A 13-year-old girl with generalized tonic and grand mal seizures had brain MRI that revealed subcortical band heterotopia (SBH) (figure 1). Twenty-four-hour EEG showed pervasive generalized slow spike and wave pattern (figure 2). Blood DNA confirmed a mutation in the doublecortin (DCX) gene. Interictal 18-fluoro-deoxy (FDG) PET showed reduced FDG uptake (∼10%) in the cerebral cortex and intensely increased uptake (∼20%) in the subcortical band (figure 1), suggesting epileptogenic subcortical band correlating with pervasive slow spike wave pattern on simultaneous EEG. Two PET reports1,2 showed similar or minor increased FDG uptake in the heterotopic band compared to the cerebral cortex.
Figure 1 Brain MRI revealing subcortical band heterotopia
T2-weighted brain MRI (A) shows subcortical band heterotopia. Compared to basal ganglion on a 10% point color scale, FDG-PET (B) shows intensely increased FDG uptake (arrows) by the subcortical band and reduced uptake by the cerebral cortex (arrowheads).
Figure 2 Interictal scalp EEG with generalized slow spike and wave (100–200 mAmp, 1.5–2.5 Hz, in runs of 2–5 seconds) maximum in the parieto-occipital (80%, illustrated in figure) and frontal (20%) regions present during 70% of record
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