JLE

Epileptic Disorders

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Early and long-term electroclinical features of patients with epilepsy and PCDH19 mutation Volume 20, issue 6, December 2018

Figure 1

Interictal EEG in the first two years. (A) Slowing of background activity with focal spikes and slow waves during a cluster of seizures in Patient 7. (B, C) Temporo-occipital slow waves with superimposed rapid rhythms in Patient 1 and 7 during sleep. (D) An interictal generalized burst of spike-waves in Patient 1 during sleep.

Figure 2

Interictal EEG during follow-up. (A, B) Normal background activity in two patients, distal to seizure clusters, at three years (A) and five years (B).

Figure 3

Ictal EEG. (A) Ictal EEG of a nine-month-old girl (Patient 7) presenting with a focal seizure. Bilateral temporal occipital slow waves are followed by alpha band rapid rhythms, predominating on left temporal occipital areas and rapidly diffusing to the contralateral hemisphere, followed by rhythmic slow waves predominating on the left side. (B) Ictal EEG of atypical absences of Patient 9 with bilateral high-amplitude spike-waves discharges, predominating on the frontal regions. (C) Ictal EEG of an 11-year-old girl presenting with a focal seizure (Patient 5). Right temporal-occipital alpha band with rapid rhythms diffuses into the left hemisphere with rapid generalization, followed by diffuse and synchronous high-amplitude spike-waves at the end of the seizure.

Figure 4

Evolution of the disease depicted as three main stages.