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Table 1 Most important antibodies and clinical syndromes

From: SOP: antibody-associated autoimmune encephalitis

AntigenCharacteristicsPreferred detectionAge/GenderTumour
Antibodies against neurotransmitter receptors [2]
NMDAR [3]Schizophreniform psychosis, perioral dyskinesia, epileptic seizures, coma, dystonia, hypoventilation; cMRI frequently normal, often CSF pleocytosis, EEG with slow waves, can show extreme delta brushCSFMost prevalent subtype of AE; All ages, peak in childhood and youth, 75% womenOvarian teratoma
GABAaREpileptic seizures, schizophreniform syndrome, refractory status epilepticus and epilepsia partialis continuaSerum or CSFYounger adults; m > f (1.5:1)Hodgkin lymphoma
GABAbRLE with frequent epileptic seizuresCSFOlder adults f = m50% lung cancer (SCLC)
AMPARLE, Epileptic seizures, memory deficits, psychosis; CSF often normalCSFOlder Adults f > m (2.3:1)In 70% lung/ breast cancer
mGluR5LE, Ophelia syndrome (depression, agitation, hallucination, memory deficits, personality changes)CSFYoung adults, m > f, (1.5:1)Hodgkin lymphoma
GlycinRPERM (progressive encephalomyelitis with rigidity and myoclonus), SPS, cognitive deficitsSerum or CSFOlder adults f = mThymoma (<  10%)
DPPXLE with tremor, myoclonus, hallucinations, therapy refractory diarrhoeaCSFOlder adults f < m (1:2.3)Not known
Antibodies against ion channel subunits or cell adhesion molecules [4, 5]
LGI1Facio-brachial dystonic seizures, amnesia, psychosis, LE, Medial temporal lobe hyperintensities in MRI, hyponatremiaSerumSecond most common type of AE; Adults > 40 years, m > f (2:1)Rare
Caspr2LE, neuromyotonia, Morvan syndrome, can slowly progress over up to 1 year; similar to LGI1, but no hyponatremiaSerumElderly m > f (9:1)Thymoma possible
IgLON5REM- and non-REM sleep disorders, sleep apnoea, stridor, dysarthria, dysphagia, dysautonomia, movement disorders, dementiaSerumOlder adults, f = mNot known
Antibodies against glial structures
GFAP [6]Headache, subacute encephalopathy, optic papillitis, myelitis, CSSerum and CSFf = mPossible
Antibodies against Intracellular (onconeural) antigens [7, 8]
Hu (ANNA-1)Encephalomyelitis, brainstem encephalitis, LE, Denny-Brown syndromeSerumLarge variability, depending on tumour occurrence>  90%, SCLC
Ri (ANNA-2)OMS, CS, encephalomyelitisSerum>  90%, Ovary, breast cancer
Yo (PCA-1)CSSerum>  90%, Ovary cancer
Ma2LE, CS, diencephalic/ hypothalamic involvementSerum >  90%, Testicular, lung cancer
CV2 (CRMP5)Encephalomyelitis, LE, CSSerum >  90%, SCLC, thymoma
AmphiphysinSPSSerum >  90%, Breast, SCLC
GADSPS, LE, ataxiaSerum and CSFMiddle aged, f > m (4:1)Only rarely associated with tumour
  1. LE: limbic encephalitis, SPS: Stiff-person syndrome, OMS: Opsoclonus-myoclonus syndrome, CS: cerebellar syndrome, SCLC: small cell lung cancer, PCD: paraneoplastic cerebellar degeneration