Marc Dörner1,2, Mihai Ceanga3, Frank Schreiber4,5, Jan-Hendrik Stahl1, Cornelius Kronlage1, Julia Wittlinger1, Magdalena Kramer1, Sophia Willikens1, Stefanie Schreiber4,5,6, Alexander Grimm1, Natalie Winter1. 1. Center for Neurology, Tuebingen University Hospital and Hertie-Institute for Clinical Brain Research, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany. 2. Department of Forensic Psychiatry, University Hospital of Psychiatry Zurich, University of Zurich, 8008 Zurich, Switzerland. 3. Hans Berger Department of Neurology, Jena University Hospital, Friedrich Schiller University Jena, 07740 Jena, Germany. 4. Department of Neurology, Otto-von-Guericke University, 39120 Magdeburg, Germany. 5. German Center for Neurodegenerative Diseases (DZNE) within the Helmholtz Association, 39120 Magdeburg, Germany. 6. Center for Behavioural Brain Sciences (CBBS), 39120 Magdeburg, Germany.
Abstract
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.
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 syndromepatients. Thus, the aim of this study was to assess possible changes and compare findings with CIDPpatients. METHODS: We retrospectively analyzed HRUS findings in three POEMS syndrome and ten CIDPpatients by evaluating cross-sectional nerve area (CSA), echogenicity and additionally calculating ultrasound pattern scores (UPSA, UPSB, UPSC and UPSS) and homogeneity scores (HS). RESULTS:CIDPpatients 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 CIDPpatients visualized both increased and decreased echointensity, while POEMS syndrome patientspictured 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.
Authors: Alexander Grimm; Debora Vittore; Victoria Schubert; Christina Lipski; Bianka Heiling; Bernhard F Décard; Hubertus Axer Journal: Clin Neurophysiol Date: 2016-04-21 Impact factor: 3.708