Table 2

Antibodies targetting neuronal cell surface and synaptic proteins

AntibodyOncological associationFrequency of tumourResponse to immunotherapyNeurological manifestations
VGKC complex
Thymoma, small-cell lung cancer.<10%GoodLimbic encephalitis, hyponatremia and faciobrachial dystonic seizures.
CASPR2Thymoma40%GoodIsaacs syndrome, Morvan’s syndrome and limbic encephalitis.
NMDAROvarian teratomas, testicular germinoma and neuroblastoma.Varies with age, sex, and ethnicityGoodPsychiatric disturbances, dyskinesias, catatonia, central hypoventilation and autonomic instability, and opsoclonus–myoclonus.
AMPARThymic tumours, lung carcinoma and breast adenocarcinoma.70%GoodLimbic encephalitis and nystagmus.
GABA-A receptorThymoma, small-cell lung cancer and rectal cancer.40%GoodStatus epilepticus, epilepsia partialis continua, psychosis, behavioural disturbances, orolingual dyskinesias and chorea.
GABA-B receptorSmall-cell lung carcinoma and other neuroendocrine neoplasia.70%GoodLimbic encephalitis and orolingual dyskinesias.
mGluR5 receptorHodgkin’s lymphoma.>90%GoodCerebellar ataxia and limbic encephalitis (Ophelia syndrome).
GlyRThymoma, breast cancer and Hodgkin’s lymphoma.<10% of published casesModerateProgressive encephalomyelitis with rigidity and myoclonus, oculomotor disturbances, dysautonomia, hyperekplexia and respiratory failure.
DPPXNone described to date.ModerateEncephalitis, sleep disturbances, myoclonus, hyperekplexia, dysautonomia and gastrointestinal dysmotility.
IgLON5None described to date.PoorNon-REM parasomnias, REM sleep behaviour disorder, apnoea, stridor and cognitive decline.
  • AMPAR, alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor; Caspr2, contactin-associated protein-like 2; DPPX, dipeptidyl-peptidase-like protein-6; GABA-A, γ-aminobutyric acid--A; GABA-B, γ-aminobutyric acid-B; GlyR, glycine receptor; LGI1, leucine rich glioma inactivated protein 1; mGluR5, metabotropic glutamate receptor 5.NMDAR, N-methyl-D-aspartate receptor; REM, rapid eye movement; VGKC, voltage gated potassium channel;