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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my.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 https://doi.org/10.4103/0028-3886.273621 doi:10.4103/0028-3886.273621 |
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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. |
format |
Article |
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 |
title |
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_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_sort |
severe form of m-protein negative distal acquired demyelinating symmetric neuropathy |
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Medknow Publications |
publishDate |
2019 |
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http://eprints.um.edu.my/23797/ https://doi.org/10.4103/0028-3886.273621 |
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1662755181397278720 |
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13.211869 |