Skip to main content
. Author manuscript; available in PMC: 2018 Jun 1.
Published in final edited form as: Curr Opin Neurol. 2017 Jun;30(3):345–353. doi: 10.1097/WCO.0000000000000449

Table 1.

Clinical features and seizure type in autoimmune encephalitis associated with antibodies to neuronal cell-surface antigens.

LGI1
[38**,41*,48
**,49,50*]
GABAAR
[47,52*,51]
GABABR
[45,46]
NMDAR
[44,5355]
AMPAR
[56,57*]
mGluR5
[6163],§
DPPX
[59,60]
GlyR
[64,65]
CASPR2
[39*,40**]
Neurexin3α
[66*]
Median age (y), Occurs in children, Gender 61
No
M≫F
40
Yes
F=M
62
No
M>F
22
Yes
F≫M
61
No
F>M
29
Yes
F=M
58
Rare
M≫F
50
Yes
F=M
61
No
M≫F
43
No
M<F
Seizure frequencyand type (% of all cases) Any type 90%;FBDS 47%, focal 66%, generalized 63% Any type 88%; focal 81%, generalized 69% Any type 90–100%; focal 40%, generalized 93% Any type 76%; focal 17%, generalized 53% Any type 36–40%; focal 10%, generalized 30% Any type 60%;§ focal 10%, generalized 55% Any type 22% In patients with encephalitis ~13% Any type 53%; in patients with LE 90% Any type 80%
Status epilepticus Yes Yes (42%) Yes (20%) Yes Yes Yes (20%§) No data Anecdotal case reports No data No data
Other clinical manifestations LE Alteration of memory, behavior; dyskinesias LE Anti-NMDAR encephalitis LE Confusion, memory deficit, altered behavior Confusion, psychosis, hyperekplexia, tremor, myoclonus, ataxia; GI, weight loss Progressive encephalomyelitis with rigidity and myoclonus, Stiff-person syndrome, LE, Morvan syndrome, neuromyotonia Confusion, decreased level of consciousness, dyskinesias, hypoventilation
Tumor 7–11 % 38%; mostly thymoma 50–58%, mostly SCLC 20–42%, mostly ovarian teratoma 64–70%, thymoma, SCLC, breast 60%§, mostly Hodgkin’s lymphoma 10%, B-cell tumors 9%, mostly thymoma 16–19%, mostly thymoma No
Response to immunotherapy 80% 86% 60% 75–81% 70–90% 90%§ 60–78% 77% 73–93% 75%
Risk of epilepsy 15% 15% 29% Low§§ Low§§ Low§§ No data No data No data No data
Relapse 27–35% No No 12% 16–50% 10%§ 23% 11–14% 25–37% No data

F=female; GI= prodromal gastrointestinal symptoms (diarrhea, constipation); LE= limbic encephalitis; M=males; SCLC= small-cell lung cancer.

§

Spatola and Dalmau, personal communication;

§§

Low = Probably <5%.