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IgM paraproteinemic neuropathy is a progressive and potentially disabling disorder. Treatment with alkylating agents, plasma exchange, corticosteroids, and IV immunoglobulin (IVIG) is frequently unsuccessful.1 Recently, improvement was observed with the purine analog fludarabine.2 We report a patient with IgM monoclonal gammopathy of undetermined significance neuropathy who had a prolonged remission after a short course of cladribine (2-chlorodeoxyadenosine; 2-CDA), a novel purine analog.
Case report.
A 53-year-old man presented with ascending tingling and numbness of his legs and later of his hands over a 14-month period. His legs felt unsteady, and climbing stairs and arising from a chair became difficult. He was clumsy with his fingers. On examination he had predominantly distal weakness in all limbs and also proximal leg weakness. Reflexes were symmetrically depressed or absent. Plantar responses were flexor. Joint position and vibration sensations in the feet were lost early.
Neurophysiology suggested chronic demyelinating sensorimotor neuropathy. Investigations for neuropathy revealed only an IgM level of 2.67g/L (normal 0.5 to 2.2 g/L) but no other abnormality on protein electrophoresis.
He gradually became …
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