Abstract
Background A 56-year-old man presented to hospital with a 6-month history of recurrent episodes of altered behavior and 'odd' episodes. He had become apathetic and uninterested in his family. He had no relevant past medical or family history. General and physical neurological examinations were unremarkable, as was bedside cognitive testing.
Investigations Brain MRI scan, 24-h electroencephalogram, serum and cerebrospinal fluid testing for voltage-gated potassium channel antibodies, blood screening for tumors, CT scans of the chest, abdomen and pelvis, whole-body PET scan, neuropsychological examination, brain 18F-fluorodeoxyglucose-PET scan.
Diagnosis Voltage-gated potassium channel antibody-related limbic encephalitis.
Management Antiepileptic drugs, immunomodulatory therapy, oral steroids, plasma exchange.
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References
Vincent A et al. (2004) Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain 127: 701–712
Gultekin SH et al. (2000) Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain 123: 1481–1494
Voltz R (2002) Paraneoplastic neurological syndromes: an update on diagnosis, pathogenesis, and therapy. Lancet Neurol 1: 294–305
Lennon VA (1994) The case for a descriptive generic nomenclature: clarification of immunostaining criteria for PCA-1, ANNA-1, and ANNA-2 autoantibodies. Neurology 44: 2412–2415
Lawn ND et al. (2003) Clinical, magnetic resonance imaging, and electroencephalographic findings in paraneoplastic limbic encephalitis. Mayo Clin Proc 78: 1363–1368
Buckley C et al. (2001) Potassium channel antibodies in two patients with reversible limbic encephalitis. Ann Neurol 50: 73–78
Buckley C and Vincent A (2005) Autoimmune channelopathies. Nat Clin Pract Neurol 1: 22–33
Schott JM et al. (2003) Amnesia, cerebral atrophy, and autoimmunity. Lancet 361: 1266
Newsom-Davis J et al. (2003) Autoimmune disorders of neuronal potassium channels. Ann NY Acad Sci 998: 202–210
Thieben MJ et al. (2004) Potentially reversible autoimmune limbic encephalitis with neuronal potassium channel antibody. Neurology 62: 1177–1182
Iranzo A et al. (2006) Rapid eye movement sleep behavior disorder and potassium channel antibody-associated limbic encephalitis. Ann Neurol 59: 178–181
Fauser S et al. (2005) FDG-PET and MRI in potassium channel antibody-associated non-paraneoplastic limbic encephalitis: correlation with clinical course and neuropsychology. Acta Neurol Scand 111: 338–343
Parthasarathi UD et al. Psychiatric presentation of voltage-gated potassium channel antibody associated encephalopathy: case report. Br J Psychiatry, in press
Scheid R et al. (2003) Neuropsychiatric findings in anti-Ma2-positive paraneoplastic limbic encephalitis. Neurology 61: 1159–1161
McArdle JP and Millingen KS (1988) Limbic encephalitis associated with malignant thymoma. Pathology 20: 292–295
Pozo-Rosich P et al. (2003) Voltage-gated potassium channel antibodies in limbic encephalitis. Ann Neurol 54: 530–533
Ances BM et al. (2005) Treatment-responsive limbic encephalitis identified by neuropil antibodies: MRI and PET correlates. Brain 128: 1764–1777
Acknowledgements
The patients and their families are thanked for their full co-operation at all stages of the assessment and treatment. The primary patient described is also thanked for allowing publication of the supplementary online material, for which his written consent was obtained. The radiological expertise of Drs Chawda, Antoun and Balaam is greatly appreciated. Professor Angela Vincent (Department of Clinical Neurology, University of Oxford, UK) is acknowledged for determining the VGKC-Ab titers in the serum and cerebrospinal fluid.
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Supplementary Video
Video clip of patient experiencing focal seizure (WMV 14412 kb)
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Harrower, T., Foltynie, T., Kartsounis, L. et al. A case of voltage-gated potassium channel antibody-related limbic encephalitis. Nat Rev Neurol 2, 339–343 (2006). https://doi.org/10.1038/ncpneuro0194
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DOI: https://doi.org/10.1038/ncpneuro0194