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 CASE REPORT
Year : 2019  |  Volume : 67  |  Issue : 6  |  Page : 1532--1535

A Severe Form of M - protein Negative Distal Acquired Demyelinating Symmetric Neuropathy


1 Division of Neurology, Department of Medicine, Faculty of Medicine, University of Malaya; Department of Neurology, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia
2 Division of Neurology, Department of Medicine, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia
3 Department of Pathology, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia

Correspondence Address:
Dr. Cheng-Yin Tan
Division of Neurology, Department of Medicine, Faculty of Medicine, University of Malaya, Kuala Lumpur
Malaysia
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Source of Support: None, Conflict of Interest: None


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.






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