Table 2.

APE and APE2 score and components [156,157].

APE Score and Components
Clinical Characteristics	Score
New-onset, rapidly progressive mental status changes that developed over 1–6 weeks or new-onset seizure activity (within 1 year of evaluation)	1
Neuropsychiatry changes; agitation, aggressiveness, emotional lability	1
Autonomic dysfunction (sustained atrial tachycardia or bradycardia, orthostatic hypotension (≥20 mm Hg fall in systolic pressure or ≥10 mm Hg fall in diastolic pressure within 3 min of standing), hyperhidrosis, persistently labile blood pressure, ventricular tachycardia, or cardiac asystole)	1
Viral prodrome (rhinorrhea, sore throat, low-grade fever) in the absence of underlying malignancy	2
Facial dyskinesias or faciobrachial dystonic movements	2
Refractory seizure	2
CSF: signs of inflammation (elevated CSF protein >50 mg/dL and/or lymphocytic pleocytosis >5 cells/dL if the total number of CSF RBC is <1000 cells/dL a	2
Brain MRI findings indicate limbic encephalitis (medial temporal T2/FLAIR signal changes) a	2
Underlying malignancy (excluding cutaneous squamous cell carcinoma and basal cell carcinoma)	2
a: No brain MRI or CSF analyses were scored zero.	Total max: 15
APE2 Score and Components
Clinical Characteristics	Score
New-onset, rapidly progressive mental status changes that developed over 1–6 weeks or new-onset seizure activity (within 1 year of evaluation)	1
Neuropsychiatry changes: agitation, aggressiveness, emotional lability	1
Autonomic dysfunction (sustained atrial tachycardia or bradycardia, orthostatic hypotension, hyperhidrosis, persistently labile blood pressure, ventricular tachycardia, cardiac asystole, or gastrointestinal dysmotility) *	1
Viral prodrome (rhinorrhea, sore throat, low-grade fever) in the absence of underlying systemic malignancy within 5 years of neurological symptom onset	2
Faciobrachial dystonic seizures	3
Facial dyskinesias in the absence of faciobrachial dystonic seizures	2
Seizure refractory to at least two antiseizure medications	2
CSF signs of inflammation # (elevated CSF protein >50 mg/dL and/or lymphocytic pleocytosis >5 cells/dL if the total number of CSF RBC is <1000 cells/dL	2
Brain MRI suggesting encephalitis (T2/FLAIR hyperintensity restricted to one or both medial temporal lobes or multifocal in gray matter, white matter, or both compatible with demyelination or inflammation)	2
Systemic cancer diagnosed within 5 years of neurological symptom onset (excluding cutaneous squamous cell carcinoma, basal cell carcinoma, brain tumor, cancer with brain metastasis)	2
* Scored only if no history of autonomic dysfunction prior to onset of suspected autoimmune syndrome and the autonomic dysfunction not attributable to medications, hypovolemia, plasmapheresis, or infection # Patients scored zero if brain MRI or CSF analysis not performed	Total max: 18

Ab, antibody, AMPA-R = amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid; CASPR-2 = contactin-associated protein 2; DPPX = dipeptidyl-peptidase-like protein 6; FLAIR = fluid-attenuated inversion recovery; GABA_AR = γ-aminobutyric acid A receptor; GABA_BR = γ-aminobutyric acid B receptor; GAD-65, glutamic acid decarboxylase 65; GFAPα = glial fibrillary acidic protein; LGI1 = leucine-rich glioma-inactivated protein 1; NMDA-R = N-methyl-d-aspartate receptor. AMPA-R = amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid; CASPR-2 = contactin-associated protein 2; DPPX = dipeptidyl-peptidase-like protein 6; FLAIR = fluid-attenuated inversion recovery; GABA_AR = γ-aminobutyric acid A receptor; GABA_BR = γ-aminobutyric acid B receptor; GFAPα = glial fibrillary acidic protein; LGI1 = leucine-rich glioma-inactivated protein 1; MOG = myelin oligodendrocyte glycoprotein; mGluR1 = metabotropic glutamate receptor 1; mGluR5 = metabotropic glutamate receptor 5; NMDA-R = N-methyl-d-aspartate receptor.