Table 4.

Seizure-related syndromes associated with antibodies to ion channels or receptors

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.