Antibodies to intracellular antigens
Antibody | Oncological association | Frequency of tumour | Response to immunotherapy | Neurological manifestations |
ANNA-1 (anti-Hu) | Small-cell carcinoma. | >90% | Poor | Limbic, cortical encephalitis. Autonomic neuropathies, sensory neuronopathy and other peripheral neuropathies. |
ANNA2 (anti-Ri) | Small-cell carcinoma, breast adenocarcinoma and bladder cancer. | >60% | Poor | Brainstem encephalitis (opsoclonus–myoclonus, laryngospasm, trismus and cranial neuropathy) and cerebellar degeneration. |
ANNA3 | Small-cell carcinoma. | >60% | Poor | Limbic and brainstem encephalitis, sensory and sensorimotor neuropathies and myelopathy. |
PCA2 | Small-cell carcinoma. | >90% | Poor | Brainstem or limbic encephalitis and cerebellar degeneration. |
Ma1, Ma2 | Testicular (Ma2); breast, colon and testicular (Ma1). | >90% | Moderate | Ma2 Limbic encephalitis, diencephalitis, brainstem encephalitis; Ma1 and Ma2 brainstem encephalitis and cerebellar degeneration. |
CRMP-5 | Small-cell carcinoma and thymoma. | >75% | Poor | Encephalitis. Optic neuritis and retinitis, myelopathy, neuropathy and Lambert–Eaton myasthenic syndrome. |
Amphiphysin | Small-cell carcinoma and breast adenocarcinoma. | >90% | Poor | Limbic encephalitis. Myelopathy, stiff-man syndrome and cerebellar degeneration. |
GAD65 | Thymoma; neuroendocrine tumours, breast or colon adenocarcinoma. | <10% | Moderate | Stiff-person syndrome, stiff-person phenomena, brainstem encephalitis and cerebellar degeneration. |
GFAP | None described to date. | Good | Meningoencephalomyelitis, 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.