Caregiver-Reported Epilepsy Management in Juvenile-Onset Huntington Disease

Area of Science:

• Neurology
• Pediatric Neurology
• Epileptology

Background:

• Juvenile Huntington disease (JHD) presents distinct symptoms compared to adult-onset HD, with seizures occurring in 30-50% of cases and sometimes as a primary symptom.
• While epilepsy care has advanced, the integration of these developments into JHD management remains unclear.

Purpose of the Study:

• To investigate current epilepsy management practices and the utilization of recent advancements in care for pediatric patients with JHD.

Main Methods:

• An anonymous, caregiver-reported electronic survey was distributed from January 2024 to September 2025.
• Distribution involved patient advocacy groups, providers, and professional organizations via multiple channels including social media and direct communication.

Main Results:

• Twelve JHD patients with seizures were surveyed; 16.7% reported seizures as the initial symptom.
• Staring spells were the most frequent seizure type. Valproic acid was the most prescribed antiseizure medication.
• Both home ambulatory EEG and in-hospital long-term video EEG were utilized, yet no patients were under the care of an epileptologist.

Conclusions:

• Older, high-risk antiseizure medications are still prevalent in JHD care, despite advancements in epilepsy management.
• Long-term video EEG is valuable for distinguishing JHD movement disorders from seizures.
• Further longitudinal studies are essential to guide optimal clinical practices for epilepsy in JHD.