Severe Paraproteinemic Demyelinating Neuropathy With Impaired Excitability of the Distal Segments of the Peripheral Nerves

imageWe report clinical and detailed nerve conduction findings in case of polyneuropathy associated with kappa light chains monoclonal gammopathy of undetermined significance with progression to lymphoproliferative disorder. A 55-year-old man had a predominantly distal, chronic (5 years duration), slowly progressive, symmetric, predominantly sensory impairment with sensory ataxia, and mild weakness. M protein was identified by serum protein electrophoresis. The kappa/lambda ratio of free light chains was significantly elevated to 11.96. The cerebrospinal fluid protein level was elevated at 3.5 g/L. This case study has revealed 2 unusual electrophysiological phenomena—a very unusual prolongation of distal motor latencies of compound muscle action potentials (CMAP) up to 86.5 ms and impaired excitability of the distal segments of the peripheral nerves. The distal CMAP areas were considerably lower compared with the proximal CMAP areas. Radiography of the skull revealed osteolytic lesions.


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