TABLE.

Antibodies Associated with Paraneoplastic Syndromes

Antibody	Neurologic Syndrome	Common Cancer Association	Comments@
Onconeuronal Antibodies*
Anti-Hu (ANNA-1)	Encephalomyelitis often with PSN	SCLC	Poor response to treatment
Anti-CV2/CRMP5	Encephalomyelitis and PSN (may have motor involvement, uveitis, chorea)	SCLC, thymoma	Poor response to treatment
Anti-Yo (PCA-1)	PCD	Ovary, breast	Poor response to treatment
Anti-Ri (ANNA-2)	PCD, opsoclonus	Gynecologic, breast	Poor response to treatment
Anti-Tr/DNER	PCD	Hodgkin’s lymphoma	80% of patients are men < 45 year
Anti-Ma proteins	Limbic, brainstem and hypothalamic encephalitis	Ma2: Men < 45: germ cell tumors of the testis Other Ma: men or women with a variety of solid tumors	About one third of young men improve with treatment; older patients rarely improve
Anti-amphiphysin	Stiff-person syndrome, encephalomyelitis, PCD	Breast, SCLC	Often improves with treatment
Anti-GAD	Limbic encephalitis, cerebellar ataxia, stiff-person syndrome	Neuroendocrine	Risk of cancer increases with age, male sex, presence of concurrent neuronal cell-surface antibodies, and limbic encephalitis
Anti-recoverin	Retinopathy	SCLC	Poor response to treatment
Anti-bipolar cells	Retinopathy	melanoma	Poor response to treatment
Neuronal Cell Surface Antibodies**
Anti-NMDAR	Anti-NMDAR encephalitis	Females >12 and < 45: ovarian teratoma Females/males > 45: rare association with solid tumors	Characteristic pattern of symptom progression. Partial syndromes or less severe phenotypes can occur; almost all patients develop several elements of the syndrome. Responds well to treatment; recovery may be prolonged.
Anti-AMPAR	Limbic encephalitis with prominent psychiatric features	~ 70% of cases: SCLC, thymoma, breast	Responds well to treatment
Anti-GABA(B)R	Limbic encephalitis with severe seizures	~ 50% of cases: SCLC	Responds well to treatment
LGI1	Limbic encephalitis	< 10% of cases: Thymoma	Responds well to treatment
Caspr2	Neuromyotonia +/− CNS involvement	Thymoma	Responds well to treatment
Anti-GluR1	PCD	Hodgkin lymphoma	Only a few cases; some improved
Anti-GluR5	Limbic encephalitis	Hodgkin lymphoma	Only a few cases; some improved
Anti-AChR	Myasthenia gravis	Thymoma	Responds well to treatment
Anti-VGCC	LEMS, PCD	SCLC	Responds well to treatment
Anti-α–GlyR	PERM	Infrequent: thymoma, lymphoma	Responds well to treatment

^*Onconeuronal Antibodies: The presence of one of these antibodies almost always indicates that the disorder is paraneoplastic). Only antibodies for which the target autoantigen and syndrome specificities have been confirmed by several investigators are listed. Antibodies that have been identified in isolated cases or small series and/or confirmation of the target antigen and syndrome specificity is pending are not listed.

^**Neuronal Cell Surface Antibodies: These antibodies are markers of the neurologic syndrome but do not distinguish between cases that are paraneoplastic or not). Other neuronal cell surface antibodies that associate with specific syndromes but have not been found to occur in association with cancer are not listed.

^@Treatment refers to treatment of the tumor if present and immunotherapy (including among others corticosteroids, intravenous immunoglobulins, plasma exchange, cyclophosphamide, rituximab).

PSN: paraneoplastic sensory neuronopathy; SCLC: small-cell lung cancer; PCD: paraneoplastic cerebellar degeneration; GAD: glutamic acid decarboxylase; NMDAR: N-methyl-D-aspartate receptor; AMPAR: α-amino-3-hydroxy-5-methylisoxazole-4-propionic acid receptor; GABA(B)R: gamma-amino-butyric acid type B receptor; LGI1: leucine-rich glioma inactivated 1; Caspr2: connectin-associated protein 2; GluR1: metabotropic glutamate receptor 1; mGluR5: metabotropic glutamate receptor 5; AChR: acetylcholine receptor; VGCC: voltage-gated calcium channel; LEMS: Lambert-Eaton myasthenic syndrome; GlyR: glycine receptor; PERM: progressive encephalomyelitis with myoclonus.