Seronegative autoimmune encephalitis

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Seronegative autoimmune encephalitis. Antibody-negative probable autoimmune encephalitis (ANPRA). Fully referenced neurology checklist.

Classification

  • Antibody-negative probable autoimmune encephalitis (ANPRA)
  • Seronegative autoimmune limbic encephalitis (SNALE)
  • Acute disseminated encephalomyelitis (ADEM)

Epidemiology

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Diagnostic criteria

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Clinical presentations

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Reasons for negative antibodies: rare antibodies

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Reasons for negative antibodies: others

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Features supporting autoimmune process

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Differential diagnosis

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Approach to management

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Poor prognostic features (RAPID score)

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Outcome

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References

  1. Najjar S, Pearlman D, Devinsky O, et al. Neuropsychiatric autoimmune encephalitis without VGKC-complex, NMDAR, and GAD autoantibodies: case report and literature review. Cogn Behav Neurol 2013; 26:36-49.
  2. Lee WJ, Lee HS, Kim DY, et al. Seronegative autoimmune encephalitis: clinical characteristics and factors associated with outcomes. Brain 2022; 145:3509-3521.
  3. Graus F, Escudero D, Oleaga L, et al. Syndrome and outcome of antibody-negative limbic encephalitis. Eur J Neurol 2018; 25:1011-1016. 
  4. Waters PJ, Irani SR. Neurexin-3α: a new antibody target in autoimmune encephalitis. Neurology 2016; 86:2222-2223.
  5. Gresa-Arribas N, Planagumà J, Petit-Pedrol M, et al. Human neurexin-3α antibodies associate with encephalitis and alter synapse development. Neurology 2016; 86:2235-2242.
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