Severe long-standing dysimmune sensory neuronopathy responsive to etanercept.
Sensory neuronopathy in association with connective tissue disease is a disabling disorder for which there is no well-established therapy. Various immunosuppressive agents, plasmapheresis, and intravenous immunoglobulin have shown only anecdotal or modest beneficial effects. Tumor necrosis factor alpha is a proinflammatory cytokine that mediates TH1-cell inflammatory responses and is a plausible contributor to dorsal root ganglion injury in sensory neuronopathy. We describe a patient with severe painful and ataxic sensory neuronopathy in association with systemic lupus erythematosus, who showed marked and sustained improvement on etanercept, a tumor necrosis factor alpha inhibitor, despite a chronic and progressive course that was refractory to several immunomodulatory interventions. We review the therapeutic potential of tumor necrosis factor alpha blockade in immune-mediated neuropathies and the reported neurologic complications from its use, most notably central and peripheral demyelination.
Department of Neurology, University of Rochester, Rochester, NY 14642, USA. david_herrmann@urmc.rochester.edu
Juan J
JJ
Figueroa
Darren
D
Tabechian
David N
DN
Herrmann
D000328:Adult; D001259:Ataxia / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology / Q000473:pathology; D001264:Athetosis / Q000150:complications / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D002908:Chronic Disease; D004576:Electromyography; D000068800:Etanercept; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000276:immunology; D009431:Neural Conduction; D009475:Neurons, Afferent / Q000276:immunology / Q000473:pathology; D010292:Paresthesia / Q000188:drug therapy* / Q000209:etiology / Q000276:immunology / Q000473:pathology; D010523:Peripheral Nervous System Diseases / Q000150:complications / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D018124:Receptors, Tumor Necrosis Factor / Q000627:therapeutic use*; D011993:Recombinant Fusion Proteins / Q000627:therapeutic use; D016896:Treatment Outcome
D007074:Immunoglobulin G; D007155:Immunologic Factors; D018124:Receptors, Tumor Necrosis Factor; D011993:Recombinant Fusion Proteins; D000068800:Etanercept