Hemimegalencephaly

Case contributed by Nick Tarasov
Diagnosis almost certain

Presentation

Seizures

Patient Data

Age: 3 weeks
Gender: Female
mri

The left hemisphere totally enlarged.

The cortex of the enlarged brain is malformed: there is no gray/white matter differentiation, sulci and gyri looks smooth and shallow.

The white matter is also abnormal: T2-hypointense and T1-hyperintense subcortical signals.

Compensatory enlargement of the left ventricle.

Case Discussion

There are typical features of hemimegalencephaly causing intractable seizures since birth.

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