Article Text

PDF
Letter
Non-paraneoplastic limbic encephalitis associated with NMDAR and VGKC antibodies
  1. Hannah L Pellkofer1,2,
  2. Tania Kuempfel1,
  3. Leslie Jacobson3,
  4. Angela Vincent3,
  5. Tobias Derfuss1,4
  1. 1Institute of Clinical Neuroimmunology, Ludwig Maximilians University, Munich, Germany
  2. 2Department of Neurology, Ludwig Maximilians University, Munich, Germany
  3. 3Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK
  4. 4Department of Neurology, University of Erlangen-Nuremberg, Erlangen, Germany
  1. Correspondence to Dr Hannah Pellkofer, Institute of Clinical Neuroimmunology, Ludwig Maximilians University of Munich, Marchioninistr. 15, Munich D-81377, Germany; hannah.pellkofer{at}med.uni-muenchen.de

Statistics from Altmetric.com

Introduction

Limbic encephalitis (LE) is characterised by seizures and impairment of short-term memory as well as behavioural and psychiatric symptoms such as anxiety, depression, personality change and hallucinations. Onset of these symptoms is typically subacute over a few weeks or months but may also evolve over a few days. In many patients, limbic encephalitis is a paraneoplastic syndrome usually preceding diagnosis of the malignancy. Associated tumours are most commonly small-cell lung cancer (SCLC), breast cancer, testicular tumour, teratoma, Hodgkin lymphoma and thymoma.1 Antineuronal autoantibodies can be detected in the sera of about 60% of patients. These autoantibodies are classically directed to intracellular antigens (eg, anti-Hu, anti-Ma1/2, anti-CRMP5/CV2, anti-amphiphysin). However, LE is now being recognised frequently in the absence of malignancy and can be associated with antibodies to voltage-gated potassium channel (VGKC-ab). More recently, a new type of immunotherapy-responsive severe LE was described by Dalmau and colleagues that is associated with antibodies to the n-methyl-d-aspartate-receptor (NMDAR) and ovarian teratoma. In rare instances, these NMDAR-ab also occur in men with testicular teratoma or SCLC.2 3 For the first time, we describe a male patient with non-paraneoplastic limbic encephalitis and with serum antibodies to both NMDAR and VGKC.

Case report

This previously healthy 56-year-old man reported the first signs of disease …

View Full Text

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.