Diagnostic criteria for autoimmune encephalitis: utility and pitfalls for antibody-negative disease

Lancet Neurol. 2023 Jun;22(6):529-540. doi: 10.1016/S1474-4422(23)00083-2.

Abstract

Increased awareness of autoimmune encephalitis has led to two unintended consequences: a high frequency of misdiagnoses and the inappropriate use of diagnostic criteria for antibody-negative disease. Misdiagnoses typically occur for three reasons: first, non-adherence to reported clinical requirements for considering a disorder as possible autoimmune encephalitis; second, inadequate assessment of inflammatory changes in brain MRI and CSF; and third, absent or limited use of brain tissue assays along with use of cell-based assays that include only a narrow range of antigens. For diagnosis of possible autoimmune encephalitis and probable antibody-negative autoimmune encephalitis, clinicians should adhere to published criteria for adults and children, focusing particularly on exclusion of alternative disorders. Moreover, for diagnosis of probable antibody-negative autoimmune encephalitis, the absence of neural antibodies in CSF and serum should be well substantiated. Neural antibody testing should use tissue assays along with cell-based assays that include a broad range of antigens. Live neuronal studies in specialised centres can assist in resolving inconsistencies with respect to syndrome-antibody associations. Accurate diagnosis of probable antibody-negative autoimmune encephalitis will identify patients with similar syndromes and biomarkers, which will provide homogeneous populations for future assessments of treatment response and outcome.

Publication types

  • Review

MeSH terms

  • Adult
  • Antibodies
  • Autoantibodies
  • Autoimmune Diseases of the Nervous System* / diagnosis
  • Brain / diagnostic imaging
  • Child
  • Encephalitis* / diagnosis
  • Humans

Substances

  • Antibodies
  • Autoantibodies

Supplementary concepts

  • Hashimoto's encephalitis