Antikörper |
Patienten |
Alter |
Männer / Frauen |
Klinik |
Syndrom |
MR |
Tumoren |
IgG-Klasse |
Antikörper-Wirkung |
NMDAR |
(>1500) |
21 yr (2 mo–85 yr) |
1:4 |
Children: seizures, dyskinesias;
adults: behavioral changes, psychiatric symptoms |
NMDAR encephalitis |
Normal findings (70%) or nonspecific changes |
Varies with age and sex;
ovarian teratoma in women 18–45 yr old (58%) |
IgG1 |
Internalization of NMDAR,
disruption of NMDAR interaction with ephrin-B2 receptor |
AMPAR |
(80) |
56 yr (23–81) |
1:2.3 |
Confusion, memory loss; in rare cases, psychiatric symptoms |
Limbic encephalitis |
Increased signal in medial temporal lobes (67%)
|
SCLC, thymoma, or breast cancer (56%) |
IgG1 |
Internalization of AMPARs |
GABABR |
(80) |
61 yr (16–77) |
1.5:1 |
Seizures, memory loss,confusion |
Limbic encephalitis, prominent seizures |
Increased signal in medial temporal lobes (45%) |
SCLC (50%) |
IgG1 |
Blocking of agonist effect of baclofen on GABABR |
LGI1 |
(400) |
64 yr (31–84) |
2:1 |
Memory loss, faciobrachial dystonic seizures, hyponatremia |
Limbic encephalitis |
Increased signal in medial temporal lobes (83%) |
Thymoma (<5%) |
IgG4 |
Inhibition of LGI1 interaction with ADAM22 and ADAM23; decrease in postsynaptic AMPAR |
CASPR2 |
(120) |
66 yr (25–77) |
9:1 |
Memory loss, insomnia, dysautonomia, ataxia, peripheral-nerve hyperexcitability, neuropathic pain |
Limbic encephalitis |
Increased signal in medial temporal lobes (67%) |
Varies with the syndrome (<5% overall) |
IgG4 |
Alteration of gephyrin clusters in inhibitory synapses |
mGluR5 |
(11) |
29 yr (6–75) |
1.5:1 |
Confusion, psychiatric symptoms |
Encephalitis |
Normal findings in 5 of 11 patients |
Hodgkin’s lymphoma in 6 of 11 patients |
IgG1 |
Decrease in density of surface mGluR5 |
D2R |
(25) |
6 yr (2–15) |
1:1 |
Parkinsonism, dystonia, psychiatric symptoms |
Basal ganglia encephalitis |
Increased signal in basal ganglia (50%) |
No associated cancer |
Unknown |
Receptor internalization and decrease in D2R surface density |
DPPX |
(45) 52 yr (13–76) |
2.3:1 |
Confusion, diarrhea, weight loss |
Encephalitis, myoclonus, tremors, hyperekplexia |
Normal findings or nonspecific changes (100%) |
B-cell neoplasms (<10%) |
IgG4 |
Decrease in density of surface DPPX and Kv4.2 |
GABAAR |
(70) |
40 yr (2 mo–88 yr) |
1:1 |
Seizures, confusion, behavioral changes |
Encephalitis, frequent status epilepticus |
Cortical and subcortical FLAIR signal abnormalities involving two or more brain regions (77%) |
Thymoma (27%) |
IgG1 |
Selective reduction of GABAAR at synapses |
Neurexin-3α |
(6) |
44 yr (23–57) |
2:4 |
Confusion, seizures |
Encephalitis |
Normal findings in 4 of 6 patients |
No associated cancer |
Unknown |
Decrease in density of surface neurexin-3α and total number of synapses in neurons undergoing development |