Table 1.
LGI1 [38**,41*,48 **,49,50*] |
GABAAR [47,52*,51] |
GABABR [45,46] |
NMDAR [44,53–55] |
AMPAR [56,57*] |
mGluR5 [61–63],§ |
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%.