J Clin Neurol.  2007 Jun;3(2):116-119. 10.3988/jcn.2007.3.2.116.

Polyneuropathy Associated with IgA Paraproteinemia and Amyloidosis: A Case Report and Literature Review

• Jhang Y
• Lee JJ
• Park JM
• Koo JS
• Kim BK
• Kwon O

• KMID: 1851025
• DOI: http://doi.org/10.3988/jcn.2007.3.2.116

Abstract

Paraproteinemia potentially causes peripheral neuropathy via an unknown underlying pathogenetic mechanism. We report a case of pathologically proven amyloid neuropathy with AL amyloidosis with an IgA kappa light chain, which was initially diagnosed as neuropathy associated with monoclonal gammopathy of undetermined significance. This case indicates that in cases of neuropathy with paraproteinemia, the other potential causes should be excluded by appropriate means, especially pathological evaluations.

Keyword

Amyloidosis; Paraproteinemia; Polyneuropathy

MeSH Terms

Amyloid Neuropathies
Amyloidosis*
Immunoglobulin A*
Monoclonal Gammopathy of Undetermined Significance
Paraproteinemias*
Peripheral Nervous System Diseases
Polyneuropathies*
Immunoglobulin A

Figure

• 

  Figure 1 Deposition of amorphous materials in the endoneurium was indicated in sections stained with H&E (A) that exhibited birefringence under polarized-light microscopy with Congo red staining (B, arrows).

• 

  Figure 2 Ultrastructural examination showed that both myelinated and unmyelinated fibers had nonbranching amyloid fibrils in a haphazard arrangement in the endoneurium.

Reference

1. Kyle RA, Rajkumar SV, Lust JA. Greer JP, Foerster J, Lukens JN, Rodgers GM, Paraskevas F, Glader B, editors. Monoclonal gammopathy of undetermined significance and smoldering multiple myeloma. Wintrobe's Clinical Hematology. 2004. 2:11th edn. Philadelphia: Lippincott Williams & Wilkins;2565–2581.

2. Kelly JJ, Kyle RA, O'Brien PC, Dyck PJ. Prevalence of monoclonal protein in peripheral neuropathy. Neurology. 1981. 31:1480–1483.

3. Braun PE, Frail DE, Latov N. Myelin-associated glycoprotein is the antigen for a monoclonal IgM in polyneuropathy. J Neurochem. 1982. 39:1261–1265.

4. Nobile-Orazio E, Barbieri S, Baldini L, Marmiroli P, Carpo M, Premoselli S, et al. Peripheral neuropathy in monoclonal gammopathy of undetermined significance: prevalence and immunopathogenetic studies. Acta Neurol Scand. 1992. 85:383–390.

5. Magy L, Chassande B, Maisonobe T, Bouche P, Vallat JM, Leger JM. Polyneuropathy associated with IgG/IgA monoclonal gammopathy: a clinical and electrophysiological study of 15 cases. Eur J Neurol. 2003. 10:677–685.

6. Pletz MW, Duda PW, Kappos L, Steck AJ. Immunemediated neuropathies: etiology and pathogenic relationship to aging processes. J Neuroimmunol. 2003. 137:1–11.

7. Sewell HF, Matthews JB, Gooch E, Millac P, Willox A, Stern MA, et al. Autoantibody to nerve tissue in a patient with a peripheral neuropathy and an IgG paraprotein. J Clin Pathol. 1981. 34:1163–1166.

8. Dhib-Jalbut S, Liwnicz BH. Binding of serum IgA of multiple myeloma to normal peripheral nerve. Acta Neurol Scand. 1986. 73:381–387.

9. Bleasel AF, Hawke SH, Pollard JD, McLeod JG. IgG monoclonal paraproteinaemia and peripheral neuropathy. J Neurol Neurosurg Psychiatry. 1993. 56:52–57.

10. Mehndiratta MM, Sen K, Tatke M, Bajaj BK. IgA monoclonal gammopathy of undetermined significance with peripheral neuropathy. J Neurol Sci. 2004. 221:99–104.