General Child Neurology
Nondominant hereditary ataxias
May. 16, 2022
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A 6.3-year-old girl with a history of a few staring episodes per day. During sleep video-EEG, frequent generalized spike-wave discharges of around 3 Hz, or occasionally fragmented, brief, double-spike or spike-wave generalized discharges lasting less than half a second to 8 seconds, were recorded. In a few of the discharges, her eyes opened slightly and closed again after discharge. On awake video-EEG during hyperventilation, she stopped overbreathing, her eyes opened and stared, and she became unsteady and took a few steps forward within about 1 second from the onset of a generalized spike-wave discharge. Some automatisms with her hands were also present. (Contributed by Athanasios Covanis MD PhD.)