Table 1 Diagnostic criteria for autoimmune epilepsy (AEp) [2]

Diagnosis of AEp requires the presence of the following two clinical criteria:

 1. Acute or subacute (< 12 weeks) onset of symptoms, and

 2. Exclusion of other causes (CNS infection, trauma, toxic, tumor, metabolic, previous CNS disease)

The presence of at least one of the following supportive features would strengthen the suspicion of AEp:

 1. The presence of a well-defined clinical syndrome such as NMDAR or limbic encephalitis;

 2. CNS inflammation manifested by at least one of the following:

  a. CSF pleocytosis (defined as > 5 white cells/mm³) or presence of oligoclonal bands, elevated IgG index, or elevated neopterin (defined as > 30 nM);

  b. MRI abnormality compatible with an inflammatory or autoimmune encephalitis including increased signal in the mesio-temporal lobe (LE-like syndrome); or

  c. Inflammatory neuropathology on biopsy

 3. History of other antibody-mediated conditions (for example myasthenia gravis), organ-specific autoimmunity, or other autoimmune disorders; or

 4. Response to immunotherapy