Axonal neuropathy in a patient with monoclonal IgM kappa reactive with Schmidt-Lantermann incisures

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Abstract

We report a patient with a progressive, predominantly sensory neuropathy and a IgMκ M-protein that binds to Schmidt-Lantermann incisures. A sural nerve biopsy showed primary axonal damage and IgM deposits at Schmidt-Lantermann incisures.were seen by direct immunoperoxidase. Serum from the patient injected into rat sciatic nerve reacts with the incisures as with those in the patient's nerve. The IgMκ M-protein reacts with chondroitin sulfate C and binds to a broad nerve protein band with a mobility of between 170 and 118 kDa.

Peripheral neuropathy may be related to the M-protein, which had immunocytochemical reactivity not previously described for patients with polyneuropathy and IgM monoclonal gammopathy.

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      The neuropathy was often symmetrical with a glove-stocking distribution but was occasionally asymmetrical. Morphological studies on nerve biopsy showed axonal degeneration with deposits of IgM in the endoneurium (Takatsu et al., 1985; Yee et al., 1989) and, in one patient, at Schmidt–Lanterman incisures (Quattrini et al., 1991). Occasional patients were reported to improve after therapeutic reduction of IgM levels suggesting a possible pathogenetic role for this reactivity.

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