Bilateral widespread segmental swelling on nerve sonography in multifocal acquired demyelinating sensory and motor neuropathy: Two case reports. Issue 46 (19th November 2021)

• Record Type: Journal Article
• Authors: Hsieh, Wan-Jen; Chang, Kai-Chieh; Hsueh, Hsueh-Wen; Chao, Chi-Chao; Hsieh, Sung-Tsang
• Editors: Saranathan., Maya
• Is Part Of: Medicine. Volume 100:Issue 46(2021)
• Page Start: e27900
• Publication Date: 2021-11-19
• Subjects: bilateral swelling -- case report -- multifocal acquired demyelinating sensory and motor neuropathy -- nerve ultrasound; Medicine -- Periodicals; Medicine -- Periodicals; Médecine -- Périodiques; Geneeskunde; Medicine; Periodicals; Periodicals; 610.5
• DOI: 10.1097/MD.0000000000027900
• Languages: English
• ISSNs: 0025-7974

Abstract

Abstract : Supplemental Digital Content is available in the text Abstract: 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.