Focal cortical dysplasia - bottom of sulcus

Case contributed by Ariel Dahan
Diagnosis certain

Presentation

Refractory focal epilepsy.

Patient Data

Age: 25 years
Gender: Male

A subtle high FLAIR transmantle band extends to the bottom of a small sulcus arising from the right post-central gyrus just behind the central sulcus. This lies 1.5 cm above the Sylvian fissure. No definite cortical thickening or abnormal grey-white matter interface.

Incidental left-sided frontal development venous anomaly.

Conclusion:

Overall, appearances favor a bottom of sulcus dysplasia (at the limit of MRI resolution). The site correlates with the focal region of interictal FDG PET hypometabolism (not shown).

Case Discussion

The patient went on to have epilepsy surgery with resection of this region of cortex.

Histology

Sections show portions of cerebral cortex demonstrating dyslamination and frequent dysmorphic neurons with abnormally distributed Nissl substance, vacuolated cytoplasm and enlarged nuclei. Occasional balloon cells are present with glassy eosinophilic cytoplasm. There is background reactive astrocytosis. A few heterotopic white matter neurons are seen. There is no evidence of a neoplastic process.

On immunohistochemistry, maloriented/dysmorphic neurons are highlighted with NeuN, NF and synaptophysin. Reactive astrocytosis is highlighted with GFAP. The ballooned cells are positive for GFAP, NF and synaptophysin.

Final diagnosis

Right parietal tissue: focal cortical dysplasia, type IIb.

The patient remains seizure free at last follow-up.

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