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Band heterotopia

Case contributed by Prashant Gupta
Diagnosis almost certain

Presentation

History of recurrent seizures

Patient Data

Age: 8 years
Gender: Female
ct

The CT scan shows a thickened cortex more so in frontal region, the gyri are reduced in number.

mri

Subsequent MRI showed Subcortical band heterotopia giving rise to a "double cortex" appearance. The overlying cortex shows pachygyria (anterior dominant)

Case Discussion

Band heterotopia is most often found in females, few cases in males have been reported.

Pateints with L1S1 mutations tend to have posterior dominant findings whereas patients with XLIS mutations have frontal dominant agyria/pachygyria.

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