High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome—A Comparative Study
Marc Dörner,
Mihai Ceanga,
Frank Schreiber,
Jan-Hendrik Stahl,
Cornelius Kronlage,
Julia Wittlinger,
Magdalena Kramer,
Sophia Willikens,
Stefanie Schreiber,
Alexander Grimm,
Natalie Winter
Affiliations
Marc Dörner
Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany
Mihai Ceanga
Hans Berger Department of Neurology, Jena University Hospital, Friedrich Schiller University Jena, 07740 Jena, Germany
Frank Schreiber
Department of Neurology, Otto-von-Guericke University, 39120 Magdeburg, Germany
Jan-Hendrik Stahl
Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany
Cornelius Kronlage
Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany
Julia Wittlinger
Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany
Magdalena Kramer
Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany
Sophia Willikens
Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany
Stefanie Schreiber
Department of Neurology, Otto-von-Guericke University, 39120 Magdeburg, Germany
Alexander Grimm
Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany
Natalie Winter
Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany
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.
