Acute limbic encephalitis and glutamic acid decarboxylase antibodies: A reality?

Blanc, F.; Ruppert, E.; Kleitz, C.; Valenti, M.P.; Cretin, B.; Humbel, R.L.; Honnorat, J.; Namer, I.J.; Hirsch, E.; Manning, L.; de Seze, J.

doi:10.1016/j.jns.2009.09.004

« Previous Next »Journal of the Neurological Sciences
Volume 287, Issue 1 , Pages 69-71, 15 December 2009

Acute limbic encephalitis and glutamic acid decarboxylase antibodies: A reality?

F. Blanc
- Services of Neuropsychology, Neurology and Epileptology, Department of Neurology, University Hospital of Strasbourg, Strasbourg, France
- LINC (Cognitive Neurosciences and Imagery Laboratory), UMR 7191, Strasbourg University and CNRS, Strasbourg, France
- CMRR from Alsace (Memory Resource and Research Centre), Department of Neurology, University Hospital of Strasbourg, Strasbourg, France
- Corresponding author. Service of Neuropsychology, Department of Neurology, University Hospital of Strasbourg, 1, place de l'Hôpital. 67091, Strasbourg, France. Tel.: +33 388116892; fax: +33 388116343.
E. Ruppert
- Services of Neuropsychology, Neurology and Epileptology, Department of Neurology, University Hospital of Strasbourg, Strasbourg, France
C. Kleitz
- Services of Neuropsychology, Neurology and Epileptology, Department of Neurology, University Hospital of Strasbourg, Strasbourg, France
- CMRR from Alsace (Memory Resource and Research Centre), Department of Neurology, University Hospital of Strasbourg, Strasbourg, France
M.P. Valenti
- Services of Neuropsychology, Neurology and Epileptology, Department of Neurology, University Hospital of Strasbourg, Strasbourg, France
B. Cretin
- Services of Neuropsychology, Neurology and Epileptology, Department of Neurology, University Hospital of Strasbourg, Strasbourg, France
- CMRR from Alsace (Memory Resource and Research Centre), Department of Neurology, University Hospital of Strasbourg, Strasbourg, France
R.L. Humbel
- Laboratory of Immuno-pathology, Esch-sur-Alzette, Luxembourg
J. Honnorat
- Department of Neurology, Neurological Hospital, Lyon, France
I.J. Namer
- LINC (Cognitive Neurosciences and Imagery Laboratory), UMR 7191, Strasbourg University and CNRS, Strasbourg, France
- Service of Nuclear Medicine, University Hospital of Strasbourg, Strasbourg, France
E. Hirsch
- Services of Neuropsychology, Neurology and Epileptology, Department of Neurology, University Hospital of Strasbourg, Strasbourg, France
- LINC (Cognitive Neurosciences and Imagery Laboratory), UMR 7191, Strasbourg University and CNRS, Strasbourg, France
L. Manning
- LINC (Cognitive Neurosciences and Imagery Laboratory), UMR 7191, Strasbourg University and CNRS, Strasbourg, France
J. de Seze
- Services of Neuropsychology, Neurology and Epileptology, Department of Neurology, University Hospital of Strasbourg, Strasbourg, France
- LINC (Cognitive Neurosciences and Imagery Laboratory), UMR 7191, Strasbourg University and CNRS, Strasbourg, France

Received 7 May 2009; received in revised form 1 September 2009; accepted 4 September 2009. published online 28 September 2009.

Abstract

Limbic encephalitis (LE) associated with glutamic acid decarboxylase antibodies (GAD-Ab) is rare. We describe a 30-year-old male with acute LE and GAD-Ab, with follow-up during 2years of cognitive status including verbal episodic memory, number of seizures recorded by high-resolution video-EEG, brain MRI, 2-[18F]-fluoro-2-deoxyglucose PET and GAD-Ab titres. Treatment with corticosteroids, IV immunoglobulins, immunosuppressors and antiepileptic drugs resulted in improved memory status, disappearance of seizures and decreased GAD-Ab titres. Review of the other cases of literature and this case is in favour of the existence of autoimmune LE associated with GAD-Ab and supports the link between memory, temporal seizures and possibly GAD-Ab titres.

Keywords: Limbic encephalitis, Autoantibodies, Glutamic acid decarboxylase, GAD65, Autoimmunity