Clinical History: 6-year-old female child presented with first episode of seizure. Developmental history is within normal limits, excepting mild recent deterioration in an academic performance.
Below are the MRI brain images of the Child. First image shows axial T2 image and coronal IR image. The green arrows point to the abnormal band of heterotopic gray matter situated between the layers of white matter (yellow arrows). The finding is more apparent in the coronal IR image.
This finding is called as band heterotopia or double cortex syndrome, which is a neuronal migration anomaly, affecting females and is considered a part of the Lissencephaly type I - subcortical band heterotopia spectrum. Children usually present with refractory epilepsy.
The overlying cortex (orange arrows) shows no pachygyria or polymicrogyria.
The most common genetic abnormality is DCX gene mutation on the long arm of chromosome X, that's why mostly females are affected. LIS1 gene, which is a gene responsible for type 1 Lissencephaly, is affected in some cases.
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