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Heterotopia - Case 2

A 22 year-old man with mild mental retardation underwent evaluation for intractable complex partial seizures.

Show the Heterotopic Gray Matter                       Note the Possibility of a "Closed Lip" Schizencephaly

Heterotopia: MP-RAGE axial MRIs. Note the presence of the malformed gray matter that extends from the left lateral ventricle to the surface of the left frontal lobe. This is most likely heterotopic gray matter. However, it is also possible that this is "closed lip" schizencephaly wherein the two sides of the cleft have fused together (see the images on schizencephaly, closed lip and open lip). If one hovers over the blue button on the right, one can see how two gyri might have fused together to create such a picture. In either case, this is a disorder of neural migration and was the etiology of the patient's seizures.

Heterotopic gray matter is a developmental malformation wherein some neurons do not migrate to their expected location, instead forming clumps of gray matter, usually in the deep cerebral white matter or lining the walls of the lateral ventricles (periventricular nodular heterotopia). This condition is on the spectrum of neuronal migration disorders. Neurological sequelae may include seizures, mental retardation, learning disabilities and impaired fine coordination.


Revised 11/30/06
Copyrighted 2006. David C Preston