Table 1.
Most important antibodies and clinical syndromes
| Antigen | Characteristics | Preferred detection | Age/Gender | Tumour |
|---|---|---|---|---|
| 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 brush | CSF | Most prevalent subtype of AE; All ages, peak in childhood and youth, 75% women | Ovarian teratoma |
| GABAaR | Epileptic seizures, schizophreniform syndrome, refractory status epilepticus and epilepsia partialis continua | Serum or CSF | Younger adults; m > f (1.5:1) | Hodgkin lymphoma |
| GABAbR | LE with frequent epileptic seizures | CSF | Older adults f = m | 50% lung cancer (SCLC) |
| AMPAR | LE, Epileptic seizures, memory deficits, psychosis; CSF often normal | CSF | Older Adults f > m (2.3:1) | In 70% lung/ breast cancer |
| mGluR5 | LE, Ophelia syndrome (depression, agitation, hallucination, memory deficits, personality changes) | CSF | Young adults, m > f, (1.5:1) | Hodgkin lymphoma |
| GlycinR | PERM (progressive encephalomyelitis with rigidity and myoclonus), SPS, cognitive deficits | Serum or CSF | Older adults f = m | Thymoma (< 10%) |
| DPPX | LE with tremor, myoclonus, hallucinations, therapy refractory diarrhoea | CSF | Older adults f < m (1:2.3) | Not known |
| Antibodies against ion channel subunits or cell adhesion molecules [4, 5] | ||||
| LGI1 | Facio-brachial dystonic seizures, amnesia, psychosis, LE, Medial temporal lobe hyperintensities in MRI, hyponatremia | Serum | Second most common type of AE; Adults > 40 years, m > f (2:1) | Rare |
| Caspr2 | LE, neuromyotonia, Morvan syndrome, can slowly progress over up to 1 year; similar to LGI1, but no hyponatremia | Serum | Elderly m > f (9:1) | Thymoma possible |
| IgLON5 | REM- and non-REM sleep disorders, sleep apnoea, stridor, dysarthria, dysphagia, dysautonomia, movement disorders, dementia | Serum | Older adults, f = m | Not known |
| Antibodies against glial structures | ||||
| GFAP [6] | Headache, subacute encephalopathy, optic papillitis, myelitis, CS | Serum and CSF | f = m | Possible |
| Antibodies against Intracellular (onconeural) antigens [7, 8] | ||||
| Hu (ANNA-1) | Encephalomyelitis, brainstem encephalitis, LE, Denny-Brown syndrome | Serum | Large variability, depending on tumour occurrence | > 90%, SCLC |
| Ri (ANNA-2) | OMS, CS, encephalomyelitis | Serum | > 90%, Ovary, breast cancer | |
| Yo (PCA-1) | CS | Serum | > 90%, Ovary cancer | |
| Ma2 | LE, CS, diencephalic/ hypothalamic involvement | Serum | > 90%, Testicular, lung cancer | |
| CV2 (CRMP5) | Encephalomyelitis, LE, CS | Serum | > 90%, SCLC, thymoma | |
| Amphiphysin | SPS | Serum | > 90%, Breast, SCLC | |
| GAD | SPS, LE, ataxia | Serum and CSF | Middle aged, f > m (4:1) | Only rarely associated with tumour |
LE: limbic encephalitis, SPS: Stiff-person syndrome, OMS: Opsoclonus-myoclonus syndrome, CS: cerebellar syndrome, SCLC: small cell lung cancer, PCD: paraneoplastic cerebellar degeneration