Clinical characteristics and immunotherapy efficacy in autoimmune-associated benign epilepsy with centrotemporal...

Benign Epilepsy with Centrotemporal Spikes (BECTS) is the most common form of focal epilepsy in pediatric patients. In clinical practice, immune dysregulation and neuroinflammation have been observed in a subset of patients with BECTS harboring latent Herpesviridae infections, including the Epstein-Barr Virus (EBV) and Human Cytomegalovirus (CMV). Therefore, the present study aimed to explore the correlation between clinical characteristics and immune dysregulation in autoimmunity-associated BECTS and latent viral or Mycoplasma infections.

Fourteen pediatric patients diagnosed with autoimmunity-associated BECTS were prospectively enrolled and underwent assessment of their presentations and etiological and immunological indicators. Further, we evaluated the effectiveness and safety of specific immune therapies (intravenous methylprednisolone and/or intravenous immunoglobulin). Therapy efficacy was determined by a reduction in Rolandic spikes on electroencephalogram recordings and seizure frequency. Potential risk factors were assessed through a retrospective comparative analysis with a control group comprising 46 patients diagnosed with cryptogenic BECTS.

The autoimmunity-associated BECTS cohort demonstrated a higher likelihood of cognitive impairment, ADHD, psychiatric symptoms, and atypical BECTS; required a greater number of anti-seizure medications (ASMs); and experienced longer delays from symptom onset to hospital admission. The prevalence of CMV infection was also found to be significantly higher in the autoimmune disease group than in the control group. Elevated levels of IL-6, IgG, and Complement C3 were observed in the sera of both infected and non-infected children with BECTS. Atypical BECTS and Bilateral Rolandic spikes in EEG were identified as key risk factors for autoimmunity-associated BECTS. Following immunotherapy (intravenous methylprednisolone and/or intravenous immunoglobulin), a substantial reduction in seizure frequency and accumulated spike-wave index (AccSWI) was observed.

These findings support the hypothesis that autoimmune mechanisms contribute to the pathogenesis of selected BECTS cases. Latent viral infections such as CMV may serve as potential triggers. Atypical presentations of BECTS, with Bilateral Rolandic spikes in EEG, indicate the possibility of an autoimmune-associated trigger. Immunotherapy appears to offer therapeutic benefits to patients with autoimmunity-associated BECTS.

This single-center, longitudinal observational study (XH-16-029/NCT02969213) included pediatric patients diagnosed with epilepsy.