Presentation
Patient with intractable epilepsy.
Patient Data
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The cortex of the middle and inferior right temporal gyrus is abnormally thickened, with increased subjacent T2 signal in the white matter. The hippocampus is unremarkable in appearance.
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The sections of temporal neocortex and white matter show foci of dyslamination in the middle temporal gyrus associated with fusion of contiguous molecular layers giving the appearances of localized polymicrogyria. Moderate numbers of large neurons are also noted in lamina I and there is disorientation of neurons within the middle laminae of the cortex. No balloon neurons are identified. Moderate numbers of mature neurons are seen in white matter. Myelination appears normal and there are no features of tumor. The features are of malformation of cortical development. The surgical margins are clear of the area of cortical dysplasia.
NeuN = neuronal nuclear antigen
Histology courtesy of Michael Gonzales, Royal Melbourne Hospital.
Case Discussion
Transmantle sign is only rarely seen in Type I focal cortical dysplasia, and usually implies a Taylor type (Type II) malformation.
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