Syndrome | Antibodies | Particular clinical features | Possible tumours | Immunotherapy response | In vitro evidence of Ab pathogenicity | Frequency or No of cases reported |
NMDAR-Ab encephalitis | NMDAR | Dyskinetic movements, decreased consciousness, psychiatric presentation in young women. Epilepsy and abnormal movements more frequent at onset in children | Ovarian teratoma. Rare in children. Up to 50% after age 18 years | Yes | In vitro and in vivo reduction of NMDA receptors |
|
LE |
| Male predominance, hyponatraemia, faciobrachial dystonic seizures, myoclonus | Rare with LGI1-Ab. Thymoma in some with CASPR2-Ab | Yes | In vitro production of epileptogenic activity in brain slices |
|
AMPAR | Possible isolated psychiatric symptoms | 70% (lung, breast, thymus) | Yes, frequent relapses | Downregulation of AMPA receptors | 14 | |
GABABR | Prominent seizures | 60% (SCLC) | Yes | None | 25 | |
mGluR5 | Ophelia syndrome | Hodgkin lymphoma | Unknown | None | 2 | |
Morvan's syndrome | CASPR2 | Encephalopathy, peripheral nerve hyperexcitability, dysautonomia | Thymoma | Yes | Not tested | 9 |
PERM | GlyR | Encephalomyelitis with myoclonus, rigidity and brainstem signs | Thymoma | Yes | Not tested | 6 |
Cerebellar ataxia | VGCC | Possible coexistence of LEMS | SCLC | Poor | Not tested | 16 |
mGluR1 | Remote history of Hodgkin lymphoma | Hodgkin lymphoma | Yes | In vivo | 3 |
The frequencies given depend on reported cases. Many cases are being diagnosed but are not reported.
Ab, antibody; AMPA, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid; AMPAR, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor; CASPR2, contactin associated protein 2; GABABR, gamma-aminobutyric acid B receptor; GlyR, glycine receptor; LE, limbic encephalitis; LEMS, Lambert-Eaton myasthenic syndrome; LGI1-Ab, leucine rich glioma inactivated 1 protein antibody; mGluR, metabotropic glutamate receptor; NMDA, N-methyl-D-aspartate; NMDAR, N-methyl-D-aspartate receptor; PERM, progressive encephalomyelitis with rigidity and myoclonus; SCLC, small cell lung cancer; VGCC, voltage gated calcium channel.