Clinical spectrum, etiology, and treatment response in neonatal autonomic seizures: A case series

Abstract

Background

Neonatal autonomic signs such as apnea, cyanosis, pallor, and desaturation rarely occur as isolated ictal phenomena and require a high degree of clinical suspicion in combination with continuous video electroencephalogram (vEEG) to establish a diagnosis.

Methods

We review the clinical profile, etiology, and treatment response in five neonates who presented with apnea as the primary seizure semiology.

Results

Ictal apneic episodes were confirmed on continuous vEEG in all five infants within 24–48 h of symptom onset. Seizure etiologies included structural, infectious, and genetic, including a neonate with ANKRD11-associated KBG syndrome, in which ictal apnea has not been previously described. Acute seizures resolved in all neonates following treatment with a single or combination of antiseizure medications.

Conclusions

Abrupt onset and clustering episodes of apnea and oxygen desaturation in term infants should raise suspicion for epileptic seizures. Genetic testing should be considered as part of the diagnostic evaluation for autonomic seizures of unknown etiology. Early diagnosis and treatment of neonatal autonomic seizures may lead to excellent treatment response in the acute setting.