[Unexplicated neuropsychiatric disorders: Do not ignore dysimmune encephalitis. A case report of a dysimmune encephalitis with anti-leucine rich glioma inactivated 1 (LGI-1) antibodies]

Rev Med Interne. 2016 Feb;37(2):127-30. doi: 10.1016/j.revmed.2015.06.007. Epub 2015 Jul 8.
[Article in French]

Abstract

Introduction: Anti-leucine rich glioma inactivated 1 encephalitis is a common and a treatable etiology of autoimmune encephalitis. Its diagnosis is a challenge because the initial diagnostic work-up is often normal.

Case report: A 48-year-old man experienced cognitive and behavioral troubles, facio-brachial dystonic seizures and a syndrome of inappropriate antidiuretic hormone secretion. First line tests excluded infectious, neoplastic, systemic inflammatory, endrocrine or toxic etiologies. Cerebral (18)Fluoro-desoxy-glucose (FDG) position emission tomography and research of specific antibodies in cerebro-spinal fluid and serum led to diagnose an anti-leucine rich glioma inactivated 1 encephalitis. Intravenous immunoglobulins and corticosteroids were partially effective. Cyclophosphamid permitted a good recovery.

Conclusion: In the presence of acute neuropsychiatric disorders with a negative etiologic research, physician should think about dysimmune encephalitis. Facio-brachial dystonic seizures and syndrome of inappropriate antidiuretic hormone secretion are highly evocative of anti-leucine rich glioma inactivated 1 encephalitis. The diagnosis needs specific diagnostic tests (cerebral (18)FDG position emission tomography and antibodies research in cerebro-spinal fluid and in serum), after the exclusion of alternative diagnoses. Extensive and repeated diagnostic work-up for neoplasia is required. Immunosupressive therapies are effective in most cases.

Keywords: Autoimmune encephalitis; Dysimmune encephalitis; Encéphalite auto-immune; Encéphalite dysimmunitaire; Facio-brachial dystonic seizures; Leucine rich glioma inactivated 1 (LGI-1); Spasmes brachio-faciaux; Voltage gate potassium channel (VGKC).

Publication types

  • Case Reports

MeSH terms

  • Autoantibodies / immunology*
  • Encephalitis / complications
  • Encephalitis / diagnosis*
  • Encephalitis / immunology*
  • Hashimoto Disease / complications
  • Hashimoto Disease / diagnosis*
  • Hashimoto Disease / immunology*
  • Humans
  • Intracellular Signaling Peptides and Proteins
  • Male
  • Mental Disorders / etiology
  • Middle Aged
  • Proteins / immunology*

Substances

  • Autoantibodies
  • Intracellular Signaling Peptides and Proteins
  • LGI1 protein, human
  • Proteins

Supplementary concepts

  • Hashimoto's encephalitis