Clinical Characteristics and Prognosis of Antibody-Negative Autoimmune Encephalitis in Children: A Single-Center Retrospective Study.

BACKGROUND

Autoimmune encephalitis (AE) is a group of immune-mediated brain diseases. However, new diagnostic criteria for AE in children indicate that partial pediatric patients with AE may be diagnosed without evidence of positive autoantibodies. Therefore, the clinical characteristics and prognosis of children with antibody-negative but probable AE require further investigation.

METHODS

Forty-one children with AE admitted to our hospital from April 2014 to January 2021 were retrospectively enrolled in this study. Children were divided into two groups according to positive or negative antibody tests. Clinical characteristics, cerebrospinal fluid, video electroencephalography, brain magnetic resonance imaging, and prognosis were analyzed, and the correlation between modified Rankin scale (mRS) and neutrophil-to-lymphocyte ratio (NLR) was examined.

RESULTS

Of 41 children, 16 cases tested positive for autoantibodies. The main features were psychiatric symptoms, cognitive disturbances, speech disturbances, movement disorders, and seizures. All the children were given a combination of intravenous methylprednisolone pulses with intravenous immunoglobulin therapy; 26 cases (63%) had a good outcome, and 15 cases (37%) had a poor outcome. Antibody-positive and antibody-negative but probable AE were analyzed by univariate analysis and showed lower lymphocyte counts and higher NLR and mRS scores in the antibody-negative group (P < 0.05). The Spearman rank correlation analysis showed a positive correlation between NLR level and mRS scores (P < 0.05).

CONCLUSIONS

Antibody-negative but possible AE is frequent in children who may have a more severe neurological impairment and higher NLR than antibody-positive AE. Aggressive immunotherapy in antibody-negative AE is essential to achieve a good prognosis.