Subcortical heterotopia

Case contributed by Prashant Kandel
Diagnosis almost certain

Presentation

History of seizure under medication.

Patient Data

Age: 20 years
Gender: Male
ct

NCCT shows an ill-defined, isodense area that is continuous with the adjacent cortex in the parieto-occipital region.

Two heterotopic grey matter-lined clefts extend towards the periventricular location in the parieto-occipital region.

mri

There are multiple, nodular, heterotopic grey matter lesions, composed of small gyri noted within the white matter of the right parieto-occipital region which are continuous with the parieto-occipital cortex. They are seen compressing the occipital horn of right lateral ventricle causing deformation of its shape. Post-contrast study shows no abnormal enhancement. It is consistent with subcortical heterotopia.

There are two heterotopic grey matter lined clefts, one measuring ~ 3.0 cm extending from the right parietal cortex and another measuring  ~ 2.8 cm extending from the right occipital cortex, both towards the occipital horn of the right lateral ventricle. No abnormal enhancement is seen in the post-contrast study.

There is a well-defined, oval-shaped, T1 low/ T2 high signal intensity lesion, measuring 1.7x1.2cm is noted within the superior cerebellar cistern. Its suppressed on FLAIR, shows no diffusion restriction on DWI/ ADC and no contrast enhancement is seen on post contrast images suggesting arachnoid cyst.

Case Discussion

Here is a case of subcortical heterotopia in the right parieto-occipital region.

The two heterotopic grey matter-lined clefts in the parieto-occipital region extending towards the peri-ventricular region of the occipital horn of the right lateral ventricle could represent closed-lip schizencephaly.

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