Antibody target |
Onset |
Features |
Clinical details |
Childhood |
Adulthood |
Isolated |
Combined |
Chorea and dyskinesia |
CV2/CRMP5 |
|
+ |
|
+ |
Typically combined with cognitive decline, neuropathy, optic neuritis, myelitis; MRI: often FLAIR hyperintensities (white matter, basal ganglia, temporomesial) |
Hu |
|
+ |
|
+ |
Typically combined with gastrointestinal pseudoobstruction, sensorineuronal hearing loss; MRI: often FLAIR hyperintensities (white matter, basal ganglia, temporomesial) |
CASPR2 |
|
+ |
+ |
+ |
Chorea preceding or combined with behavioural changes |
LGI1 |
+ |
+ |
+ |
+ |
Chorea preceding or combined with cognitive impairment and encephalopathy; typically in (later) adulthood |
NMDAR |
+ |
+ |
+ |
+ |
Chorea or characteristic orofacial and limb dyskinesias; truly isolated presentations are rare, mostly combined with ataxia (in children), neuropsychiatric symptoms, epilepsy, or other signs of encephalopathy |
Neurexin-3α |
|
+ |
|
+ |
Mild orofacial dyskinesia combined with encephalopathy with epilepsy, altered consciousness, memory deficits, psychomotor agitation |
GABAAR |
+ |
+ |
+ |
+ |
Chorea as part of an encephalopathic syndrome with epilepsy, behavioural or cognitive problems or reduced consciousness, can be combined with ataxia or dystonia; MRI: frequent T2-weighted hyperintensities |
D2R |
+ |
|
+ |
+ |
As part of encephalitis in children, or in 'Sydenham's chorea' |
IgLON5 |
|
+ |
|
+ |
Combined with prominent sleep behaviour disorder and bulbar symptoms; possible additional features: cognitive decline, ataxia, dysautonomia, central hypoventilation, oculomotor disturbance |
Dystonia |
CV2/CRMP5 |
|
+ |
|
+ |
Combined with other signs of encephalopathy |
Ma2 |
|
+ |
|
+ |
Combined with other signs of encephalopathy |
NMDAR |
+ |
+ |
+ |
+ |
Combined with other signs of encephalopathy (e.g. behavioural changes, epilepsy); rarely, hemidystonia or dystonia of neck and larynx as the most prominent symptom in children and young adults |
GABAAR |
+ |
+ |
|
+ |
Dystonia as part of an encephalopathic syndrome with epilepsy, behavioural or cognitive problems or reduced consciousness, can be combined with ataxia or chorea; MRI: frequent T2-weighted hyperintensities |
D2R |
+ |
|
|
+ |
Combined with other signs of encephalopathy in children |
Myoclonus |
LGI1 |
|
+ |
|
+ |
Combined with other signs of encephalopathy, important mimic of Creutzfeldt-Jakob disease |
CASPR2 |
|
+ |
|
|
Myoclonus affecting stance and gait, mainly in elderly males, combined with neuropsychiatric, cognitive or neuropathic symptoms |
DPPX |
+ |
+ |
|
+ |
Combined in a multifocal encephalopathy, red flag: gastrointestinal symptoms (particularly diarrhoea) |
Neurexin-3α |
|
+ |
|
+ |
Combined with other signs of encephalopathy, resembling encephalitis with NMDAR antibodies |
Ri |
|
+ |
|
+ |
Adult paraneoplastic OMSa |
Ma2 |
|
+ |
|
+ |
Adult paraneoplastic OMSa |
Zic4 |
|
+ |
|
+ |
Adult paraneoplastic OMSa |
Hu |
|
+ |
|
+ |
Adult paraneoplastic OMSa |
Yo |
|
+ |
|
+ |
Adult paraneoplastic OMSa |
CV2/CRMP5 |
|
+ |
|
+ |
Adult paraneoplastic OMSa |
VGCC |
|
+ |
|
+ |
Adult paraneoplastic OMSa |
GAD |
|
+ |
|
+ |
OMSa without underlying malignancy |
GQ1B |
|
+ |
|
+ |
OMSa without underlying malignancy |
NMDAR |
+ |
+ |
|
+ |
OMSa without underlying malignancy |
GABAAR |
+ |
+ |
|
+ |
OMSa without underlying malignancy |
DPPX |
+ |
+ |
|
+ |
OMSa without underlying malignancy |
GABABR |
+ |
+ |
|
+ |
Paediatric and adult cases of OMS |
GlyR |
+ |
+ |
|
+ |
Myoclonus typically as part of → combined SPSD, rarely in OMSa |
Parkinsonism |
D2R |
+ |
|
|
+ |
Vary rare: combined with other signs of encephalopathy, in children |
NMDAR |
+ |
+ |
|
+ |
Combined with other signs of encephalopathy |
LGI1 |
|
+ |
|
+ |
Combined with other signs of encephalopathy |
CRMP5 |
|
+ |
|
+ |
Combined with other signs of encephalopathy |
Ri |
|
+ |
|
+ |
Combined with other signs of encephalopathy |
DPPX |
|
+ |
|
+ |
Combined with other signs of encephalopathy |
Ma2 |
|
+ |
|
+ |
Subacute parkinsonism/PSP phenotype with supranuclear gaze palsy (vertical > horizontal) and constant eye closure resembling apraxia of lid opening, combined with additional signs of limbic, diencephalic or brainstem encephalitis, myelopathy or radiculoplexopathy; red flags: hypothalamic-pituitary endocrine dysfunction, weight gain, prominent sleep disorders; MRI: T2 hyperintensities of pons, midbrain, thalamus, basal ganglia, cerebellar peduncles, hypothalamus, amygdala, or temporal lobe; sometimes only atrophy or no abnormalities |
IgLON5 |
|
+ |
|
+ |
Combined with prominent sleep behaviour disorder and bulbar symptoms; possible additional features: gait instability and supranuclear gaze palsy (PSP phenotype); other oculomotor disturbance, cognitive decline, dysautonomia, central |
Ataxia |
GAD |
+ |
+ |
+ |
+ |
Isolated or combined with SPSD, focal epilepsy, limbic encephalitis; often preceding episodes of brainstem or cerebellar dysfunction; often organ-specific autoimmunity (diabetes, thyroiditis, vitiligo, pernicious anaemia) |
CASPR2 |
|
+ |
+ |
+ |
Isolated ataxia or combined with encephalopathy with seizures and cognitive impairment |
DPPX |
+ |
+ |
|
+ |
Combined with encephalopathy; red flag: gastrointestinal symptoms (particularly diarrhoea) |
NMDAR |
+ |
+ |
|
+ |
Combined with other signs of encephalopathy; ataxia is more frequent in children |
IgLON5 |
|
+ |
|
+ |
Combined with prominent sleep behaviour disorder and bulbar symptoms; possible other features: chorea, cognitive decline, dysautonomia, central hypoventilation, oculomotor disturbance |
VGCC |
|
+ |
+ |
+ |
Paraneoplastic cerebellar degeneration (mostly lung cancer), isolated or combined with Lambert-Eaton syndrome or limbic encephalitis |
Yo/CDR2 |
|
+ |
+ |
+ |
Paraneoplastic cerebellar degeneration (gynaecological tumours), isolated or combined e.g. with brainstem encephalitis, neuropathy |
Hu/ANNA-1 |
|
+ |
+ |
+ |
Paraneoplastic cerebellar degeneration (mostly lung cancer) combined with limbic or brainstem encephalitis, myelitis or neuropathy |
Ri/ANNA-2 |
|
+ |
+ |
+ |
Paraneoplastic cerebellar degeneration combined with limbic or brainstem encephalitis, myelitis, |
PCA2 |
|
+ |
+ |
+ |
Paraneoplastic cerebellar degeneration combined with limbic or brainstem encephalitis, myelitis, neuropathy, Lambert-Eaton Syndrome |
ANNA3 |
|
+ |
+ |
+ |
Paraneoplastic cerebellar degeneration combined with limbic or brainstem encephalitis, myelitis, neuropathy |
Zic4 |
|
+ |
+ |
+ |
Paraneoplastic cerebellar degeneration (mostly lung cancer), mostly isolated, very rarely combined with Lambert-Eaton myasthenic syndrome |
Sox1 |
|
+ |
+ |
+ |
Paraneoplastic cerebellar degeneration, isolated or combined with brainstem encephalitis neuropathy, Lambert-Eaton syndrome |
DNER |
|
+ |
+ |
+ |
Isolated ataxia or combined with encephalopathy or neuropathy |
mGluR1 |
|
+ |
+ |
+ |
Isolated or combined with dysgeusia, memory or attention deficits, psychiatric problems |
GABABR |
+ |
+ |
|
+ |
Isolated or combined with brainstem encephalitis or in encephalitis with opsoclonus, chorea and seizures |
GQ1b |
+ |
+ |
|
+ |
Miller-Fisher syndrome with ophthalmoplegia, mydriasis and areflexia |
GFAP |
+ |
+ |
|
+ |
Combined in meningoencephalomyelitis (or limited forms) with encephalopathy with epilepsy, cognitive or psychiatric problems, myelopathy (longitudinal or transversal); red flags: meningeal symptoms (headache, photophobia, neck stiffness), optic disk oedema, myelopathy; MRI: frequently characteristic radial linear periventricular or cerebellar gadolinium enhancement |
Ca/ARHGAP26 |
|
+ |
+ |
+ |
Rare; isolated or combined with hyperekplexia or cognitive decline |
Homer-3 |
|
+ |
+ |
+ |
Rare; isolated or combined with encephalopathy |
ITPR1 |
|
+ |
+ |
|
Rare; clinical data scarce |
CARP VIII |
|
+ |
+ |
|
Rare; rapidly progressive paraneoplastic cerebellar ataxia |
PKC-γ |
|
+ |
+ |
|
Rare; two patients with paraneoplastic cerebellar ataxia |
GluR-δ2 |
+ |
+ |
+ |
+ |
Rare; isolated or combined with encephalopathy |
Nb/AP3B2 |
|
+ |
|
+ |
Rare; combined with pyramidal involvement |
ATP1A3 |
|
+ |
|
+ |
Rare; combined with vertical gaze palsy, spastic tetraparesis, deterioration of visual acuity |
Stiff person spectrum disorders |
GAD |
+ |
+ |
+ |
+ |
Isolated or combined SPSD e.g. with ataxia, epilepsy, oculomotor disturbance, dysautonomia, pyramidal signs, sensory symptoms or encephalopathy; often associated with organ-specific autoimmunity, e.g. diabetes type 1, vitiligo, thyroiditis, pernicious anaemia |
GlyR |
+ |
+ |
+ |
+ |
Isolated or combined SPSD e.g. oculomotor disturbance, bulbar symptoms, dysautonomia, pyramidal signs, sensory symptoms, encephalopathy |
Amphiphysin |
|
+ |
+ |
+ |
Isolated or combined with with sensory ganglionopathy, myelopathy |
Paraneoplastic SPSD with breast or small cell lung cancer |
GABAAR |
+ |
+ |
+ |
+ |
Isolated or combined with epilepsy; partly co-occurring with → GAD antibodies |
DPPX |
+ |
+ |
|
+ |
Combined SPSD with prominent hyperekplexia and myoclonus, cerebellar ataxia, dysautonomia, pyramidal signs, sensory symptoms, cognitive problems; red flags: prolonged diarrhoea, other gastrointestinal symptoms |
Gephyrin |
|
+ |
|
+ |
Single case, combined SPSD with dysarthria and dysphagia |
GlyT2 |
|
+ |
+ |
+ |
Preliminary report of two cases, patients were also positive for → GAD antibodies |
GABARAP |
|
+ |
+ |
+ |
All reported patients were also positive for → GAD antibodies |
Ri |
|
+ |
|
+ |
Combined SPSD as part of brainstem encephalitis |
Paroxysmal dyskinesias |
LGI1 |
|
+ |
+ |
+ |
Characteristic FBDS, isolated or combined with other signs of limbic encephalitis; red flags: hyponatraemia, bradycardia as neurocardiac prodrome |
NMDAR |
+ |
+ |
+ |
+ |
Paroxysmal dystonic posturing preceding encephalitis |
AQP4 |
+ |
+ |
+ |
+ |
Painful tonic spasms in neuromyelitis optica, often combined with sensory, motor, visual or sphincter disturbance |
Neuromyotonia and myokymia |
CASPR2 |
|
+ |
+ |
+ |
Main cause of immune-mediated peripheral nerve hyperexcitability, either in isolation or combined with pain, neuropathy or as part of Morvan syndrome |
LGI1 |
|
+ |
+ |
+ |
Rarely in CASPR2 antibody-negative cases |
Tics |
D2R |
+ |
|
+ |
|
Very rare; reported in 4/44 children with Tourette's syndrome, relevance in clinical practice still to be established |
Tremor |
MAG |
|
+ |
|
+ |
In chronic inflammatory demyelinating neuropathy |
LGI1 |
|
+ |
|
+ |
As part of more widespread involvement in encephalitis |
CASPR2 |
|
|
|
|
As part of more widespread involvement in encephalitis |
DPPX |
+ |
+ |
|
+ |
As part of more widespread involvement in encephalitis |
NMDAR |
+ |
+ |
|
+ |
As part of more widespread involvement in encephalitis |
Yo |
|
+ |
|
+ |
Holmes tremor in cerebellar ataxia |
GFAP |
+ |
+ |
|
+ |
Combined in meningoencephalomyelitis (or limited forms) with encephalopathy with epilepsy, cognitive or psychiatric problems, myelopathy (longitudinal or transversal), or ataxia; red flags: meningeal symptoms (headache, photophobia, neck stiffness), optic disk oedema, myelopathy; MRI: frequently characteristic radial linear periventricular or cerebellar gadolinium enhancement |
Sleep movement disorders |
NMDAR |
+ |
+ |
|
+ |
Status dissociatus and agrypnia excitata in encephalopathic syndrome |
CASPR2 |
|
+ |
|
+ |
Status dissociatus and agrypnia excitata in Morvan syndrome |
GABABR |
|
+ |
|
+ |
Agrypnia excitata in encephalopathic syndrome |
Ma2 |
|
+ |
|
+ |
RBD in characteristic → parkinsonism syndrome |
LGI1 |
|
+ |
|
+ |
RBD in limbic encephalitis |
DPPX |
|
+ |
|
+ |
Periodic limb movements of sleep |
IgLON5 |
|
+ |
|
+ |
RBD and non-RBD and parasomnias |