Table 4.
Target channel | Antibodies to: | Clinical syndrome | Clinical features | Associated features | Key references |
---|---|---|---|---|---|
Kv1 complex includes LGI1, CASPR2 | LGI1, CASPR2 | Limbic encephalitis | Amnesia, change in personality or psychosis, temporal lobe and other seizure types, mainly partial complex seizures | Serum hyponatraemia | Vincent et al. 2004; Irani et al. 2010 |
Kv1 | LGI1 | Faciobrachial dystonic seizures (FBDS) | Brief frequent dystonic seizures usually unilateral often involving the arm and ipsilateral face | Can precede limbic encephalitis Often resistant to AEDs Immunotherapy-responsive | Irani et al. 2011 |
AMPAR1/2 | AMPAR1/AMPAR2 mainly | Limbic encephalitis | As above but with more evident psychosis | Lai et al. 2009 | |
AMPAR3 | AMPAR3 in a few reports, otherwise none defined | Rasmussen's encephalitis | Intractable unilateral seizures with hemiplegia | Patients develop epilepsia partialis continua (EPC) | Bien et al. 2004 |
GABAbR | GABAbR | Limbic encephalitis | As above but often dominated by TLE | Lancaster et al. 2010 | |
NMDAR | NR1 | Psychiatric features and seizures | Seizures are part of the presentation but are not well defined. | Most patients progress over days or weeks to a complex encephalopathy | Dalmau et al. 2011; Niehusmann et al. 2009 |
Full references can be found in Supplementary information. The neuronal localization of some of the channel proteins is shown in Figure 1.