Article Figures & Data
Tables
Screening Tool: Antibody Prevalence in Epilepsy and Encephalopathy (APE2) score (Dubey 2018)14 +1 point each: • New-onset, rapidly progressive mental status changes (1–6 weeks) or new-onset seizures (past year)
• Neuropsychiatric symptoms
• Autonomic dysfunction
+2 points each: • Viral prodrome
• Facial dyskinesias (if no faciobrachial dystonic seizures)
• Seizures refractory to >2 antiepileptic medications
• CSF findings of inflammation
• MRI showing demyelination or inflammation (such as T2/FLAIR medial temporal hyperintensity)
• Systemic cancer within 5 years of neurological symptom onset
+3 points: Faciobrachial dystonic seizures APE2 score ≥4 suggests possible AE /autoimmune psychosis: APE2 score ≥7 suggests probable or definite AE / autoimmune psychosis: Possible AE (Graus 2016)9 Rapid progression (<3 mo) of working memory deficits, altered mental status, or psychiatric symptoms, AND at least one of: • New focal CNS findings• Unexplained seizures• CSF pleocytosis (>5 WBCs/μL)• Characteristic signs of demyelination or inflammation on brain T2 MRI AND reasonable exclusion of alternative diagnosesPossible autoimmune psychosis (Pollak 2020)15 Rapid progression (<3 mo) of current psychotic symptoms, AND at least one of: • Current/recent tumor diagnosis• Movement disorder• Adverse response to antipsychotics (e.g., NMS)• Significant cognitive dysfunction• Decreased consciousness• Unexplained seizures• Autonomic dysfunctionAlso see Graus et al 20169 for diagnostic criteria for autoantibody-negative but probable AE and other encephalitis syndromes. Probable anti-NMDAR encephalitis9 Rapid progression (<3 mo) of at least four of (or at least three if +teratoma):• Behavioral or cognitive dysfunction• Speech dysfunction• Seizures• Movement disorder• Decreased level of consciousness• Autonomic dysfunction or central hypoventilationAND EEG abnormalities AND/OR CSF pleocytosis or oligoclonal bands Definite anti-NMDAR encephalitis,9 1+ symptom groups from “Probable,” AND serum and CSF IgG anti-GluN1 antibodies, or antibodies in serum with neuronal confirmatory tests Definite autoimmune limbic encephalitis9Rapid progression (<3 mo) of working memory deficits, seizures, or psychiatric symptoms suggesting limbic involvement AND bilateral medial temporal lobe abnormalities on brain T2 MRI AND EEG temporal lobe abnormalities AND/OR CSF pleocytosis AND reasonable exclusion of alternative diagnoses Probable autoimmune psychosis15 Criteria met for “Possible,” AND at least one of: • CSF pleocytosis (>5 WBCs/μL)• Bilateral medial temporal lobe abnormalities on brain T2 MRI AND/OR at least two of: • EEG abnormalities• CSF oligoclonal bands or increased IgG index• Serum anti-neuronal antibody AND reasonable exclusion of alternative diagnoses Definite autoimmune psychosis15 Criteria met for “Probable,” AND presence in CSF of IgG class anti-neuronal antibodies
