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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References
- The prevalence of monoclonal gammopathy in peripheral neuropathy.Neurology. 1981; 31: 1480-1483
- Dangerous small B-cell clones.Blood. 2006; 108: 2520-2530
- Prevalence of monoclonal gammopathy of undetermined significance.N Engl J Med. 2006; 354: 1362-1369
- Peripheral neuropathy in monoclonal gammopathy of undetermined significance: prevalence and immunopathogenetic studies.Acta Neurol Scand. 1992; 85: 383-390
- Long-term prognosis of neuropathy associated with anti-MAG IgM M-proteins and its relationship to immune therapies.Brain. 2000; 123: 710-717
- Prognosis of neuropathy with monoclonal gammopathy.Muscle Nerve. 2000; 23: 150-152
- Paraproteinemic neuropathies.Brain Pathol. 2001; 11: 399-407
- Peripheral neuropathy in IgM monoclonal gammopathy and Waldenström's macroglobulinemia: a frequent complication in elderly males with low MAG-reactive serum monoclonal component.Am J Hematol. 1994; 45: 25-31
- Light chain deposition in peripheral nerve as a cause of mononeuritis multiplex in Waldenström's macroglobulinaemia.J Neurol Sci. 2010; 291: 89-91
- Waldenström's macroglobulinemia and peripheral neuropathy: deposition of M-component and kappa light chain in the endoneurium.Neurology. 1985; 35: 603-606
- Light chain deposition disease neuropathy resembling amyloid neuropathy in a multiple myeloma patient.Ital J Neurol Sci. 1998; 19: 229-233
- Vasculitic neuropathy in a patient with cryoglobulinemia and anti-MAG IGM monoclonal gammopathy.Muscle Nerve. 1992; 15: 891-898
- Amyloid neuropathy and tremor in Waldenström's macroglobulinaemia.Arch Neurol. 1980; 37: 240-242
- Polyneuropathy in Waldenström's macroglobulinaemia.J Neurol Sci. 1974; 21: 341-354
- Placebo-controlled trial of Rituximab in IgM anti-myelin-associated glycoprotein antibody demyelinating neuropathy.Ann Neurol. 2009; 65: 286-293
- Neuropathy with predominant small fibre involvement associated with abnormal anti-MAG titre.Intern Med. 2010; 49: 2627-2629
- Anti-myelin-associated glycoprotein neuropathy.Curr Opin Neurol. 2006; 19: 458-463
- Monoclonal gammopathy and neuropathy: myelin-associated glycoprotein reactivity and clinical characteristics.Neurology. 1986; 36: 75-88
- Anti-myelin-associated glycoprotein antibodies and endoneurial cryoglobulin deposits responsible for a severe neuropathy.Acta Neuropathol. 2001; 102: 409-412
- Intranervous immunoglobulin deposits: an underestimated mechanism of neuropathy.Muscle Nerve. 2008; 38: 904-911
- IgM MGUS anti-MAG neuropathy with predominant muscle weakness and extensive muscle atrophy.Muscle Nerve. 2010; 42: 433-435
- Ultrastructural study of the human diseased peripheral nerves.2nd ed. Elsevier, London, Amsterdam, New York1987
- Morphological progression of myelin abnormalities in IgM-monoclonal gammopathy of undetermined significance anti-myelin-associated glycoprotein neuropathy.J Neuropathol Exp Neurol. 2010; 69: 1143-1157
- Anti-MAG IgM penetration into myelinated fibers correlates with the extent of myelin widening.Muscle Nerve. 1999; 22: 1030-1037
- Lymphocytes in sural nerve biopsies from patients with plasma cell dyscrasia and polyneuropathy.J Peripher Nerv Syst. 1999; 4: 91-98
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.