Developmental Malformations
Norrie disease
Aug. 11, 2024
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Tuberous sclerosis complex: multiple bihemispheric potential surgical targets in a child
This 2-year-old girl began seizing shortly after birth and at one point had infantile spasms. After treatment of the infantile spasms, she converted to partial and generalized seizures. The problem was to identify which tubers were the ones responsible for the intractable epilepsy. MRI (A) disclosed multiple tubers over both hemispheres. Scalp EEG showed bilateral interictal discharges. However, magnetoencephalography studies identified one tuber in the left inferior frontal gyrus that generated most of the interictal spike discharges, and an ictal event while in the magnetoencephalography machine was localized to be in the same region. After removal of that single tuber (focal resection), this child has remained seizure-free for over 8 years. Thus, patients with multiple bihemispheric cortical abnormalities may be surgical candidates if the areas generating seizures can be accurately identified. (Contributed by Dr. Gary W Mathern.)