Clinical features and outcomes in children with seronegative autoimmune encephalitis

• Published in: Developmental medicine and child neurology Vol. 66; no. 10; pp. 1310 - 1318
• Main Authors: Madani, Jihan, Yea, Carmen, Mahjoub, Areej, Brna, Paula, Jones, Kevin, Longoni, Giulia, Nouri, Maryam Nabavi, Rizk, Tamer, Stewart, Wendy A., Wilbur, Colin, Yeh, E. Ann
• Format: Journal Article
• Language: English
• Published: England 01-10-2024
• ISSN: 0012-1622; 1469-8749; 1469-8749
• DOI: 10.1111/dmcn.15896

Aim
To characterize the presenting features and outcomes in children with seronegative autoimmune encephalitis, and to evaluate whether scores at nadir for the Modified Rankin Scale (mRS) and Clinical Assessment Scale for Autoimmune Encephalitis (CASE) or its paediatric‐specific modification (ped‐CASE) are predictive of outcomes.
Method
This observational study included children younger than 18 years of age with seronegative autoimmune encephalitis. Demographics and clinical data were collected. The mRS and CASE/ped‐CASE scores were used to evaluate disease severity. Descriptive statistics and logistic regression were used for data analysis and to evaluate associations between scale scores and outcomes.
Results
Sixty‐three children were included (39 [62%] females, median age 7 years, interquartile range [IQR] 4 years 1 months–11 years 6 months), with follow‐up available for 56 out of 63 patients (median follow‐up 12.2 months, IQR 13.4–17.8). The most frequent presenting neurological manifestation was encephalopathy (81%). Median CASE/ped‐CASE and mRS scores at nadir were 12.0 (IQR 7.0–17.0) and 1.0 (IQR 0–2.0) respectively. Thirty‐three patients (59%) had persistent neurological deficits at follow‐up. Both scoring systems suggested good functional recovery (mRS score ≤2, 95%; CASE/ped‐CASE score <5, 91%). CASE/ped‐CASE score was more likely than mRS to distinguish children with worse outcomes.
Interpretation
Children with seronegative autoimmune encephalitis are likely to have neurological deficits at follow‐up. CASE/ped‐CASE is more likely to distinguish children with worse outcomes than MRS.
What this paper adds
Encephalopathy was the most common reason for presentation in children with seronegative autoimmune encephalitis.
Children with seronegative autoimmune encephalitis are likely to have neurological deficits at follow‐up.
The Clinical Assessment Scale for Autoimmune Encephalitis or its paeditric version at nadir was better at distinguishing children with poor outcomes than the Modified Rankin Scale.
What this paper adds
Encephalopathy was the most common reason for presentation in children with seronegative autoimmune encephalitis.
Children with seronegative autoimmune encephalitis are likely to have neurological deficits at follow‐up.
The Clinical Assessment Scale for Autoimmune Encephalitis or its paeditric version at nadir was better at distinguishing children with poor outcomes than the Modified Rankin Scale.
Presenting features and outcomes in children with seronegative autoimmune encephalitis.
This original article is commented by Titulaer and Neuteboom on pages 1267–1268 of this issue.