Table 2. Major paraneoplastic antineuronal antibodies reactive with intracellular neuronal antigen.
Antibody | Common neurological
phenotypes |
Common associated
malignancies |
Frequency of
underlying malignancy |
Response to
treatment a |
---|---|---|---|---|
Anti-CRMP5 | Optic neuritis
Cerebellar degeneration Encephalomyelitis |
Small-cell lung cancer
Breast carcinoma |
Common | Poor |
Anti-GAD65 | Stiff person syndrome
Limbic encephalitis Cerebellar ataxia |
Thymoma
Renal cell carcinoma |
Uncommon | Good in stiff
person syndrome; otherwise poor |
Anti-Hu
(ANNA-1) |
Limbic encephalitis,
encephalomyelitis, dorsal sensory neuropathy |
Small-cell lung cancer
Neuroendocrine tumors Retinoblastoma (infants) |
Almost invariable | Poor |
Anti-Ma1 | Limbic or brain-stem
encephalitis |
Non-small-cell lung
cancer; other |
Almost invariable | Poor |
Anti-Ma2 | Limbic or brain-stem
encephalitis |
Testicular or other germ
cell tumors Non-small-cell lung cancer |
Almost invariable | Good if testicular
neoplasm detected and treated |
Anti-Ri
(ANNA-2) |
Cerebellar degeneration,
opsoclonus myoclonus, brain-stem encephalitis |
Breast
Small-cell lung cancer |
Almost invariable | Stabilization in
some but not all patients |
Anti-Tr | Cerebellar degeneration | Hodgkin’s disease | Almost invariable | Poor |
Anti-Yo
(PCA-1) |
Cerebellar degeneration | Ovary, uterus, adnexa
Breast |
Almost invariable | Poor |
Modified from 13. aTreatment involves both immunosuppressive treatment directed against the autoimmune process and treatment of the underlying malignancy.