ABSTRACT Introduction: Antibodies to voltage-gated potassium channels have been implicated in causing a host of peripheral and central nervous system disorders. However, the presence of these antibodies has not been previously associated with motor neuropathy. We describe the first case of acquired motor neuron disease associated with voltage-gated potas-sium channel antibodies. Case Report: The patient is an 81-year-old female who developed signs and symptoms of an idiopathic motor neuron disease. The patient was found to have increased antibodies to voltage-gated potassium chan-nels in the absence of a known metastatic or autoimmune process. Magnetic resonance imaging of the cervical spine demonstrated increased signal in the anterior horn regions of the cervical and upper thoracic spinal cord on T2-weighted imaging. The patient’s disease progression was refractory to both intravenous immunoglobulin and ster-oid therapy. Conclusion: Voltage-gated potassium channels may be causal or simply associated with motor neuron disease; this relationship needs to be elucidated. Testing for these antibodies may be warranted in cases of idiopathic rapidly progressing motor neuron disease.
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nullE. Thompson and R. gan, "Motor Neuron Disease Associated with Voltage-Gated Potassium Channel Antibodies," Neuroscience and Medicine, Vol. 2 No. 2, 2011, pp. 68-70. doi: 10.4236/nm.2011.22010.
 J. Newsom-Davis, C. Buckley, L. Clover, I. Hart, P. Maddison, E. Tuzum and A. Vincent, “Autoimmune disorders of neuronal potassium channels,” Ann N Y Acad Sci, Vol. 998, No., 2003, pp. 202-210.
 I.K. Hart, P. Maddison, J. Newsom-Davis, A. Vincent and K.R. Mills, “Phenotypic variants of autoimmune peripheral nerve hyperexcitability,” Brain, Vol. 125, No. Pt 8, 2002, pp. 1887-1895.
 K.A. Josephs, M.H. Silber, R.D. Fealey, T.B. Nippoldt, R.G. Auger and S. Vernino, “Neurophysiologic studies in Morvan syndrome,” J Clin Neurophysiol, Vol. 21, No. 6, 2004, pp. 440-445.
 T. Harrower, T. Foltynie, L. Kartsounis, R.N. De Silva and J.R. Hodges, “A case of voltage-gated potassium channel antibody-related limbic encephalitis,” Nat Clin Pract Neurol, Vol. 2, No. 6, 2006, pp. 339-343.
 H.J. Majoie, M. de Baets, W. Renier, B. Lang and A. Vincent, “Antibodies to voltage-gated potassium and calcium channels in epilepsy,” Epilepsy Res, Vol. 71, No. 2- 3, 2006, pp. 135-141.
 S.A. Rudnicki and J. Dalmau, “Paraneoplastic syndromes of the peripheral nerves,” Curr Opin Neurol, Vol. 18, No. 5, 2005, pp. 598-603.
 F. Ferracci, G. Fassetta, M.H. Butler, S. Floyd, M. Solimena and P. De Camilli, “A novel antineuronal antibody in a motor neuron syndrome associated with breast cancer,” Neurology, Vol. 53, No. 4, 1999, pp. 852-855.
 B.K. Evans, C. Fagan, T. Arnold, E.J. Dropcho and S.J. Oh, “Paraneoplastic motor neuron disease and renal cell carcinoma: improvement after nephrectomy,” Neurology, Vol. 40, No. 6, 1990, pp. 960-962.
 R.G. Weleber, R.C. Watzke, W.T. Shults, K.M. Trzupek, J.R. Heckenlively, R.A. Egan and G. Adamus, “Clinical and electrophysiologic characterization of paraneoplastic and autoimmune retinopathies associated with antienolase antibodies,” Am J Ophthalmol, Vol. 139, No. 5, 2005, pp. 780-794.
 E.J. Dunstan and J.B. Winer, “Autoimmune limbic encephalitis causing fits, rapidly progressive confusion and hyponatraemia,” Age Ageing, Vol. 35, No. 5, 2006, pp. 536-537.
 S. Karimi-Abdolrezaee, E. Eftekharpour and M.G. Fehlings, “Temporal and spatial patterns of Kv1.1 and Kv1.2 protein and gene expression in spinal cord white matter after acute and chronic spinal cord injury in rats: implications for axonal pathophysiology after neurotrauma,” Eur J Neurosci, Vol. 19, No. 3, 2004, pp. 577-589.
 R. Nashmi and M.G. Fehlings, “Mechanisms of axonal dysfunction after spinal cord injury: with an emphasis on the role of voltage-gated potassium channels,” Brain Res Brain Res Rev, Vol. 38, No. 1-2, 2001, pp. 165-191.