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Septal agenesis and lissencephaly with colpocephaly presenting as the ′Crown Sign′

Keywords: lissencephaly , colpocephaly , septal agenesis , crown sign

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Abstract:

Developmental malformations of the cortex in neuronal migration disorders result in constellation of findings on radiological scanning. Isolated defects are common but sometimes they occur in varying degrees of combination giving a unique appearance on the imaging studies. We describe a case of neuronal migration disorder in which the computed tomography scan showed the presence of lissencephaly, colpocephaly. and Septal agenesis. These findings make the ventricular system appear in shape of a crown, which we refer to as "CROWN SIGN", first described in neurosurgical literature.

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