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Epileptic Disorders

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Semiological study of ictal affective behaviour in epilepsy and mental retardation limited to females (EFMR) Volume 14, numéro 3, September 2012



Figure 1 Family pedigree shows episodes of febrile seizures in the father's line and of febrile and afebrile seizures in the mother's line. The index case reports a de novo mutation of the PCDH19 gene (c.1464_1466del p.Ser489del -p.S489del- mutation). Mutational analysis for PCDH19 in both parents was negative.



Figure 2 EEG tracing. First episode (08:09:55): the fearful behaviour corresponded, on scalp EEG, to a pattern of single, rapid spikes followed by sharp waves, predominant on the left fronto-temporal and right frontal regions (highlighted in the figure). The motor behaviour (08:10:07) coincided with the onset of a diffuse paroxysmal activity (outlined by arrows) with high-voltage spikes that appeared to originate from the left temporal-parietal-occipital regions and that abruptly ceased (outlined by arrow at 08:11:12), followed by a slow activity, predominant on the left hemisphere (highlighted).



Figure 3 EEG tracings. (A) Second episode (08:21:25): the motor behavior (dystonic posture on the right arm) was preceded by a slow, sharp-wave activity, predominant on the left hemisphere (highlighted in the figure), followed a few seconds later by a fast, poly-spike activity (outlined by arrows), originating from the left temporal-parietal regions. At the end of motor manifestation (08:22: 02), there was a slow activity, predominant on the left hemisphere (highlighted). (B) Third episode (08:22:40): the episode of staring and manual automatism (08:22:53) coincided, on scalp EEG, with an activity of slow wave complexes of high amplitude (highlighted in the figure), predominant on the left hemisphere, namely on left temporal-parietal regions.