John Libbey Eurotext

Hypermotor-tonic-spasms seizure sequence related to CDKL5 deficiency disorder: a typical case Volume 24, issue 6, December 2022

The video depicts a nine-month-old female with a typical hypermotor-tonic-spasms seizure sequence associated with CDKL5 deficiency disorder. From a drowsy-quiet state at the onset of the video, the patient suddenly becomes agitated, exhibiting a very brief hypermotor phase with both hips flexed alternatingly. A tonic phase involving all four limbs occurs simultaneously with bilateral anterior quadrant attenuation on the EEG. This lasts for about 12 seconds, followed by the spasm phase, which lasts for almost five minutes (shortened in the video). The patient cries after each spasm in the first half of the cluster. Settings: lowfrequency filter (LFF) at 1 Hz, high-frequency filter (HFF) at 70 Hz, notch ON (60 Hz), timebase at 15 mm/sec, sensitivity at 30 mV/mm.

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