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Bilateral widespread segmental swelling on nerve sonography in multifocal acquired demyelinating sensory and motor neuropathy: Two case reports

INTRODUCTION: Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) is an asymmetric immune-related neuropathy with conduction block. We report 2 MADSAM cases with detailed clinical, electrophysiological, and sonography profiles. PATIENT CONCERNS AND DIAGNOSIS: Two cases presented...

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Autores principales: Hsieh, Wan-Jen, Chang, Kai-Chieh, Hsueh, Hsueh-Wen, Chao, Chi-Chao, Hsieh, Sung-Tsang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8601274/
https://www.ncbi.nlm.nih.gov/pubmed/34797343
http://dx.doi.org/10.1097/MD.0000000000027900
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author Hsieh, Wan-Jen
Chang, Kai-Chieh
Hsueh, Hsueh-Wen
Chao, Chi-Chao
Hsieh, Sung-Tsang
author_facet Hsieh, Wan-Jen
Chang, Kai-Chieh
Hsueh, Hsueh-Wen
Chao, Chi-Chao
Hsieh, Sung-Tsang
author_sort Hsieh, Wan-Jen
collection PubMed
description INTRODUCTION: Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) is an asymmetric immune-related neuropathy with conduction block. We report 2 MADSAM cases with detailed clinical, electrophysiological, and sonography profiles. PATIENT CONCERNS AND DIAGNOSIS: Two cases presented with patchy sensorimotor impairment in both clinical and electrophysiological findings. Notably, nerve ultrasound demonstrated multifocal nerve enlargement not only at sites of conduction blockade but also at the unaffected contralateral sites. Interestingly, in our first case, focal radial nerve enlargement was observed prior to the clinical manifestations, suggesting nerve dynamic pathogenesis with variable clinical significance. INTERVENTIONS AND OUTCOMES: The first patient was initially treated with prednisolone, however, 3 months after steroid therapy, her symptoms progressed. After treatment with intravenous immunoglobulin for 3 months, the symptoms stabilized. The second patient showed improvement after 2 months of prednisolone treatment. CONCLUSION: These observations suggest a more widespread pathomechanism underlying MADSAM, and ultrasound may detect nerve lesions earlier than clinical electrophysiology studies, and is warranted for early detection and thorough documentation of nerve pathology.
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spelling pubmed-86012742021-11-20 Bilateral widespread segmental swelling on nerve sonography in multifocal acquired demyelinating sensory and motor neuropathy: Two case reports Hsieh, Wan-Jen Chang, Kai-Chieh Hsueh, Hsueh-Wen Chao, Chi-Chao Hsieh, Sung-Tsang Medicine (Baltimore) 5300 INTRODUCTION: Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) is an asymmetric immune-related neuropathy with conduction block. We report 2 MADSAM cases with detailed clinical, electrophysiological, and sonography profiles. PATIENT CONCERNS AND DIAGNOSIS: Two cases presented with patchy sensorimotor impairment in both clinical and electrophysiological findings. Notably, nerve ultrasound demonstrated multifocal nerve enlargement not only at sites of conduction blockade but also at the unaffected contralateral sites. Interestingly, in our first case, focal radial nerve enlargement was observed prior to the clinical manifestations, suggesting nerve dynamic pathogenesis with variable clinical significance. INTERVENTIONS AND OUTCOMES: The first patient was initially treated with prednisolone, however, 3 months after steroid therapy, her symptoms progressed. After treatment with intravenous immunoglobulin for 3 months, the symptoms stabilized. The second patient showed improvement after 2 months of prednisolone treatment. CONCLUSION: These observations suggest a more widespread pathomechanism underlying MADSAM, and ultrasound may detect nerve lesions earlier than clinical electrophysiology studies, and is warranted for early detection and thorough documentation of nerve pathology. Lippincott Williams & Wilkins 2021-11-19 /pmc/articles/PMC8601274/ /pubmed/34797343 http://dx.doi.org/10.1097/MD.0000000000027900 Text en Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0 (https://creativecommons.org/licenses/by/4.0/)
spellingShingle 5300
Hsieh, Wan-Jen
Chang, Kai-Chieh
Hsueh, Hsueh-Wen
Chao, Chi-Chao
Hsieh, Sung-Tsang
Bilateral widespread segmental swelling on nerve sonography in multifocal acquired demyelinating sensory and motor neuropathy: Two case reports
title Bilateral widespread segmental swelling on nerve sonography in multifocal acquired demyelinating sensory and motor neuropathy: Two case reports
title_full Bilateral widespread segmental swelling on nerve sonography in multifocal acquired demyelinating sensory and motor neuropathy: Two case reports
title_fullStr Bilateral widespread segmental swelling on nerve sonography in multifocal acquired demyelinating sensory and motor neuropathy: Two case reports
title_full_unstemmed Bilateral widespread segmental swelling on nerve sonography in multifocal acquired demyelinating sensory and motor neuropathy: Two case reports
title_short Bilateral widespread segmental swelling on nerve sonography in multifocal acquired demyelinating sensory and motor neuropathy: Two case reports
title_sort bilateral widespread segmental swelling on nerve sonography in multifocal acquired demyelinating sensory and motor neuropathy: two case reports
topic 5300
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8601274/
https://www.ncbi.nlm.nih.gov/pubmed/34797343
http://dx.doi.org/10.1097/MD.0000000000027900
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