Rapidly progressive temporal lobe epilepsy associated with hippocampal sclerosis and Glutamic Acid Decarboxylase 65 (GAD65) antibody- a case report and literature review
Abstract number :
3.353
Submission category :
18. Case Studies
Year :
2016
Submission ID :
197015
Source :
www.aesnet.org
Presentation date :
12/5/2016 12:00:00 AM
Published date :
Nov 21, 2016, 18:00 PM
Authors :
Shasha Wu, The University of Chicago; Naoum Issa, The University of Chicago; Sandra Rose, The University of Chicago; Peter Warnke, The University of Chicago; John Jacobsen, The University of Chicago; Vernon L. Towle, University of Chicago, Chicago, Illino
Rationale: Autoimmune mediated encephalitis represents an increasingly recognized cause of epilepsy. Glutamic Acid Decarboxylase 65 (GAD65) antibodies are directed against intracellular antigen and have been associated with a wide range of neurological disorders, including stiff person syndrome, cerebellar ataxia, and epilepsy (Pittock SJ, et al., 2006; Saiz A, et al., 2008; Matter MP, et al., 2010). GAD65 associated limbic encephalitis are often non-paraneoplastic origin (Mata S et al., 2008). We report a case of rapidly progressive intractable epilepsy and MRI hippocampal hyperintensity associated with GAD65 antibody. Methods: Case report and review of the literature with the term: "autoimmune epilepsy", "autoimmune encephalitis", "GAD65 encephalitis", and "paraneoplastic encephalitis". Results: A 35-year-old man with no past medical history presented with seizures that started in2012. He initially failed treatment with Phenytoin. His seizure frequency increased from once every few months to weekly despite treatment with a combination of Oxcarbazepine 900 mg bid and Clobazam 20 mg bid. Clinically, he has complex partial seizures with occasional secondary generalization. His EEG showed bitemporal interictal discharges (L:R=9:1). Eight seizures were captured in two admissions for long term video EEG monitoring. Five seizures originated from the left temporal region and three seizures could not be lateralized. The MRIs in 2012 and 2014 were unremarkable. However, the third MRI in 2015 showed right hippocampal atrophy; a follow up MRI in 2016 showed progression of atrophy with hyperintensity on FLAIR sequence. . A positron emission tomography (PET) scan showed hypermetabolic activity in the right hippocampus after the patient had a seizure prior to PET scan. Serological epilepsy autoimmune panel (Mayo clinic) revealed a high titer of GAD65 antibody. Conclusions: GAD65 antibody-associated encephalitis may present with rapidly progressive intractable temporal lobe epilepsy and hippocampal sclerosis. An autoimmune etiology should be considered in such patients so that immunotherapy can be started as early as possible. Funding: None
Case Studies