Characteristics | Value* |
Age-at–symptom onset (years), median (range) | 66 (17–87) |
Male sex | 78 (66) |
Length of follow-up (months), median (range) | 20 (0–184) |
Time to treatment (months), median (range) | 5 (0–53) |
Symptoms at presentation | |
Memory loss | 103 (87) |
Seizures (any type) | 104 (88) |
Faciobrachial dystonic seizures | 62 (53) |
Pilomotor seizures | 13 (11) |
mRS score,† median (range) | 3 (0–5) |
Accompanying malignancy‡ | 7 (6) |
Kokmen STMS scale score, median (range) | 32 (18–38) |
Immunosuppressive treatments | |
First-line acute treatment | |
Intravenous steroids alone (with or without an oral steroid taper) | 49 (42) |
IVIg only | 21 (18) |
Intravenous steroids and maintenance immunotherapy | 18 (15) |
No treatment | 12 (10) |
Other combination§ | 18 (15) |
Overall treatment at any time point | |
Intravenous steroids | 93 (79) |
IVIg | 47 (40) |
Oral steroids | 41 (35) |
Mycophenolate mofetil | 38 (32) |
Plasma exchange | 14 (12) |
Azathioprine | 12 (10) |
Rituximab | 11 (9) |
Antiseizure medication use | 109 (92) |
*Represented as n (%) unless otherwise specified.
†Available in 98 patients.
‡Within±1 year of diagnosis; prostate cancer (four patients), squamous cell carcinoma (two patients), thymoma (one patient) and colorectal cancer (one patient).
§See online supplemental etable 1.
IVIg, intravenous immunoglobulin; LGI1, leucine-rich glioma-inactivated 1; mRS, modified Rankin Scale; STMS, Short Test of Mental Status.