Clinical features of autonomic dysfunction in children with anti-N-methyl-D aspartic receptor encephalitis.

Abstract

Background: Anti-N-methyl-D-aspartic receptor encephalitis (Anti-NMDAR encephalitis) is the most prevalent form of autoimmune encephalitis in pediatric patients. Autonomic dysfunction is a frequent symptom of Anti-NMDAR encephalitis, yet it often goes unnoticed by pediatricians. Studies have indicated that pediatric patients with autonomic dysfunction exhibit a poorer prognosis compared to those without. To date, research on autonomic dysfunction in encephalitis has predominantly focused on adults, with no studies conducted on pediatric populations. This analysis examines the clinical features of pediatric patients with Anti-NMDAR encephalitis complicated by autonomic dysfunction.

Methods: We performed a retrospective analysis of patients diagnosed with Anti-NMDAR encephalitis at the Department of Neurology, Children's Hospital affiliated to the Capital Institute of Pediatrics, from June 2017 to June 2023. Patients were categorized based on the presence or absence of autonomic dysfunction during their illness. We summarized and compared the clinical features of children with autonomic dysfunction and analyzed the risk factors for its development in pediatric Anti-NMDAR encephalitis patients.

Results: A total of 56 children were included in this study. Twenty-two (39.3%) exhibited autonomic nervous dysfunction. The most prevalent symptom of autonomic dysfunction was cardiovascular autonomic dysfunction(21/22, 95%),with the specific manifestations being sinus tachycardia (8 cases), ventricular premature beats (2 cases), atrioventricular block (2 cases), atrial premature beats (3 cases), and sinus bradycardia (4 cases),hypertension(1 case) and cardiac arrest(1 case). Other symptoms included gland secretion dysfunction (19/22, 86%),ventilate dysfunction(3/22,14%), thermoregulatory dysfunction (3/22,14%), bladder dysfunction(2/22,9%). Compared to the group without autonomic dysfunction, the group with dysfunction showed significantly higher rates of prodrome infection, tumor complications (all ovarian teratoma), consciousness disturbance, elevated cerebrospinal fluid protein, initiation of second-line and long-term immunotherapy, length of hospital stay, and hospitalization costs (P < 0.05).

Conclusion: Among pediatric patients with Anti-NMDAR encephalitis, cardiovascular autonomic dysfunction is the most common form of autonomic dysfunction. Those with autonomic dysfunction have a worse prognosis and longer hospital stays. Active initiation of second-line and long-term immunotherapy is recommended.