Successful treatment of acute autoimmune limbic encephalitis with negative VGKC and NMDAR antibodies

Anna Modoni, Marcella Masciullo, Pietro Spinelli, Camillo Marra, Tommaso Tartaglione, Francesca Andreetta, Pietro Tonali, Gabriella Silvestri

Research output: Contribution to journalArticlepeer-review

Abstract

Objective: To describe a case of acute nonherpetic limbic encephalitis (LE) with negative testing for antibodies directed against onconeuronal and cell membrane antigens, including voltage-gated potassium channels and N-methyl-D-aspartate receptor, that showed a dramatic response to immune therapy. Materials and Methods: A 30-year-old woman manifested generalized seizures, altered consciousness, and memory impairment shortly after a prodromal viral illness. Few days later the patient developed a drug-resistant epileptic status. Results: Electroencephalograph showed bitemporal slowing and paroxysmal slow wave bursts. Brain magnetic resonance imaging showed bilateral swelling in the medial temporal lobes. Cerebrospinal fluid analysis ruled out viral etiologies. A diagnostic search for cancer, including serum testing for known onconeuronal antibodies proved negative. Screening for cell membrane antigen antibodies, including voltage-gated potassium channels and N-methyl-D-aspartate receptor, was also negative. Suspecting an autoimmune etiology, we started an immunomodulatory treatment with intravenous immunoglobulin followed by a short course of oral prednisone, obtaining a full clinical recovery. Conclusions: Our report confirms previous observations of "seronegative" autoimmune LE, suggesting the presence of other, still unknown central nervous system antigens representing a target of a postinfectious, autoimmune response in these patients. Moreover, it emphasizes the importance of early recognition and treatment of acute autoimmune LE, to reduce the risk of intensive care unit-related complications and the occurrence of permanent cognitive or behavioral defects.

Original languageEnglish
Pages (from-to)63-66
Number of pages4
JournalCognitive and Behavioral Neurology
Volume22
Issue number1
DOIs
Publication statusPublished - Mar 2009

Keywords

  • Autoimmune limbic encephalitis
  • NMDAR
  • VGKC

ASJC Scopus subject areas

  • Neuropsychology and Physiological Psychology
  • Psychiatry and Mental health
  • Cognitive Neuroscience
  • Medicine(all)

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