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Agenesis of corpus callosum with schizencephaly

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Partial epilepsy.

Patient Data

Age: 4 years
Gender: Male

MRI sequences demonstrate complete callosal dysgenesis with parallel lateral ventricles, asymmetrical dilatation of the occipital horns (colpocephaly), absence of the cingulate sulcus, narrow frontal horns, high-riding 3rd ventricle, and widened mid-portion of the interhemispheric fissure.

There is a left parietal parenchymal cleft of CSF signal intensity, extending from the cortex surface to the body of the ipsilateral ventricle, lined by a thickened and irregular heterotopic grey matter. 

Case Discussion

MRI features of complete agenesis of the corpus callosum associated with schizencephaly and polymicrogyria. This association is rarely described in the literature 1.

It is almost always struggled to be dogmatic about open vs closed lip schizencephaly, especially in the absence of a definite continuous CSF along the parenchymal cleft from the cerebral surface to the adjacent ventricular system.

 

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