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Updated: Jul 21, 2026

Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
Clinical Spectrum, Treatment and Outcome of Children with Autoimmune Encephalitis
Mohammad Raza1, Khairunnisa Mukhtiar1, Shahnaz Ibrahim1
1Department of Paediatrics and Child Health, Section of Paediatric Neurology, The Aga Khan University Hospital, Karachi, Pakistan.
Insights
Autoimmune encephalitis (AE) in children often presents with behavioral changes and seizures. While immunotherapy is common, outcomes vary, highlighting the need for early diagnosis and treatment of AE.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Clinical Medicine
Background:
- Autoimmune encephalitis (AE) is a serious neurological condition affecting children.
- Understanding the clinical spectrum, treatment options, and outcomes is crucial for pediatric AE management.
- Key factors include antibodies, such as anti-N-methyl-D-aspartate receptor (NMDAR), immunotherapies, and outcome measures like the modified Rankin Scale (mRS).
Purpose of the Study:
- To evaluate the clinical characteristics, therapeutic interventions, and patient outcomes in pediatric autoimmune encephalitis.
- To identify potential prognostic factors influencing the outcome of AE in children.
Main Methods:
- A descriptive study was conducted reviewing medical records of children diagnosed with AE.
- Data collected included clinical features, treatments administered, and outcomes assessed using a modified Rankin Scale (mRS) at 3-month follow-up.
- Descriptive statistics and logistic regression were employed to analyze prognostic factors associated with outcomes.
Main Results:
- Thirty-three pediatric patients with AE were identified; 39.3% tested antibody-positive, predominantly for anti-NMDAR antibodies (92%).
- Common symptoms included behavioral abnormalities (87.8%), seizures (81.8%), and movement disorders (66.6%).
- Good outcomes (mRS 0-2) were observed in 48.2% of patients, with 12.1% mortality. Chorea, elevated CSF protein, and positive CSF antibodies showed trends towards poorer outcomes but did not reach statistical significance.
Conclusions:
- Early diagnosis of AE is essential in children presenting with behavioral symptoms and chorea.
- While certain factors like chorea, elevated CSF protein, and positive CSF antibodies suggest a higher risk of poor prognosis, statistical significance was not achieved in this cohort.
- Further research with larger sample sizes may be needed to confirm the prognostic value of these factors in pediatric autoimmune encephalitis.
Objective:
To assess the clinical spectrum, treatment, and outcome of children with autoimmune encephalitis (AE).
Study Design:
Descriptive study. Place and Duration of the Study: Department of Paediatrics, The Aga Khan University Hospital, Karachi, Pakistan, from January 2017 to December 2021.
Methodology:
Medical records of children with a diagnosis of AE were reviewed for clinical features, treatment details, and outcomes. Outcome was defined as good (0-2) or poor (3-6) based on a modified Rankin Scale (mRS) score at 3-month follow-up. Descriptive statistics were reported and logistic regression was used to assess the prognostic factors associated with outcome.
Results:
Thirty-three patients were identified with AE. Thirteen (39.3%) were antibody positive. Anti-N-methyl-D-aspartate receptor (NMDAR) antibody was seen in 92% of positive cases. Behavioural abnormalities (87.8%), seizures (81.8%), movement disorders (66.6%), psychiatric symptoms (63.6%), and mutism (33.3%) were the prominent symptoms. Thirty (91%) patients received first-line immunotherapy. Good outcome was seen in 14 (48.2%) patients. Univariable analysis showed that the odds of having poor outcome were 2.5 (95% confidence interval [CI] 0.37-16.88, p=0.34) in patients with chorea. In addition, an elevated cerebrospinal fluid (CSF) protein had an odds ratio (OR) of 8.6 (CI 0.88-84.83, p=0.064) and positive CSF antibodies had an OR of 3.7 (CI 0.79-17.72, p=0.095) for a poor outcome. Mortality was seen in 4 (12.1%) patients.
Conclusion:
A very low threshold is needed for the diagnosis of AE in children presenting with behavioural symptoms and chorea. Although the odds for poor prognosis were higher in patients with chorea, elevated CSF protein and positive CSF antibodies, the p-value did not come out significant.
Key Words:
Autoimmune encephalitis, Antibodies, NMDAR, Immunotherapies, mRS score, Outcome.
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