PMC4907789_01
Sleep EEGs of the patient at different timepoints.. A. The first EEG at 3 weeks of life showed a good background organization with no clear epileptiform abnormalities.. B. At the age of 2 months, sleep and awake EEG continued to show a good background organization. Very rare and isolated spikes appeared only during sleep on the left temporal region (T3).. C. At the age of 5 months, the EEG pattern begins to change. Epileptiform abnormalities now become sustained and multifocal, with an initial tendency to spread, at least in the same hemisphere. This kind of EEG pattern might be considered as a "point of no return", after which, a progressive EEG deterioration with a significant increase of epileptiform abnormalities is likely to occur in a few weeks.. D. Significant evolution of the EEG pattern, which now shows recurrent and sustained epileptiform abnormalities with spikes and spike and waves with a tendency to spread with a pseudo-periodism.
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During the first evaluations, his neurologic examination and the wakefulness and sleep video-EEG were normal for his age (Fig. 1A). At the age of 2 months, some isolated sharp waves and spikes appeared on the left temporal region during sleep (Fig. 1B); this epileptic focus appeared to be in topographic concordance with a large left temporal tuber seen by MRI. At the age of 5 months, the epileptiform abnormalities became multifocal, and bilateral temporoparietal slow waves were also recorded (Fig. 1C). EEG was more active, showing recurrent multifocal slowing and epileptiform abnormalities, with a pseudo-periodism (Fig. 1D). Epileptiform abnormalities became more frequent and often generalized, with epileptic foci mainly localized in the right hemisphere.
This chart shows the developmental age of the child over time. After the first evaluation at the age of 6 months, he never appeared to develop according to his chronological age, and after 1 year without significant acquisitions, he significantly lost many abilities.
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After the age of 8 months, a gradual deceleration of the developmental trajectory was observed, confirmed by a decrease in DQ values (99 at 6 months, 71 at 12 months, and below 50 at 18, 24, and 30 months of age) reflecting a lack of acquisitions with a minimal increase of developmental age over time up to the age of 24 months followed by a severe regression (Fig. 2).