Abstract

Background A 39-week (GA) female, with no relevant family history or parental consanguinity, with a fetal ultrasonographic image of an intracardiac tumor. Physical examination was unremarkable. Magnetic-resonance-imaging (MRI) showed subependymal nodules and cortical tubers. Methods and results Eight channel video-EEG was performed four times. 1. EEG recorded within nine days following birth was asymmetric with excessive slow activity over the left hemisphere and isolated sharp waves over the left-central region. Seven rhythmic trains of theta activity evolving to a higher voltage delta and intermixed sharp waves showed left-sided preponderance. Duration ranged from 5–60 s with no obvious clinical correlation. Amplitude-integrated EEG showed persistent electrical seizures over the left hemisphere. Thus antiepileptic drug treatment was indicated. 2. EEG recorded age-30-days showed asymmetry persistence and decrease in subclinical seizure burden (2). 3. EEG recorded age-58-days was again asymmetric, some isolated and pseudoperiodic (1–1.5 Hz) sharp waves over the left- central region, and three subclinical seizures (1–1.5-min duration). 4. EEG recorded at four months of age (wake and sleep stages) showed 4 Hz posterior rhythm in both hemispheres, bilateral sleep spindles. There was slight anterior asymmetry but no seizures. Conclusion Neonatal-onset seizures in TSC patients are infrequent, usually starting at the age of 3rd-5th months. Although EEG recording is not often used as a diagnosis tool, as there may be patients presenting with clinically silent seizures, we encourage the use of video- EEG monitoring. To find seizures in the second week of life was a surprise, but allowed early control of seizures.

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