Table 1

Antibodies to intracellular antigens

AntibodyOncological associationFrequency of tumourResponse to immunotherapyNeurological manifestations
ANNA-1
(anti-Hu)
Small-cell carcinoma.>90%PoorLimbic, cortical encephalitis.
Autonomic neuropathies, sensory neuronopathy and other peripheral neuropathies.
ANNA2
(anti-Ri)
Small-cell carcinoma, breast adenocarcinoma and bladder cancer.>60%PoorBrainstem encephalitis (opsoclonus–myoclonus, laryngospasm, trismus and cranial neuropathy) and cerebellar degeneration.
ANNA3Small-cell carcinoma.>60%PoorLimbic and brainstem encephalitis, sensory and sensorimotor neuropathies and myelopathy.
PCA2Small-cell carcinoma.>90%PoorBrainstem or limbic encephalitis and cerebellar degeneration.
Ma1, Ma2Testicular (Ma2); breast, colon and testicular (Ma1).>90%ModerateMa2 Limbic encephalitis, diencephalitis, brainstem encephalitis; Ma1 and Ma2 brainstem encephalitis and cerebellar degeneration.
CRMP-5Small-cell carcinoma and thymoma.>75%PoorEncephalitis.
Optic neuritis and retinitis, myelopathy, neuropathy and Lambert–Eaton myasthenic syndrome.
AmphiphysinSmall-cell carcinoma and breast adenocarcinoma.>90%PoorLimbic encephalitis.
Myelopathy, stiff-man syndrome and cerebellar degeneration.
GAD65Thymoma; neuroendocrine tumours, breast or colon adenocarcinoma.<10%ModerateStiff-person syndrome, stiff-person phenomena, brainstem encephalitis and cerebellar degeneration.
GFAPNone described to date.GoodMeningoencephalomyelitis, headache, papillitis and cerebellar ataxia.
  • ANNA, antineuronal nuclear antibody; CRMP-5, collapsin response mediator protein-5; GAD65, glutamic acid decarboxylase 65; GFAP, glial fibrillar acidic protein; PCA, Purkinje cell cytoplasmic antibody.