Chronic ataxic neuropathy with cold agglutinins: atypical phenotype and response to anti-CD20 antibodies.

Abstract

A syndrome of sensory ataxia has been associated with immunoglobulin (Ig) M antibodies to NeuNAc (alpha2–8), NeuNAc (alpha2–3), and disialylated gangliosides, including GD1b, GD3, GD2, GT1a, GT1b, and GQ1b.1-3⇓⇓ In some patients, cold agglutinins have also been present.4 The acronym CANOMAD encompasses the clinical phenotype of c hronic a taxic n europathy with o phthalmoplegia, M -protein, a gglutination, and anti d isialosyl antibodies.1,4⇓ This condition is distinguished from the Miller Fisher variant of Guillain–Barre syndrome by the presence of cold agglutinins and occasionally cryoglobulins and by the chronic and progressive nature of the phenotype. A 53-year-old man developed slowly progressive unsteadiness and painful paraesthesias in his extremities during a 5-month period. His examination revealed marked gait disturbance, sensory ataxia, and weakness in the distal extremities. The remainder of his neurologic examination was normal. Lumbar puncture yielded an elevated CSF protein (85 mg%). Neurophysiologic studies revealed temporal dispersion and prolonged distal latencies that were more marked in the sensory nerves than in the motor nerves. Denervation activity was …