title: Immunotherapy-responsive limbic encephalitis with antibodies to glutamic acid decarboxylase.
abstract: Glutamic acid decarboxylase (GAD) has been recently identified as a target of humoral autoimmunity in a small subgroup of patients with non-paraneoplastic limbic encephalitis (NPLE). We present a patient with NPLE and positive anti-GAD antibodies who showed significant improvement after long-term immunotherapy. A 48-year old female was admitted with a two-year history of anterograde amnesia and seizures. Brain MRI revealed bilateral lesions of medial temporal lobes. Screening for anti-neuronal antibodies showed high anti-GAD titers in both serum and cerebrospinal fluid (CSF) with strong evidence of intrathecal production. The patient received treatment with prednisolone and long-term plasma exchange. During a 12-month follow-up, she exhibited complete seizure remission and an improvement in memory and visuo-spatial skills. Anti-GAD antibodies may serve as a useful marker to identify a subset of NPLE patients that respond to immunoregulatory treatment.
authors.affiliation: Department of Neurology, "St. Panteleimon" General State Hospital, 3 Mantouvalou St., 18454 Nikaia, Greece. Electronic address: jmarkak2@otenet.gr.
authors.forename: Ioannis
authors.initials: I
authors.lastname: Markakis
authors.affiliation: Neuroimmunology Unit, Department of Pathophysiology, Medical School, University of Athens, 75 Mikras Asias St., 11527 Athens Greece. Electronic address: halexo@gmail.com.
authors.forename: Harry
authors.initials: H
authors.lastname: Alexopoulos
authors.affiliation: Laboratory of Experimental Neurophysiology, Department of Neurology, Medical School, University of Athens, 72-74 Vas. Sophias Ave., 115 28 Athens, Greece. Electronic address: cpoulop@med.uoa.gr.
authors.forename: Cornelia
authors.initials: C
authors.lastname: Poulopoulou
authors.affiliation: Neuroimmunology Unit, Department of Pathophysiology, Medical School, University of Athens, 75 Mikras Asias St., 11527 Athens Greece. Electronic address: akrivousof@gmail.com.
authors.forename: Sofia
authors.initials: S
authors.lastname: Akrivou
authors.affiliation: Department of Neurology, Essex Centre for Neurological Sciences, Queen's Hospital, Romford, Essex, UK. Electronic address: tpapathanasiou@gmail.com.
authors.forename: Athanasios
authors.initials: A
authors.lastname: Papathanasiou
authors.affiliation: Department of Radiology, "St. Panteleimon" General State Hospital, 3 Mantouvalou St., 18454 Nikaia, Greece. Electronic address: vaso@otenet.gr.
authors.forename: Vassiliki
authors.initials: V
authors.lastname: Katsiva
authors.affiliation: Department of Neurology, "St. Panteleimon" General State Hospital, 3 Mantouvalou St., 18454 Nikaia, Greece. Electronic address: geolyr@hotmail.com.
authors.forename: Georgios
authors.initials: G
authors.lastname: Lyrakos
authors.affiliation: Department of Neurology, "St. Panteleimon" General State Hospital, 3 Mantouvalou St., 18454 Nikaia, Greece. Electronic address: g_gkekas@hotmail.com.
authors.forename: Georgios
authors.initials: G
authors.lastname: Gekas
authors.affiliation: Neuroimmunology Unit, Department of Pathophysiology, Medical School, University of Athens, 75 Mikras Asias St., 11527 Athens Greece. Electronic address: mdalakas@med.uoa.gr.
authors.forename: Marinos C
authors.initials: MC
authors.lastname: Dalakas
mesh_terms: D000906:Antibodies / Q000097:blood* / Q000134:cerebrospinal fluid*; D001224:Aspartic Acid / Q000031:analogs & derivatives / Q000378:metabolism; D002794:Choline; D003072:Cognition Disorders / Q000209:etiology / Q000628:therapy; D003401:Creatine; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans; D007167:Immunotherapy / Q000379:methods*; D020363:Limbic Encephalitis / Q000097:blood / Q000134:cerebrospinal fluid / Q000150:complications / Q000628:therapy*; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D010468:Perceptual Disorders / Q000209:etiology / Q000628:therapy; D010951:Plasma Exchange / Q000379:methods; D011239:Prednisolone / Q000627:therapeutic use; D014796:Visual Perception / Q000187:drug effects / Q000502:physiology
chemical_list: D000906:Antibodies; D001224:Aspartic Acid; C000179:N-acetylaspartate; D011239:Prednisolone; D005968:Glutamate Decarboxylase; D003401:Creatine; D002794:Choline
keywords: Autoimmune; Glutamic acid decarboxylase; Limbic encephalopathy; Plasma exchange; Steroids; Treatment