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Research paper
Limbic encephalitis due to GABAB and AMPA receptor antibodies: a case series
  1. M Dogan Onugoren1,
  2. D Deuretzbacher2,
  3. C A Haensch3,
  4. H J Hagedorn4,
  5. S Halve5,
  6. S Isenmann6,
  7. C Kramme1,
  8. H Lohner7,
  9. N Melzer8,
  10. R Monotti9,
  11. S Presslauer10,
  12. W R Schäbitz11,
  13. S Steffanoni9,
  14. K Stoeck12,
  15. M Strittmatter13,
  16. F Stögbauer14,
  17. E Trinka15,
  18. T J von Oertzen16,
  19. H Wiendl8,
  20. F G Woermann1,
  21. C G Bien1
  1. 1Krankenhaus Mara, Epilepsy Center Bethel, Bielefeld, Germany
  2. 2Department of Neurology, Landeskrankenhaus, Bruck/Mur, Austria
  3. 3Department of Neurology, Maria Hilf Kliniken GmbH Mönchengladbach, University of Witten/Herdecke, Mönchengladabch, Germany
  4. 4Laboratory Krone, Bad Salzuflen, Germany
  5. 5Evangelic Hospital Unna, University of Duisburg-Essen, Unna, Germany
  6. 6Department of Neurology, Helios Klinikum Wuppertal, Center for Clinical Research, and University Witten/Herdecke, Wuppertal, Germany
  7. 7Department of Neurology, RoMed Kliniken Rosenheim, Rosenheim, Germany
  8. 8Department of Neurology, University of Münster, Münster, Germany
  9. 9Department of Internal Medicine, Ospedale La Carità, Locarno, Switzerland
  10. 10Department of Neurology, Wilhelminenspital der Stadt Wien, Wien, Austria
  11. 11Department of Neurology, Bethel-EvKB, Bielefeld, Germany
  12. 12Department of Neurology, Georg-August-University, Göttingen, Germany
  13. 13Department of Neurology, Klinikum Merzig, Merzig, Germany
  14. 14Department of Neurology, Klinikum Osnabrück, Osnabrück, Germany
  15. 15Department of Neurology, Christian Doppler Medical Centre, Paracelsus Medical University, Salzburg, Austria
  16. 16Department of Neurology, Wagner-Jauregg Neuroscience Centre, Linz, Austria
  1. Correspondence to Dr Müjgan Dogan Onugoren, Krankenhaus Mara, Epilepsy Center Bethel, Maraweg 17, Bielefeld D-33617, Germany; mdogan{at}live.de

Abstract

Background Two novel antibodies (abs) directed to γ-aminobutyric acid B receptor (GABABR) and α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) in patients with limbic encephalitis (LE) were first described by the Philadelphia/Barcelona groups and confirmed by the Mayo group. We present a novel series for further clinical and paraclinical refinement.

Methods Serum and cerebrospinal fluid samples from a diagnostic laboratory were selected if found to be positive for GABABR or AMPAR abs within a broad antineuronal ab panel. Data were retrospectively compiled.

Results In 10 patients, we detected abs to GABABR. Median age was 70 years. Five of them were diagnosed with small cell lung cancer (SCLC). Intrathecal GABABR ab synthesis was found in all six patients with sufficient data available (median ab-index: 76.8). On MRI, we found bilateral mediotemporal and in two cases cortical abnormalities. EEG revealed encephalopathy, partly with epileptiform discharges. Five patients received immunotherapy, two patients tumour treatment and three both therapies. Three patients died, in five patients cognitive functions declined, one patient improved slightly and one patient fully recovered. AMPAR abs were detected in three patients with mnestic disturbances. Median age was 60.7 years. The only female patient was diagnosed with ovarian cancer. None of the patients had intrathecal ab synthesis. MRI findings showed bilateral mediotemporal abnormalities. EEG was normal in all patients. Two of the three immunologically treated patients improved, one patient stabilised on a low level.

Discussion GABABR and AMPAR abs are well associated with LE. GABABR abs lead to severe clinical, neuroradiological and EEG abnormalities with poorer outcome.

  • NEUROIMMUNOLOGY
  • EPILEPSY
  • CLINICAL NEUROLOGY
  • MRI

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