TABLE 1

Autoantibody biomarkers of autoimmune encephalitis: Intracellular autoantibodies

ANTIBODY TARGETCENTRAL NERVOUS SYSTEM FEATURESPERIPHERAL NERVOUS SYSTEM FEATURESOTHERASSOCIATED MALIGNANCY
High-risk paraneoplastic autoantibodies
ANNA-1 (Hu)Limbic encephalitis
Encephalomyelitis
Cerebellar ataxia
Sensory neuropathyGastrointestinal dysmotilitySCLC Rare: neuroblastoma
ANNA-2 (Ri)Encephalomyelitis
Cerebellar ataxia
Rhombencephalitis
Jaw dystonia LaryngospasmSCLC Breast carcinoma
ANNA-3Limbic encephalitis
Encephalomyelitis
Cerebellar degeneration
Sensory and sensorimotor neuropathiesSCLC
AmphiphysinStiff-person spectrum
disorder
SCLC Breast or ovarian carcinoma
CRMP-5Limbic encephalitis
Cerebellar ataxia
Chorea
Myelopathy
Cranial neuropathies
(optic neuritis)
PolyradiculoneuropathySCLC Thymoma carcinoma
GAD65Stiff-person spectrum
disorder
Limbic encephalitis
Cerebellar ataxia
Rare
GFAPMeningoencephalitis
Myelitis
Optic neuritis
Ovarian teratoma Adenocarcinomas of various sites
PCA-1 (Yo)Cerebellar ataxiaBreast or ovarian carcinoma
PCA-2Limbic encephalitis
Cerebellar ataxia
PolyneuropathySCLC
PCA-Tr (DNER)Limbic encephalitis
Cerebellar ataxia
Hodgkin lymphoma
Ma 1 andDiencephalitisMa1: Common, diverse
Ma 2 (Ta)Limbic encephalitis
Brain stem encephalitis
Cerebellar degeneration
Ma2: Testicular seminoma
  • ANNA = antineuronal nuclear antibody; CRMP-5 = collapsin response mediator protein 5; DNER = delta/notch-like epidermal growth factor-related receptor; GAD = glutamic acid decarboxylase; GFAP = glial fibrillary acidic protein; PCA = Purkinje cell cytoplasmic antibody; SCLC = small-cell lung cancer