Characteristics of children with hypsarrhythmia detected on EEG: A large pediatric cohort


Akıncı Göktaş Ö., Bektaş Ö., Yıldırım M., Öz Tunçer G., Çiçek S., Teber S.

16th European Paediatric Neurology Society Congress, Munich, Almanya, 8 - 12 Temmuz 2025, ss.391, (Özet Bildiri)

  • Yayın Türü: Bildiri / Özet Bildiri
  • Basıldığı Şehir: Munich
  • Basıldığı Ülke: Almanya
  • Sayfa Sayıları: ss.391
  • Ankara Üniversitesi Adresli: Evet

Özet

Objectives We aimed to evaluate the etiology, associated seizure types and treatment of patients with hypsarrhythmia detected by electroencephalography (EEG). Methods This study retrospectively evaluated demographics, treatment modalities, etiologies, associated seizure types, follow-up EEG and magnetic resonance imaging (MRI) findings at the end of a minimum 12-month follow-up of 125 patients with hypsarrhythmia detected on EEG at a tertiary pediatric neurology center from January 2013 to January 2024. Results Comprehensive clinical evaluation and neuroimaging revealed that structural causes were the most common etiology (50.4%). Other causes were as follows: idiopathic (23.2%), both genetic and structural (8%), metabolic (7.2%), genetic (5.6%), infectious (4%) and immunological (1.6%). Epileptic spasms were the most common type of seizure associated with hypsarrhythmia; tonic, myoclonic and focal seizures were also observed to accompany hypsarrhythmia, in that order. At final evaluation, it was noted that all patients received treatment. There was no significant difference in treatment response between Adrenocorticotropic hormone (ACTH) and vigabatrin when used as the first drug. However, in the group using ACTH as a second drug, the response rate was significantly higher at 57.8% compared to vigabatrin and other drugs (p=0.009). Nine patients achieved seizure control with a single medication (7.2%), while 52 patients (41.6%) were considered drug resistant due to persistent seizures despite the use of multiple antiseizure medication. Anti-seizure medication was discontinued in 25 patients (20%). While no association was found between the age of onset of hypsarrhythmia and response to treatment (p=0.160), the shorter the time between detection of hypsarrhythmia and initiation of treatment, the better the response to treatment (p=0.047). In the structural etiology group, the probability of abnormal findings on final EEG was found to be higher than the other groups. Conclusions Patients with EEG-detected hypsarrhythmia are often associated with different types of seizures, mainly epileptic spasms, and almost always require treatment. Although ACTH, which is often the preferred treatment, increases the chances of success, a significant proportion of patients remain in the drug-resistant group. On reviewing repeated EEG recordings of patients, especially in the structural etiology group, even if the hypsarrhythmia disappears on the EEG, various levels of epileptic activity remain.