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Table 1 Most important antibodies and clinical syndromes

From: SOP: antibody-associated autoimmune encephalitis

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

  1. LE: limbic encephalitis, SPS: Stiff-person syndrome, OMS: Opsoclonus-myoclonus syndrome, CS: cerebellar syndrome, SCLC: small cell lung cancer, PCD: paraneoplastic cerebellar degeneration