelius Kronlage4Julia Wittlinger5Magdalena Kramer6Sophia Willikens7Stefanie Schreiber8Alexander Grimm9Natalie Winter10Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyHans Berger Department of Neurology, Jena University Hospital, Friedrich Schiller University Jena, 07740 Jena, GermanyDepartment of Neurology, Otto-von-Guericke University, 39120 Magdeburg, GermanyCenter for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyCenter for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyCenter for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyCenter for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyCenter for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyDepartment of Neurology, Otto-von-Guericke University, 39120 Magdeburg, GermanyCenter for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyCenter for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, GermanyBackground: 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. <b><i>Discussion:</i></b> 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.https://www.mdpi.com/2075-4418/11/2/264peripheral nerve imaginghigh-resolution nerve ultrasoundPOEMS syndromeCIDPimmune-mediated neuropathies |
| spellingShingle | Marc Dörner Mihai Ceanga Frank Schreiber Jan-Hendrik Stahl Cornelius Kronlage Julia Wittlinger Magdalena Kramer Sophia Willikens Stefanie Schreiber Alexander Grimm Natalie Winter High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome—A Comparative Study Diagnostics peripheral nerve imaging high-resolution nerve ultrasound POEMS syndrome CIDP immune-mediated neuropathies |
| 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 | peripheral nerve imaging high-resolution nerve ultrasound POEMS syndrome CIDP immune-mediated neuropathies |
| url | https://www.mdpi.com/2075-4418/11/2/264 |
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