Encephalopathy Associated With Neurochondrin Autoantibodies

Area of Science:

• Neurology
• Immunology
• Pediatrics

Background:

• Autoimmune encephalitis and epilepsy are increasingly recognized in children.
• Identifying specific neural autoantibodies aids diagnosis and treatment.
• The spectrum of neural antigens targeted by autoantibodies requires further investigation.

Purpose of the Study:

• To determine the prevalence of autoantibodies against established and novel neural antigens in pediatric patients with suspected autoimmune neurological conditions.
• To investigate potential new autoantibody targets in this population.

Main Methods:

• Prospective cohort study of 103 children and adolescents (0-18 years) with suspected autoimmune encephalitis, epilepsy, or epileptiform activity.
• Screening of a panel of 35 autoantibodies against neural cell-surface and intracellular antigens.
• Comparison with 104 age-matched healthy controls.

Main Results:

• Autoantibodies were detected in 15.5% of patients versus 5.8% of controls (P = .02).
• Neurochondrin autoantibodies were identified in 3 patients, with no detection in controls.
• Patients with neurochondrin autoantibodies presented with severe behavioral disturbances, cognitive impairment, and reduced responsiveness, but without seizures and with normal MRI.

Conclusions:

• Autoantibodies against neural antigens are significantly more prevalent in pediatric patients with suspected autoimmune neurological disorders.
• Neurochondrin autoantibodies represent a potential novel target associated with specific severe neurological symptoms in children.
• Further research is warranted to elucidate the role and clinical significance of neurochondrin autoantibodies.