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Septo-optic dysplasia with schizencephaly

Case contributed by Heba Abdelmonem
Diagnosis almost certain

Presentation

Right side weakness

Patient Data

Age: 50 years
Gender: Female

Grey matter lined CSF cleft extending from the right lateral ventricle to the right frontotemporal cortex, finding consistent with open lip schizencephaly. The relatively small size of the right cerebral peduncle suggests Wallerian degeneration.

The following subsidiary findings are seen as well (absent septum pellucidum, "point down" appearance of the lateral ventricular frontal horns on coronal images, small pituitary gland, and the stalk is not clearly seen).

Abnormal high signal of the periventricular white matter as well as multiple scattered foci of FLAIR high signal of the deep white matter consistent with small vessel ischemia.

Annotated image shows the "point down" appearance of the lateral ventricular frontal horns on coronal image.

Case Discussion

In patients with septo-optic dysplasia associated with schizencephaly, the optic apparatus is less severely affected.

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