Schizencephaly

Case contributed by Sepehr Haghighi
Diagnosis certain

Presentation

Interactable seizure with developmental delay

Patient Data

Age: 4 years
Gender: Female
mri

Absent of septum pellucidum is visible associated with open lip schizencephaly in left cerebral hemisphere. Polymicrogyria at left frontal lobe along the cleft is noted.

Case Discussion

Schizencephaly is a rare cortical malformation that manifests as a grey matter lined cleft extending from the ependyma to the pia mater.

Schizencephaly can sometimes be bilateral, and is divided into two morphological types:

  • open-lip

    • the cleft walls are separated and filled with CSF

    • most common form in bilateral cases

  • closed-lip

    • the cleft walls are in apposition

    • most common form in unilateral cases

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