Subependymal heterotopia

Case contributed by Varun Babu
Diagnosis almost certain

Presentation

New onset of three episodes of partial seizures with secondary generalization.

Patient Data

Age: 20 years
Gender: Female

Brain

mri
  • asymmetry and reduction in volume of right parieto occipital cortex giving a pseudo left hemimegalencephaly appearance.  
  • irregular wavy margins of body of right lateral ventricle. 
  • subependymal grey matter seen lining parallel to the right peritrigonal region with subtle thin linear bands communicating with cortical grey matter (superior temporal gyrus). 
  • no pachygyria agyria complex. 
  • megacisterna magna.   

Case Discussion

Subependymal heterotopias present late in females and usually in the form of new onset partial seizures. Unlike their male counterparts who usually have associated cardiovascular defects and present much early at birth, females present later on in adolescence. 

The accompaniment of megacisterna magna has an association with X linked inheritance. 

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