Abstract
Background
IgM-related neuropathy generally presents as a late-onset demyelinating polyneuropathy
with predominant sensory loss and ataxia. Sporadic cases with atypical presentation
have been described.
Patients and methods
We report clinical and pathological findings from 31 patients with IgM-related neuropathy
followed in our Institute of Neurology over a 20-year period.
Results
Typical presentation with predominant sensory ataxic neuropathy was observed in 18/31
patients. In the remaining 13/31 (42%), we observed an atypical phenotype, characterized
by multiple mononeuropathy or polyneuropathy with predominant motor impairment; one
patient had polyneuropathy with predominant small-fibre involvement. Uncommon pathological
findings consisting in inflammatory infiltrates, focal axonal loss or light chain
deposition were observed in 8 patients, all with atypical clinical phenotype. Almost
all patients with atypical phenotype improved with immunosuppressive therapy.
Conclusions
A significant proportion of patients with IgM-related neuropathy presents with atypical
clinical features. In these patients, sural nerve biopsy helps clarify heterogeneous
disease mechanisms and identify patients who might benefit from immunosuppressive
therapy.
Keywords
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Article info
Publication history
Published online: May 28, 2012
Accepted:
May 4,
2012
Received in revised form:
May 2,
2012
Received:
February 9,
2012
Identification
Copyright
© 2012 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
