A severe form of M-protein negative distal acquired demyelinating symmetric neuropathy

Distal acquired demyelinating symmetric neuropathy (DADS) is a variant of chronic inflammatory demyelinating polyneuropathy (CIDP) characterized by symmetrical, distal, sensory or sensorimotor involvement. DADS with M-protein (DADS-M) is less responsive to immunotherapy compared to those without M-p...

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Main Authors: Ong, Tien Lee, Goh, Khean Jin, Shahrizaila, Nortina, Wong, Kum Thong, Tan, Cheng Yin
Format: Article
Published: Medknow Publications 2019
Subjects:
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author Ong, Tien Lee
Goh, Khean Jin
Shahrizaila, Nortina
Wong, Kum Thong
Tan, Cheng Yin
author_facet Ong, Tien Lee
Goh, Khean Jin
Shahrizaila, Nortina
Wong, Kum Thong
Tan, Cheng Yin
author_sort Ong, Tien Lee
collection UM
description Distal acquired demyelinating symmetric neuropathy (DADS) is a variant of chronic inflammatory demyelinating polyneuropathy (CIDP) characterized by symmetrical, distal, sensory or sensorimotor involvement. DADS with M-protein (DADS-M) is less responsive to immunotherapy compared to those without M-protein (DADS-I). We report a case of DADS-I with severe clinical presentation viz. early hand involvement with marked wasting, inexcitable peripheral nerves on neurophysiology and poor response to immunotherapy. Despite the unusual presentation, ancillary tests including cerebrospinal fluid analysis, nerve biopsy and nerve ultrasound were supportive of an inflammatory demyelinating polyneuropathy. This case demonstrated the heterogeneity of the disorder and expands the clinical spectrum of DADS neuropathy. © 2019 Neurology India, Neurological Society of India.
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spelling um.eprints-237972020-02-14T03:04:05Z http://eprints.um.edu.my/23797/ A severe form of M-protein negative distal acquired demyelinating symmetric neuropathy Ong, Tien Lee Goh, Khean Jin Shahrizaila, Nortina Wong, Kum Thong Tan, Cheng Yin R Medicine Distal acquired demyelinating symmetric neuropathy (DADS) is a variant of chronic inflammatory demyelinating polyneuropathy (CIDP) characterized by symmetrical, distal, sensory or sensorimotor involvement. DADS with M-protein (DADS-M) is less responsive to immunotherapy compared to those without M-protein (DADS-I). We report a case of DADS-I with severe clinical presentation viz. early hand involvement with marked wasting, inexcitable peripheral nerves on neurophysiology and poor response to immunotherapy. Despite the unusual presentation, ancillary tests including cerebrospinal fluid analysis, nerve biopsy and nerve ultrasound were supportive of an inflammatory demyelinating polyneuropathy. This case demonstrated the heterogeneity of the disorder and expands the clinical spectrum of DADS neuropathy. © 2019 Neurology India, Neurological Society of India. Medknow Publications 2019 Article PeerReviewed Ong, Tien Lee and Goh, Khean Jin and Shahrizaila, Nortina and Wong, Kum Thong and Tan, Cheng Yin (2019) A severe form of M-protein negative distal acquired demyelinating symmetric neuropathy. Neurology India, 67 (6). pp. 1532-1535. ISSN 0028-3886, DOI https://doi.org/10.4103/0028-3886.273621 <https://doi.org/10.4103/0028-3886.273621>. https://doi.org/10.4103/0028-3886.273621 doi:10.4103/0028-3886.273621
spellingShingle R Medicine
Ong, Tien Lee
Goh, Khean Jin
Shahrizaila, Nortina
Wong, Kum Thong
Tan, Cheng Yin
A severe form of M-protein negative distal acquired demyelinating symmetric neuropathy
title A severe form of M-protein negative distal acquired demyelinating symmetric neuropathy
title_full A severe form of M-protein negative distal acquired demyelinating symmetric neuropathy
title_fullStr A severe form of M-protein negative distal acquired demyelinating symmetric neuropathy
title_full_unstemmed A severe form of M-protein negative distal acquired demyelinating symmetric neuropathy
title_short A severe form of M-protein negative distal acquired demyelinating symmetric neuropathy
title_sort severe form of m protein negative distal acquired demyelinating symmetric neuropathy
topic R Medicine
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