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Autoimmune encephalitis

BMJ 2011; 342 doi: (Published 21 April 2011) Cite this as: BMJ 2011;342:d1918
  1. Sarosh R Irani, neurology registrar1,
  2. Angela Vincent, emeritus professor of neuroimmunology12,
  3. Jonathan M Schott, clinical senior lecturer2
  1. 1Nuffield Department of Clinical Neuroscience, John Radcliffe Hospital, University of Oxford, Oxford, UK
  2. 2Dementia Research Centre, Institute of Neurology UCL, London WC1N 3BG, UK
  1. jschott{at}

Treatable syndromes with characteristic clinical features

It is essential not to miss potentially treatable causes of new onset confusion or amnesia. Once toxic and metabolic causes have been excluded, infectious encephalitis must be considered and treated. However, the results of a recent surveillance study in the United Kingdom found that only 42% of patients with encephalitis had an identifiable infectious cause.1 Even just a decade ago, the cause of encephalitis in those without an identifiable infection may well have remained obscure. There is now unequivocal evidence that specific autoantibodies directed against neuronal proteins crucial to the control of neurotransmission are responsible for a proportion (~8% in one series 1) of such cases.2 Importantly, these autoimmune encephalitides may be treatable with immunotherapy.3 4 5 6 7 8

Antibodies against two targets, the voltage gated potassium channel (VGKC) complex and the N-methyl-D-aspartic acid (NMDA) receptor, have emerged as important causes—more than 100 related or relevant articles have been published in the past two years alone. These autoimmune encephalitides have distinctive clinical features and can be diagnosed by simple serological tests. Despite almost certainly being underdiagnosed, about 400 patients with clinically relevant raised VGKC complex or NMDA antibody titres have been …

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