Schizencephaly

Case contributed by A.Prof Frank Gaillard

Presentation

Cerebral palsy and left hemiparesis and blind in the right eye. Previous CT brain demonstrated hemiatrophy of the right cerebrum.

Patient Data

Age: 25 years
Gender: Female
MRI

MRI brain

Bilateral polymicrogyria is demonstrated involving extensive parts of both cerebral hemispheres.  On the left, it involves the frontal lobe, which has abnormal sulcation, as well as a perisylvian region.  On the right, it predominantly involves the perisylvian region, and is associated with schizencephaly extending to the abnormally shaped trigone of the lateral ventricle, and appearing of the 'close lipped' variety.  

The septum pellucidum is absent, and the optic chiasm is small, particularly on the right, with a markedly atrophic right optic nerve.  

The right thalamus and posterior parts of the caudate nucleus are also markedly reduced in volume, with ex-vacuo dilatation of the adjacent ventricle.  As the fornices pass down towards the mammillary bodies, a region of grey matter intensity is demonstrated which is projecting into the roof of the 3rd ventricle and is not usually present, presumably representing further heterotopic abnormal grey matter. 

The pituitary stalk is present and hypothalamus appears largely unremarkable.  The posterior fossa is normal in appearance.  

Annotated image

Annotated MRI

Closed lip schizencephaly (blue arrows) lined by polymicrogyria (green arrows) which is also present more widely bilaterally. The septum pellucidum is absent (orange arrow) and the optic chiasm and tracts small (yellow arrows) in keeping with septo-optic dysplasia. 

Case Discussion

This case illustrates extensive bilateral polymicrogyria with right-sided 'close lipped' schizencephaly, associated with septum pellucidum absence and optic chiasm abnormality suggests co-existent septo-optic dysplasia, frequently associated with schizencephaly. Of interest, in cases of schizencephaly the degree of optic involvement in septo-optic dysplasia is less severe than when septo-optic dysplasia is found in isolation. 

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Case information

rID: 15806
Case created: 12th Nov 2011
Last edited: 7th Nov 2015
Inclusion in quiz mode: Included

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