Bilateral schizencephaly - septo-optic dysplasia

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Epilepsy

Patient Data

Age: 20 years
Gender: Female

The MRI sequences demonstrate bilateral schizencephaly:

  • on the right, there is an abnormal grey matter (polymicrogyria) extending from the cortex to the body of the lateral ventricle with a slight indentation of the ventricular wall, in keeping with a closed-lip variety.
  • on the left, the parenchymal defect is lined by heterotopic grey matter with polymicrogyria, extending from the cortex surface to the body of left lateral ventricle with a cleft filled by CSF, in keeping with an open-lip variety

 

Absent septum pellucidum with small optic chiasma which may represent an associated septo-optic dysplasia.

Case Discussion

The MRI features are consistent with bilateral schizencephaly, closed-lip on the right, and open-lip on the left with polymicrogyria and associated septo-optic dysplasia.

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