Abnormal brain gamma oscillations in response to auditory stimulation in Dravet syndrome
Auditory evoked potentials
Dravet syndrome
Epileptic encephalopathy
Inhibitory interneurons
Oscillatory gamma activity
Issue Date: 
Sanchez-Carpintero R, Urrestarazu E, Cieza S, Alegre M, Artieda J, Crespo-Eguilaz N, Valencia M. Abnormal brain gamma oscillations in response to auditory stimulation in Dravet syndrome. Eur J Paediatr Neurol. 2020 Jan;24:134-141
Objective: To evaluate the capability of children with Dravet syndrome to generate brain γ-oscillatory activity in response to auditory steady-state stimulation. Methods: Fifty-one subjects were included: 13 with Dravet syndrome with SCN1A gene alterations, 26 with non-Dravet epilepsies and 12 healthy controls. Responses to auditory steady-state stimulation elicited with a chirp-modulated tone between 1 and 120 Hz were collected in subjects and compared across groups. Results: Subjects with Dravet syndrome showed weak or no responses in the 1-120 Hz frequency range. Healthy controls showed oscillatory responses following the frequency of the modulation that were maximal in the low (30-70 Hz) and high (80-120) γ-ranges both, in the power and inter-trial coherence estimates. Non-Dravet epileptic children showed differences in the auditory responses when compared with the healthy controls but were able to generate oscillatory evoked activities following the frequency-varying stimulation. Conclusions: The ability to generate brain γ-oscillatory activity of children with Dravet in response to a chirp-modulated auditory stimulus is highly impaired, is not due to epilepsy and is consistent with the Nav1.1 channel dysfunction affecting interneuron activity seen in Dravet mouse models. Significance: The reported deficits in the brain oscillatory activity evoked by chirp modulated tones in children with Dravet is compatible with Dravet syndrome disease mechanisms and constitutes a potential biomarker for future disease-modifying interventions.

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