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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.
AuthorsIoannis Markakis, Harry Alexopoulos, Cornelia Poulopoulou, Sofia Akrivou, Athanasios Papathanasiou, Vassiliki Katsiva, Georgios Lyrakos, Georgios Gekas, Marinos C Dalakas
JournalJournal of the neurological sciences (J Neurol Sci) Vol. 343 Issue 1-2 Pg. 192-4 (Aug 15 2014) ISSN: 1878-5883 [Electronic] Netherlands
PMID24950904 (Publication Type: Case Reports, Journal Article)
CopyrightCopyright © 2014 Elsevier B.V. All rights reserved.
Chemical References
  • Antibodies
  • Aspartic Acid
  • N-acetylaspartate
  • Prednisolone
  • Glutamate Decarboxylase
  • Creatine
  • Choline
Topics
  • Antibodies (blood, cerebrospinal fluid)
  • Aspartic Acid (analogs & derivatives, metabolism)
  • Choline
  • Cognition Disorders (etiology, therapy)
  • Creatine
  • Female
  • Glutamate Decarboxylase (immunology)
  • Humans
  • Immunotherapy (methods)
  • Limbic Encephalitis (blood, cerebrospinal fluid, complications, therapy)
  • Magnetic Resonance Imaging
  • Middle Aged
  • Perceptual Disorders (etiology, therapy)
  • Plasma Exchange (methods)
  • Prednisolone (therapeutic use)
  • Visual Perception (drug effects, physiology)

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