A 60-year-old woman presented with asymmetric distal limb weakness and muscle atrophy. Electrophysiologic study revealed demyelinating polyneuropathy, and she was diagnosed with multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy as a subtype of chronic inflammatory demyelinating polyneuropathy (CIDP) based on the established diagnostic criteria.1 Anti-neurofascin155 antibody was negative. Cervical and lumbosacral nerve roots showed asymmetric multifocal hypertrophy (figure 1), which were distinct from those in typical CIDP.2 Brain MRI revealed multifocal nodular hypertrophy of trigeminal nerves (figure 2 and video 1). These hypertrophic patterns in trigeminal nerves as well as nerve roots might be characteristics of MADSAM neuropathy.
(A and B) Coronal cervical STIR MRI; (C–E), coronal lumbar STIR MRI. STIR = short tau inversion recovery.
(A) Sagittal T2-weighted MRI; (B and C) coronal fluid-attenuated inversion recovery MRI; (D–F) axial T2-weighted MRI.
Video 1
3D reconstruction image of trigeminal nerves hypertrophy was made based on thin-slice images of brain FLAIR MRI using Synapse Vincent (Fuji film, Co, Ltd, Tokyo, Japan). Asymmetric multifocal nodular hypertrophy of trigeminal nerves can be visually recognized.Download Supplementary Video 1 via http://dx.doi.org/10.1212/NXI.0000000000000553.inline-media
Author contributions
M. Kuwahara has contributed to the acquisition, analysis, and interpretation of data and drafted the article. I. Numoto has analyzed and interpreted data. S. Kusunoki has made substantial contributions to the conception and design of the study and also revised the paper critically for important intellectual content. S. Kusunoki provided the final approval to the paper.
Study funding
No targeted finding reported.
Disclosure
M. Kuwahara received speaker honoraria from Teijin, Nihon, and Japan Blood Products Organization. I. Numoto reports no disclosures. S. Kusunoki received speaker honoraria from Teijin, Nihon, Japan Blood Products Organization, Biogen, Novartis, Dainippon Sumitomo, Kyowa Kirin, Ono Pharma, Pfizer, Alexion, and Chugai; served on the editorial board for Journal of Neuroimmunology, Neurology; served as Editor-in-Chief for Neurology and Clinical Neuroscience; received research support from Novartis, Dainippon Sumitomo, Sanofi, Japan Blood Products Organization, Ohtsuka, Kyowa Kirin, Daiichi Sankyo, Eisai, Takeda, and Nihon; received research support from Ministry of Education, Culture, Sports, Science and Technology of Japan and Japan Agency for Medical Research and Development. Disclosures available: Neurology.org/NN.
Footnotes
Go to Neurology.org/NN for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
The Article Processing Charge was funded by the authors.
- Received January 4, 2019.
- Accepted in final form January 28, 2019.
- Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
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