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JAMA Neurol. 2013 Apr;70(4):502-5. doi: 10.1001/jamaneurol.2013.658.

IgG4-related neuropathy: a case report.

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1
Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Abstract

IMPORTANCE:

The newly recognized entity IgG4-related disease (IgG4-RD) is characterized by an elevated IgG4 serum concentration and tissue infiltration by IgG4-positive plasma cells. We describe, for the first time, the clinical features and nerve biopsy findings of a patient with IgG4-RD who presented with neuropathy in the extremities.

OBSERVATIONS:

A 55-year-old man had histopathologically defined IgG4-RD that manifested as sensory-motor neuropathy. The neuropathic features were multiple mononeuropathies with electrophysiological findings suggestive of axonal neuropathy. Marked thickening with abundant collagen fibers and infiltration of IgG4-positive plasma cells were observed in the epineurium of the biopsied sural nerve. A moderate degree of myelinated fiber loss without evidence of segmental demyelination was present, whereas necrotizing vasculitis was not found. Oral prednisolone therapy ameliorated the neuropathic symptoms.

CONCLUSIONS AND RELEVANCE:

This case of IgG4-RD presented as sensory-motor neuropathy with pain and sclerosis of the skin in the extremities. The differential diagnosis of neuropathy should include IgG4-RD.

PMID:
23440288
DOI:
10.1001/jamaneurol.2013.658
[Indexed for MEDLINE]
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