Table 4.
Dystonia associated with antibodies
| Anbody | Typical age at onset |
Clinical features | Association with malignancy |
|---|---|---|---|
| AQP4 | children or adults | tonic spasms may resemble paroxysmal dystonia; often combined with optic neuritis and/or myelitis | sometimes |
| CV2 or CRMP5 | children or adults | dystonia may be combined with chorea, parkinsonism, optic neuritis, myelitis and encephalopathy | frequent |
| D2R | children | dystonia may be combined with chorea or parkinsonism, in setting of encephalopathy that includes prominent behavioral and sleep disturbances | not usually |
| GAD or amphiphysin | adults | stiff-person syndrome may present with a syndrome that is easily mistaken for dystonia | occasional |
| GABAAR | children or adults | dystonia may be combined with chorea ataxia, sometimes opsoclonus-myoclonus in setting of broader encephalopathy | frequent |
| LGI1 | adults | faciobrachial dystonic seizures may resemble paroxysmal segmental dystonia, often with encephalopathy and sleep disorder | occasional |
| Ma2 | children or adults | dystonia may be combined with parkinsonism and sleep disorder in setting of broader encephalopathy | frequent |
| NMDAR | children or adults | dystonia may paroxysmal or combined with chorea, ataxia, myoclonus, parkinsonism, oculogyric crises in setting of broader encephalopathy | frequent |
| Ri or ANNA-2 | adults | dystonia may be combined with ataxia, myoclonus, rigidity, opsoclonus and cranial nerve signs of brainstem encephalitis | frequent |