Abstract

A 4-year-old-girl with Rett syndrome (RS) presented with drug-resistant epilepsy since 4 years of age. Her video-EEG captured rhythmic hand tapping with bilateral central spike-and-waves (figure and video 1). Characteristic EEG features in RS include prominent central rhythmic theta activity during wakefulness and needle-like central spikes evoked by contralateral passive finger-tapping. These central spikes may represent exaggerated somatosensory evoked potential secondary to the presence of hyperexcitable motor and sensory cortices. Reflex seizures (focal and atonic) provoked by rhythmic hand pressure have been described in 2 children with RS.1 In contrast, hand clapping has also been reported to suppress continuous centrotemporal spikes.2 Thus, the clinical significance of these provoked central spikes is unclear.

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