The list of known autoantibodies and associated clinical features in autoimmune encephalitis
Autoantibody | Associated clinical features |
---|---|
Anti-NMDAR IgG1 | - Encephalopathy with altered consciousness - Psychiatric symptoms like hallucinations, agitation - Seizures & Orofacial dyskinesias - Movement disorders such as choreoathetosis - Autonomic instability including hyperthermia, tachycardia - Coma and central apnea |
Anti-LGI1 IgG4 | - Subacute amnesia and confusion suggestive of limbic encephalitis - Seizures, most notably faciobrachial dystonic seizures - Sleep disturbances - Hyponatremia due to syndrome of inappropriate antidiuretic hormone secretion (SIADH) |
Anti-CASPR2 IgG4 | - Limbic encephalitis with memory deficits - Morvan syndrome, which presents with neuromyotonia, sleep disturbances, and autonomic symptoms - Peripheral nerve hyperexcitability or neuromyotonia alone |
Anti-GABA(B)R IgG1 | - Limbic encephalitis presenting with short-term memory loss, seizures, and psychiatric symptoms - Some cases might exhibit ataxia or opsoclonus |
Anti-AMPAR | - Acute to subacute onset limbic encephalitis - Amnesia, confusion, seizures, and psychiatric/behavioral symptoms |
Anti-DPPX | - Multifocal encephalitis with myoclonus, tremors, and hyperekplexia - Diarrhea and weight loss |
Anti-GlyR IgG1/3 |
- Stiff-person syndrome like muscle rigidity and spasms - Progressive encephalomyelitis with rigidity and myoclonus (PERM) - Limbic encephalitis |
Anti-mGluR5 | - Limbic encephalitis often associated with Hodgkin lymphoma, termed Ophelia syndrome - Presents with memory deficits, seizures, and psychiatric changes |
Anti-MOG | - Neuromyelitis optica (Optic neuritis and transverse demyelinating disease) - Acute disseminated encephalomyelitis |
Anti-IgLON5 IgG1/4 |
- Sleep disorder (REM sleep dysfunction and sleep apnea) - Brainstem signs with bulbar symptoms, speaking and swallowing difficulty - Movement disorder, chorea, dystonia, and parkinsonism gait disturbances - Cognitive syndrome and behavioral disorder |
NMDAR, N-methyl-D-aspartate receptor; LGI1, Leucine-rich glioma-inactivated 1; CASPR2, Contactin-associated protein-like 2; AMPA receptor, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor; GABA, gamma-aminobutyric acid; DPPX, Dipeptidyl-peptidase-like protein-6; mGluR5, metabotropic glutamate receptor 5.