Successful treatment of acute autoimmune limbic encephalitis with negative VGKC and NMDAR antibodies: a case report

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

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21 Citations (Scopus)

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
DOIs
Publication statusPublished - 2009

Keywords

  • NMDAR Ab
  • VGKC antibodies
  • limbic encephalitis

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