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J Coll Physicians Surg Pak · Mar 2024
Clinical Spectrum, Treatment and Outcome of Children with Autoimmune Encephalitis.
- Mohammad Raza, Khairunnisa Mukhtiar, and Shahnaz Ibrahim.
- Department of Paediatrics and Child Health, Section of Paediatric Neurology, The Aga Khan University Hospital, Karachi, Pakistan.
- J Coll Physicians Surg Pak. 2024 Mar 1; 34 (3): 323328323-328.
ObjectiveTo assess the clinical spectrum, treatment, and outcome of children with autoimmune encephalitis (AE).Study DesignDescriptive study. Place and Duration of the Study: Department of Paediatrics, The Aga Khan University Hospital, Karachi, Pakistan, from January 2017 to December 2021.MethodologyMedical 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.ResultsThirty-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.ConclusionA 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 WordsAutoimmune encephalitis, Antibodies, NMDAR, Immunotherapies, mRS score, Outcome.
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