Table 6. Autoimmune encephalitis studies in pediatric epilepsy.
| Surface antigens (channels and receptor targets) | |
|---|---|
| Epilepsy syndrome/epilepsy type | Autoimmune marker |
| Rasmussen |
Glutamate receptor targets
○ AMPAR ▪ GluR3 47, 48 ▪ GluR2-GluR3 Complex 47, 49 ○ NMDAR GluN2 48 |
| FIRES | GABA
AR
48,
50
VGKV Complex 48 GAD65 48 AMPAR 48 GluA3 48 |
| West syndrome | NMDR
51
GABA AR 51 GAD 51 |
| LGS | GABA
AR
51
GlyR 51 |
| Human AD Lateral TLE | Contactin 2 46 |
| Severe epilepsy - status epilepticus, epilepsia partialis continua | GABA AR 46, 48, 51, 52 |
| Encephalitis with Faciobrachial Dystonic Seizures | LGI1 & CASPR2 46, 48, 51, 52 |
| Progressive encephalomyelitis with rigidity and myoclonus | GlyR 51, 52 |
| Cerebral folate deficiency Myoclonic, atypical absence, generalized tonic-clonic seizures
and developmental delay and irritability |
FRα 53 |
|
Limbic Encephalitis
Anti-AMPAR-psychosis and seizures Anti-CASPR2-Neuromyotonia, polyneuropathy, bulbar weakness Anti-NMDAR- psychiatric changes, partial seizures, movement disorders Anti-GABA B- frequent temporal lobe-onset simple and complex partial seizures. Neuropsychological changes Focal onset Seizures memory and psychiatric changes, partial seizures, insomnia |
AMPAR
○ GluA1 46, 48 ○ GluA2 46, 48 CASPR2 51 NMDAR ○ GluN1 48, 51, 52 GABA BR 48, 52 VGKC Complex 52 Contactin 2 46 CASPR2 LGI1 46, 48 |
| Other limbic encephalitis | GAD/GAD65
46,
48,
51,
52
Ri (Nova) 46, 48 Yo 46, 48, 52 Hu 46, 48, 52 MA2 46, 52 Tr 46, 48, 52 Amphiphysin 46 CRMP5 46 DNER 46 |
This table lists many of the identified auto-antibodies that are associated with different encephalitides that cause seizures in pediatric patients. It lists antibodies according to their mechanism of action, clinical syndromes, and nervous system antigen target. An evaluation confirming possible autoimmune encephalitis and treatment for autoimmune encephalitis should precede the return of test results for autoimmune encephalitis. AMPAR, AMPA receptor; CASPR2, contactin-associated protein-like 2; FRα, folate receptor α; GABAAR, GABA A receptor; GAD, glutamic acid dehydrogenase; GAD65, Glutamate dehydrogenase protein; GluA1, GluA2, Glur2, and GluR2 and 3, AMPA receptor subunits; GluN2, NMDA subunit; GluA3, GluN2, and GluN1, NMDAR subunits; GlyR, glycine receptor; NMDR, NMDA receptor; VGKC- and LGI1, voltage-gated K channel complex proteins; VGKV complex, voltage-gated K channel protein.