Table 2

Anatomical-clinical syndromes of autoimmune encephalitis

Anatomical classification of autoimmune encephalitisCorresponding clinical syndromesPossible associated antibodies
Limbic encephalitis

  • Cognitive presentation

  • Psychiatric presentation

  • Epileptic presentation

Hu, CRMP5/CV2, Ma2, NMDAR, AMPAR, LGI1, CASPR2, GAD65, GABABR, DPPX, mGluR5, AK5, Neurexin-3α antibodies
Cortical/subcortical encephalitis

  • Cognitive presentation

  • Seizure presentation

PCA-2 (MAP1b), NMDAR, GABA A/B R, DPPX, MOG antibodies
Striatal encephalitis

  • Movement disorder presentation

CRMP5/CV2, DR2, NMDAR, LGI1, PD10A antibodies
Diencephalic encephalitis

  • Autonomic presentation

  • Sleep disorder presentation

Ma 1–2, IgLON5, DPPX, AQP4 antibodies
Brainstem encephalitis

  • Cognitive presentation

  • Movement disorder presentation

  • Cranio-bulbar presentation

Ri, Ma 1–2, KLHL11, IgLON5, DPPX, AQP4, MOG, GQ1b antibodies
Cerebellitis or cerebellar degeneration

  • Ataxic presentation

Hu, Ri, Yo, Tr, CASPR2, KLHL11, NIF, mGluR1, GAD65, VGCC antibodies
Meningoencephalitis

  • Cognitive presentation

  • Seizure presentation

  • Meningeal presentation

GFAP antibody or seronegative AE
Encephalomyelitis

  • Movement disorder presentation including PERM and SPS

  • Spinal presentation

  • Opticospinal presentation

GAD65, amphyphysin, glycine receptor, PCA-2 (MAP1B), GABA A/B R, DPPX, CRMP5/CV2, AQP4, MOG antibodies
Possible associated peripheral syndromes
Neuropathy/neuronopathy

  • Ataxic presentation

  • Sensoriomotor presentation

Hu, PCA-2 (MAP1B), CRMP5, Amphiphysin, CASPR2, CASPR1, CONTACTIN1, NIF155 antibodies
Autonomic neuropathy/ganglionopathy

  • Autonomic presentation

Hu, CRMP5, anti-ganglionic AChR antibodies
Neuromuscular junction dysfunction

  • Myasthenic presentation

VGCC, AchR antibodies
Myopathy

  • Motor presentation

Striational antibodies

  • AchR, Acetyl Choline Receptor; AE, autoimmune encephalitis; AK5, Adenylate kinase 5 Ab ; AMPAR, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor ; AQP4, aquaporin-4; CASPR, Contactin-associated protein-like ; CRMP5, Collapsin response mediator protein 5 ; DPPX, Dipeptidyl-peptidase-like protein 6 ; GABAR, Gamma-Amino butyric acid Receptor ; GAD65, Glutamic acid decarboxylase 65 ; GFAP, glial fibrillary acidic protein; GQ1b, ganglioside Q1B antibody; IgLON5, immunoglobulin-like cell adhesion molecule 5; KLHL11, kelch-like protein 11 ; LGI1, Leucine-rich glioma inactivated ; mGluR1, Metabotropic glutamate receptor 1 ; mGluR5, Metabotropic glutamate receptor ; MOG, myelin oligodendrocyte glycoprotein; NIF, Neuronal intermediate filament ; NMDAR, N-Methyl D-Aspartate Receptor ; PCA2, Purkinje Cell Cytoplasmic Ab Type 2 ; PERM, progressive encephalomyelitis with rigidity and myoclonus; SPS, stiff person syndrome ; VGCC, voltage gated calcium channel.