Axial T1 FLAIR image in a child with seizures shows a cortic | Figure 1
CincyKidsRad
Cincinnati Children's Radiology
Axial T1 FLAIR image in a child with seizures shows a cortical malformation with polymicrogyria (arrow) and nodular grey matter heterotopia (arrowhead).
Polymicrogyria and grey matter heterotopia are both neuromigrational disorders. Polymicrogyria is most common in the perisylvian region.
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A1 - (a) Subependymal heterotopia
The majority of cases of subependymal heterotopia are sporadic. Some are X-linked. Affected females have mild cognitive deficits. Affected males are spontaneously aborted or have severe deficits. Tuberous sclerosis patients have subependymal nodules, but they are not true heterotopias.
The most common clinical presentation is seizures and developmental delay.
Heterotopias can be diffuse as in this case or focal most commonly in the regions of the trigones or occipital horns.
Differential diagnosis includes tuberous sclerosis. Tubers are usually calcified and have higher T2 signal than normal brain tissue. There are often cutaneous stigmata such as adenoma sebaceum, shagreen patches, or ash leaf spots.
Gray matter heterotopias may be associated with agenesis of the corpus callosum as well as pachygyria, schizencephaly, poly microgyria and Chiari II.
Spectroscopy in grey matter heterotopias demonstrates a decreased NAA/CR ratio, although often mild.