Table 1.
Features and antibodies characteristic of autoimmune encephalitis in children and adolescents. The list of antibodies may not be exhaustive, as the field is still young and there are still reports of new associations from the pediatric age range. Abbreviations are spelled out in the list at the beginning of the article
Features | Typical antibodies in the pediatric age range: Anti-… |
---|---|
Syndromes | |
Limbic encephalitis | LGI1, CASPR2, GABABR, GAD65, Hu, DNER |
Encephalopathy (in the sense of a diffuse affection of brain function) | NMDAR, GABABR, mGluR5 |
Brainstem encephalitis | GQ1b |
Cerebellitis/autoimmune cerebellar syndrome | DNER, VGCC, NMDAR, mGluR1a |
Opsoclonus myoclonus syndrome | Hu, GAD65 |
Progressive encephalomyelitis with rigidity and myoclonus | GAD65, GlyR |
Demyelinating diseases | MOG, Aquaporin-4, NMDAR* |
Patient and medical history | |
Girls | GAD65 (NMDAR?) |
Other autoimmune disease | GAD65 |
Epilepsy onset along with prominent psychiatric or cognitive symptoms | LGI1, CASPR2, NMDAR, GABABR, GAD65, Hu, DNER |
Onset with status epilepticus or very high seizure frequency | NMDAR, LGI1, GABAAR, GABABR, GAD65 |
Non-viral secondary disease after viral encephalitis | NMDAR and not further characterized surface antigens |
Encephalopathy after respiratory infection | MOG |
Neoplasm | Onconeural; mGluR5 (Hodgkin disease) |
Paraclinical findings | |
EEG: “extreme delta brush” | NMDAR |
Increased CSF count or autochthonous oligoclonal bands | All except LGI1 |
MRI: encephalitic lesion(s) | LGI1, CASPR2, GABABR, GAD, Hu, DNER |
MRI: demyelination | MOG, Aquaporin-4, NMDAR |
Encephalitic histopathology | All |
aUnpublished own observation
*Overlap syndromes