Syuzanna Bzhemukhova
V.F. Voyno-Yasenetsky Scientific and Practical Center of Specialized Medical Care for Children, Russian FederationPresentation Title:
Modern approaches to the diagnosis of autoimmune encephalitis in pediatric practice
Abstract
General Characteristics of Autoimmune Encephalitis (AE) in Children:
- AE is a group of heterogeneous, non-infectious, immune-mediated inflammatory diseases of the CNS.
- Key characteristic: diverse neuropsychiatric symptoms associated with antibodies against neuronal and glial proteins, receptors, and ion channels.
Epidemiology and Relevance of the Problem:
- According to recent data, the prevalence of AE may reach up to 13.7 cases per 100,000 population, which is comparable to the incidence of infectious encephalitis.
- In recent years, there has been an increase in AE detection rates, linked to the discovery of new antibodies and advancements in diagnostic methods.
Main Diagnostic Challenges in Pediatrics:
- Lack of approved clinical guidelines for AE specifically tailored to the pediatric age group.
- A significant proportion of patients (up to 60%) show no abnormalities on brain MRI.
- Seronegative forms exist (up to 40% of cases), where specific antibodies are not detected in serum and Cerebrospinal Fluid (CSF).
Diagnostic Approach (Illustrated by a Case of Autoimmune Psychosis):
- The diagnostic algorithm for acute psychotic disorders (onset within less than 3 months) includes:
- Neuroimaging (brain MRI).
- Electroencephalography (EEG).
- CSF examination (clinical analysis, search for oligoclonal bands, infectious markers).
- Blood test for antineuronal antibodies.
- Cancer screening to rule out a paraneoplastic origin of encephalitis.
The Key Role of Laboratory Diagnostics:
- Detection of specific antineuronal antibodies (in serum and/or CSF) is central to confirming the diagnosis and defining the specific type of AE.
- In the clinical example, a 9-year-old girl was found to have a positive anti-MOG status.
Clinical Case: A 9-year-old Girl with Anti-MOG Encephalitis:
- History: Acute onset with seizure activity and cognitive decline. A brain tumor (protoplasmic astrocytoma) was initially suspected and operated on, but histological re-evaluation did not confirm it. There was no response to radiation therapy.
- Neurological Status: Cognitive deficit (short-term memory loss), psycho-emotional agitation, echolalia, mild right-sided pyramidal signs.
- Key Findings: Video-EEG Monitoring (VEEGM) showed slowing in the left parieto-central region. CSF analysis revealed antibodies to MOG (myelin oligodendrocyte glycoprotein).
- Treatment and Outcome: Pulse therapy with methylprednisolone was initiated. Follow-up MRI showed positive dynamics after treatment.
Modern Trends in Treatment and Scientific Prospects:
- The cornerstone of acute therapy consists of immunosuppressive drugs (corticosteroids, intravenous immunoglobulins - IVIg, plasmapheresis).
- Groundbreaking scientific work (Nobel Prize 2025) studying the role of regulatory T-cells in immune response control has been noted. These discoveries pave the way for the development of more targeted and safer therapies for autoimmune diseases, including AE.
Conclusion:
Despite progress in diagnostics (discovery of new antibodies), establishing a reliable diagnosis of AE in children remains a challenging task. This is due to the lack of pediatric-specific guidelines, the frequent absence of MRI changes, and the high proportion of seronegative forms.
Biography
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