Retrospective Comparison of Patients Evaluated for Pediatric Autoimmune Encephalitis with Typical and Atypical Premorbid Neuropsychiatric Development.

PURPOSE: Patients with neurodevelopmental disorders (NDD) (i.e. autism, developmental delay, early-onset psychiatric or seizure disorders) increasingly seek evaluation of new or exacerbated symptoms concerning for autoimmune encephalitis (AE). Clinical AE evaluation can be challenging in NDD patients with symptom overlap between anti-neuronal autoimmunity and baseline atypical neurodevelopment. This study sought to explore differences in AE features by neurodevelopmental status. METHODS: This retrospective chart review included 67 children with typical development (TD) or NDD evaluated for AE at the authors' institution. AE diagnosis included seronegative AE or seropositive AE with anti-NMDAR or anti-GAD antibodies. Reported AE clinical domains, symptom onset acuity, and treatment response were compared between three groups: (1) TD children with AE (TD-AE, N = 24); (2) NDD children with AE (NDD-AE, N = 21); and (3) NDD children with a non-AE diagnosis following appropriate workup (NDD-nonAE, N = 22). RESULTS: Children with AE had a greater number of reported clinical domains than non-AE children with NDD (p < 0.0001) regardless of baseline developmental status. There were no observed differences in reported domains between TD-AE and NDD-AE groups. Onset acuity differed across the three groups (p = 0.04). No treatment response differences were observed between groups. CONCLUSION: NDD children with AE had a comparable number of reported clinical domains relative to TD children and a similar treatment response. NDD patients with AE had a greater number of reported clinical domains than their NDD peers without an AE diagnosis. These findings suggest that AE is a multi-domain process in both TD and NDD children.

Duke Scholars

Author Reginald (Gino) Lerebours  

Author Heather Ann Van Mater Pediatrics, Rheumatology