Supplementary Online Content

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1 Supplementary Online Content Quek AM, Britton JW, McKeon A, et al. Autoimmune epilepsy: clinical characteristics and response to immunotherapy. Arch Neurol. Published online March 26, doi: /archneurol etable 1. EEG features. etable 2. Summary of MRI changes in 32 patients with autoimmune epilepsy. etable 3. Comparison between patients treated with immunotherapy who achieved seizure freedom or improvement (n =22) vs patients who did not respond (n = 5). efigure. Cell binding assays confirmed specificities of VGKC complex and NMDA receptor IgGs. This supplementary material has been provided by the authors to give readers additional information about their work.

2 etable 1: EEG Features Number of EEGs per patient 2 (1-14) (IQR 1-4) Long-term video EEG 13 (41%) Abnormal 29 (91%) Interictal epileptiform discharge 20 (63%) Temporal 14 Extra-temporal 3 Both 3 Focal slowing 13 (41%) Temporal 10 Extra-temporal 1 Both 2 Generalized slowing 12 (38%) Ictal discharges 15 (47%) Temporal 8 Extra-temporal 4 Both 3

3 etable 2: Summary of MRI Changes in 32 Patients with Autoimmune Epilepsy Number of MRIs per patient 3 (1-9) (IQR 2-4) Normal or clinically non-significant MRI changes 10 (31%) Post operative changes 2 (6%) Probable Inflammatory changes 20 (63%) (detected on initial MRI, 15) (detected on follow-up MRI, 5) Medial temporal lobe only 14 (44%) Lateral temporal lobe only 1 (3%) Extratemporal only 2 (6%) Both medial temporal and extratemporal 3 (9%) Gadolinium enhancing 6/19 (32%) Restricted diffusion 5/19 (26%) Unilateral 13/20 (65%) Bilateral 7/20 (35%) MRI available for follow-up of probable 15 inflammatory changes post-immunotherapy Improvement of hyperintensities post- 5 immunotherapy Spontaneous radiographic resolution before 1 immunotherapy Evolution to changes suggestive of medial temporal 5 sclerosis No change in baseline hyperintensities 4 MRI suggestive of medial temporal sclerosis 11 (34%) Evolved from medial temporal encephalitis 7 (pre-immunotherapy, 2) (post-immunotherapy, 5) Developed after previously noted normal MRIs 2 (both pre-immunotherapy) Post-brain surgery 2

4 etable 3: Comparison between patients treated with immunotherapy who achieved seizure freedom or improvement (n =22) vs patients who did not respond (n = 5) Seizure Freedom/ Improvement (n= 22) No Seizure Response (n= 5 ) P-values Demographic information Age at presentation (median, 56 (16-74) 58 (47-79) range) (IQR 39-64) (IQR 50-70) Men (%) 10 (46%) 1 (20%) Seizure characteristics Age of onset (years) 56 (16-74) 56 (45-79) (IQR 37-63) (IQR 46-68) Duration of seizures (months) 4 ( ) 22 ( ) (IQR ) (IQR ) Associated clinical features Cognitive deficits 13 (59%) 4 (80%) Personality 7 (32%) 1 (20%) Depression 4 (18%) Anxiety 2 (9%) 1 (20%) Seizure type Simple partial and/or aura 21 (96%) 2 (40%) Complex partial 18 (82%) 4 (80%) Generalized tonic clonic 14 (64%) 1 (20%) Number of antiepileptic medications 2 19 (86%) 2 (40%) Seizure frequency At least: 1 per day 19 (86%) 5 (100%) Coexisting autoimmune 10 (46%) 2 (40%) disease History of malignancy 3 (14%) 1 (20%) CSF features Elevated protein 11 (52%) 3 (60%) Elevated leukocytes 5 (24%) Oligoclonal bands 1 (6%) 3 (60%) MRI signal abnormalities 14 (64%) 4 (80%) Presence of MTS 3 (14%) 2 (40%) Detected neural autoantibody a VGKC complex 15 (68%) GAD65 3 (14%) 2 (40%) a CRMP-5, Ma-2 and NMDAR antibodies were excluded from comparison due to small numbers.

5 efigure. Cell binding assays confirmed specificities of VGKC complex and NMDA receptor IgGs. Top row: binding to Lgi1- transfected HEK293 cells (left, 20X), but not Caspr2-transfected (middle, 20X) or non-transfected cells (right, 20X); middle row: binding to Caspr2-transfected (middle, 20X) but not Lgi1-transfected (left, 20X) or non-transfected cells (right, 20X;Euroimmun); bottom row: binding to NMDA receptor-transfected cells (left 20X;middle 40X) but not non-transfected cells (right, 20X).

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