Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy.
OBJECTIVE
To evaluate a trial of immunotherapy as an aid to diagnosis in suspected autoimmune epilepsy.


METHODS
We reviewed the charts of 110 patients seen at our autoimmune neurology clinic with seizures as a chief complaint. Twenty-nine patients met the following inclusion criteria: (1) autoimmune epilepsy suspected based on the presence of ≥ 1 neural autoantibody (n = 23), personal or family history or physical stigmata of autoimmunity, and frequent or medically intractable seizures; and (2) initiated a 6- to 12-week trial of IV methylprednisolone (IVMP), IV immune globulin (IVIg), or both. Patients were defined as responders if there was a 50% or greater reduction in seizure frequency.


RESULTS
Eighteen patients (62%) responded, of whom 10 (34%) became seizure-free; 52% improved with the first agent. Of those receiving a second agent after not responding to the first, 43% improved. A favorable response correlated with shorter interval between symptom onset and treatment initiation (median 9.5 vs 22 months; p = 0.048). Responders included 14/16 (87.5%) patients with antibodies to plasma membrane antigens, 2/6 (33%) patients seropositive for glutamic acid decarboxylase 65 antibodies, and 2/6 (33%) patients without detectable antibodies. Of 13 responders followed for more than 6 months after initiating long-term oral immunosuppression, response was sustained in 11 (85%).


CONCLUSIONS
These retrospective findings justify consideration of a trial of immunotherapy in patients with suspected autoimmune epilepsy.


METHODS
This study provides Class IV evidence that in patients with suspected autoimmune epilepsy, IVMP, IVIg, or both improve seizure control.
From the Departments of Neurology (M.T., J.W.B., A.M., C.S., V.A.L., E.S., G.A.W., G.D.C., C.J.K., T.D.L., E.C.W., K.C.N., S.J.P.), Laboratory Medicine and Pathology (A.M., V.A.L., A.M.L.Q., C.J.K., S.J.P.), and Immunology (V.A.L.), Mayo Clinic, College of Medicine, Rochester, MN.
M
M
Toledano
J W
JW
Britton
A
A
McKeon
C
C
Shin
V A
VA
Lennon
A M L
AM
Quek
E
E
So
G A
GA
Worrell
G D
GD
Cascino
C J
CJ
Klein
T D
TD
Lagerlund
E C
EC
Wirrell
K C
KC
Nickels
S J
SJ
Pittock
D000293:Adolescent; D000328:Adult; D000368:Aged; D001323:Autoantibodies; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004827:Epilepsy / Q000134:cerebrospinal fluid / Q000276:immunology* / Q000628:therapy*; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007167:Immunotherapy / Q000379:methods*; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use*; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000276:immunology; D018696:Neuroprotective Agents / Q000627:therapeutic use*; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D009419:Nerve Tissue Proteins; D018696:Neuroprotective Agents; D008775:Methylprednisolone