Corpus callosal agenesis and grey matter heterotopia

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

Chronic headaches.

Patient Data

Age: 65 years
Gender: Male

The lateral ventricles are widely spaced and parallel with left-sided colpocephaly. No corpus callosum is present. A nodule of heterotopic grey matter is seen on the right. 

Incidental left-sided staphyloma is also present. 

Numerous congenital abnormalities are identified including complete agenesis of the corpus callosum and a nodular region of grey matter heterotopia extending from just above the trigone of the right lateral ventricle up to an abnormally deep sulcus corresponding probably to the postcentral sulcus although sulcation is abnormal. The sulcus communicates with a prominent interhemispheric  CSF space which is however separate from the ventricles (thus not schizencephaly).  There is marked colpocephaly on the left with marked enlargement of the left temporal horn, which distorts the left hippocampus. A number of hippocampal remnant cysts are noted but otherwise, the hippocampus appears of unremarkable size and signal.

The posterior fossa appears normal as does the brainstem. Incidental left-sided staphyloma is also present.

Case Discussion

It is unusual for significant congenital malformations to be first identified so late in life, however the sign from both grey matter heterotopia and corpus callosal dysgenesis can be minor. 

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