Abstract
Background Autoimmune encephalitis (AE) is the third most common encephalitis in children. Diagnosis
can be challenging due to overlapping and diverse clinical presentations as well as
various investigation results. This study aims to characterize the clinical, diagnostic
features, as well as treatment and outcomes of AE in children and determine the incidence
of pediatric AE in Latvia.
Methods The study was conducted at the Children's Clinical University Hospital in Riga. The
study participants were patients under the age of 18 years diagnosed with AE from
2014 to 2022. Data regarding clinical characteristics, investigation findings, treatment
strategy, and outcomes were retrospectively collected from the medical history data
system.
Results We included 18 pediatric patients diagnosed with AE. The mean incidence of pediatric
AE in Latvia was 0.56 per 100,000 children. Most patients (66.6%) had seronegative
AE. In the seropositive group, the most common was anti-methyl-D-aspartate receptor
AE, with two patients having other antibodies. The most prevalent clinical features
were personality change, cognitive impairment, autonomic dysfunction, and movement
disorders. The majority of patients (58.8%) received first-line treatment only. More
than half (55.6%) of our AE patient group had long-term sequelae.
Conclusions Our study shows that the pediatric AE incidence in Latvia is similar to what has
been previously reported in other studies. A relatively high proportion of seronegative
AE was present in our cohort, indicating that awareness of possible misdiagnosis should
be raised. Further research is needed to better understand the underlying mechanisms,
characterize clinical features, and determine the treatment of choice in different
situations to improve long-term outcomes.
Keywords
autoimmune encephalitis - NMDA - pediatric AE incidence
