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Table 1 Clinical features of anti-synaptic and anti-glutamic acid decarboxylase autoimmune encephalitides

From: Neuroinflammation and psychiatric illness

Clinical features NMDAR, (NR1 subunit) VGKC-complex (Kv1 subunit, LGI1, CASPR2) AMPAR (GluR1, GluR2 subunits) GABABR (B1 subunit) GAD (65 kDa)
Age (years) 18 to 50 Less than 50 Less than 50 60 to 70 Less than 50
Sex (% female) 75% 66% Greater than 50% 50% Less than 50%
Etiology      
  Paraneoplastic (%, and commonly occurring cancers) 9% to 56% have ovarian teratoma, predominately females less than 18 years of age 10% to 30%; low titers; SCLC; thymoma; CASPR2>>LGI-1 50% to 70%; SCLC, breast carcinoma; thymoma 50%; SCLC thymoma Rarely associated with cancer
  Nonparaneoplastic Approximately 50% 70%; high titers 30% to 50% 50% Frequent
Anatomical subtype      
  Limbic encephalitis Less common Typical Typical Typical Typical
  Panencephalitis Typical Rare (involving basal ganglia) Unclear Unclear Less common
CSF abnormal (%) 90% 40% 90% 80% 20%
Psychiatric Features Common and pronounced: Anxiety, agitation, paranoid delusions, perceptual changes, erratic behavior, speech changes, severe psychosis Agitation, anxiety, panic-attacks, depression, psychosis, hallucinations, delusions, delirium, confabulation Atypical psychosis, which can be isolated Paranoia, behavioral changes Depression, atypical psychosis (case reports)
Neurological Features Early features: seizures, cognitive/memory impairment; Late features: catatonia, orofacial and limb dyskinesia, dystonia, autonomic dysfunction, reduced level of consciousness, aphasia, central hypoventilation LGI1: limbic encephalitis (more common): amnesia temporal lobe seizures, tonic seizures, and hypernatremia. Extrapyramidal symptoms (choreoathetosis) and extra-temporal (faciobrachial dystonic) seizures (less common). CASPR2: limbic encephalitis, Morvan's syndrome (neuromyotonia, REM disorder, insomnia, and autonomic dysfunction). Memory impairment, temporal lobe seizures Prominent temporal lobe seizures, memory impairment, concomitant glutamic acid decarboxylase autoantibodies Stiff-person syndrome, cerebellar ataxia, cognitive/memory impairment, epilepsy (often mesial temporal)
Response to treatment Highly responsive to immune therapy and removal of ovarian teratoma Highly responsive to immune therapy Moderately responsive to immune therapy Moderately responsive to immune therapy Often refractory to immune therapy
Relapse risk 20% often with psychiatric signs; may indicate tumor reoccurrence Rarely relapses Tendency to relapse (based on small case series) Tendency to relapse (based on small case series) Tendency to be chronic and relapse
  1. AMPAR, 2-amino-3-(5-methyl-3-oxo-1,2-oxazol-4-yl)-propanoic acid receptor; CASPR-2, contactin associated protein 2; CNS, central nervous system; CSF, cerebrospinal fluid; GABA B R, gamma aminobutyric acid B receptor; GAD, glutamic acid decarboxylase; LGI-1, leucine-rich glioma inactivated-1; NMDAR, N-methyl-D-aspartate receptor; REM, rapid eye movements; SCLC small cell lung cancer; VGKC; voltage-gated potassium channel.