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.

Exp Neurobiol 2024;33:1~17 https://doi.org/10.5607/en23036
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