Limbic encephalitis associated with anti-GAD antibody and common variable immune deficiency

Cigdem I. Akman, Marc C. Patterson, Arye Rubinstein, Ronit Herzog

Research output: Contribution to journalArticle

41 Citations (Scopus)

Abstract

A variety of autoantibodies have been identified with complex neurological disorders including limbic encephalitis. The underlying trigger for the immune-mediated process and the role of autoantibodies in the pathogenesis of limbic encephalitis remain to be clarified. Here, we report a 16-year-old female who was diagnosed with acute-onset non-neoplastic limbic encephalitis. The initial treatment with pulse doses of i.v. methylprednisolone improved the neurological symptoms. During the next 12 months, progressive decline was reported in her academic functioning and seizure control. Additional diagnostic evaluation revealed no evidence of malignancy or central nervous system infection but circulating anti-GAD antibodies were present in the serum and cerebrospinal fluid. Intravenous gammaglobulin infusion was initiated and continued monthly. Intravenous and oral steroids were added to the intravenous immunoglobulin treatment because of the worsening course and seizures, despite treatment with antiepileptic medications. Screening for quantitative immunoglobulins demonstrated hypogammaglobulinaemia with low immunoglobulin M and G in addition to low immunoglobulin A levels. There was a lack of protective pneumococcal antibody titers before and after immunization. Therefore, common variable immunodeficiency was suspected despite there being no history of recurrent infections. To our knowledge, this is the first report describing a possible link between immune-mediated limbic encephalitis and immune deficiency.

Original languageEnglish (US)
Pages (from-to)563-567
Number of pages5
JournalDevelopmental Medicine and Child Neurology
Volume51
Issue number7
DOIs
StatePublished - 2009

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Limbic Encephalitis
Common Variable Immunodeficiency
Anti-Idiotypic Antibodies
Autoantibodies
Seizures
Central Nervous System Infections
Agammaglobulinemia
Intravenous Immunoglobulins
Methylprednisolone
Nervous System Diseases
Intravenous Infusions
Anticonvulsants
Immunoglobulin A
Immunoglobulin M
Cerebrospinal Fluid
Immunoglobulins
Immunization
Therapeutics
Immunoglobulin G
Steroids

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience

Cite this

Limbic encephalitis associated with anti-GAD antibody and common variable immune deficiency. / Akman, Cigdem I.; Patterson, Marc C.; Rubinstein, Arye; Herzog, Ronit.

In: Developmental Medicine and Child Neurology, Vol. 51, No. 7, 2009, p. 563-567.

Research output: Contribution to journalArticle

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