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Neurol India. 2019 Nov-Dec;67(6):1532-1535. doi: 10.4103/0028-3886.273621.

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

Author information

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.

Abstract

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.

KEYWORDS:

Anti-myelin associated glycoprotein neuropathy; chronic inflammatory demyelinating polyneuropathy; distal acquired demyelinating symmetric neuropathy; nerve ultrasound; paraproteinemic neuropathy

PMID:
31857554
DOI:
10.4103/0028-3886.273621
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