Subgroup comparison between patients treated acutely with corticosteroids or IVIg monotherapy
| Corticosteroids (n=49)* | IVIg (n=21) | P value | |
| Baseline | |||
| Demographics | |||
| Age at symptom onset (years), median (range) | 66 (27–87) | 66 (17–79) | 0.83 |
| Male sex | 37/49 (76%) | 14/21 (67%) | 0.56 |
| Acute treatment | |||
| Months from onset, median (range) | 5 (0–53) | 7 (1–15) | 0.51 |
| Pretreatment disability | |||
| Memory loss | 43/49 (88%) | 17/21 (81%) | 0.47 |
| mRS score | 3 (0–5) | 3 (2–4) | 0.17 |
| Kokmen STMS scale score, median (range) | 33.5 (6–38) | 31 (13–37) | 0.12 |
| Seizures (any type) | 45/49 (92%) | 19/21 (91%) | >0.99 |
| FBDS | 23/49 (47%) | 15/21 (71%) | 0.07 |
| Antiseizure medications | 46/49 (94%) | 19/21 (91%) | 0.63 |
| First follow-up | |||
| mRS score | 1 (0–5) | 3 (0–3) | 0.004 |
| Improvement in ΔmRS score | 2 (0–4) | 0 (0–3) | 0.008 |
| Kokmen STMS scale score, median (range) | 36 (31–38) | 31 (10–37) | 0.01 |
| FBDS resolution | 14/23 (61%) | 1/15 (7%) | 0.002 |
| FBDS >50% resolution | 18/23 (78%) | 4/15 (27%) | 0.003 |
| Seizure (any type) resolution | 33/44 (75%)# | 4/19 (21%) | <0.001 |
| Months after first treatment, median (range) | 2 (0–18) | 1 (0–20) | 0.66 |
| Adverse effects | 23/49 (47%) | 5/21 (24%) | 0.11 |
| Subsequent therapies | 23/49 (47%) | 14/21 (67%) | 0.19 |
| Corticosteroids | 22/23 (96%) | 13/14 (93%) | >0.99 |
| IVIg | 9/23 (39%) | 2/14 (14%) | 0.15 |
| PLEX | 3/23 (13%) | 3/14 (21%) | 0.65 |
| Maintenance immunotherapy† | 14/23 (61%) | 6/14 (43%) | 0.33 |
| Long-term follow-up after >24 months | (n=23) | (n=7) | |
| mRS score | 1 (0–6) | 1 (0–3) | 0.45 |
| Improvement in ΔmRS score | .5 (0–3) | 1 (0–3) | >0.99 |
| Kokmen STMS scale score, median (range) | 37 (28–38) | 33.5 (30–36) | 0.35 |
| FBDS resolution | 8/9 (89%) | 4/4 (100%) | >0.99 |
| FBDS >50% resolution | 8/9 (89%) | 4/4 (100%) | >0.99 |
| Seizure (any type) resolution | 20/23 (87%) | 7/7 (100%) | >0.99 |
| Patients who relapsed | 5/23 (22%) | 0/7 (0%) | 0.30 |
| Patients with early recurrence | 3/23 (13%) | 1/7 (14%) | >0.99 |
| Development of hippocampal atrophy/MTS | 9/23 (39%) | 3/6 (50%) | 0.67 |
| Disease duration,‡ median (range) | 21 (2–80) | 14 (6–68) | 0.45 |
#details not available about seizure resolution in one patient
*33 patients had intravenous steroid monotherapy; 14 patients received intravenous steroids with oral taper; 2 patients received high dose oral steroids with taper but without intravenous steroids.
†Azathioprine, mycophenolate mofetil or rituximab.
‡Months from symptom onset to maximal resolution.
FBDS, faciobrachial dystonic seizure; IVIg, intravenous immunoglobulin; mRS, modified Rankin Scale; MTS, mesial temporal sclerosis; PLEX, plasma exchange; STMS, Short Test of Mental Status.