Familial occurrence of dysembryoplastic neuroepithelial tumor

Dysembryoplastic neuroepithelial tumor (DNT) is a clinicopathologically distinct intracortical lesion that most commonly presents with complex partial seizures often refractory to antiepileptic drugs.1–3⇓⇓ Although some evidence suggests a dysembryoplastic origin of these benign tumors, the factors contributing to the development of DNT remain elusive.1 In particular, genetic and molecular mechanisms underlying the tumorigenesis of DNT are entirely unknown. We report a 5-year-old girl and her father, in whom DNT was confirmed histopathologically. ### Case 1. A 5-year-old girl sought treatment for focal seizures with eye deviation to the right accompanied by nystagmus. On admission, the results of the neurologic examination were normal, except for right-sided hemianopsia. MRI revealed a non–contrast-enhancing mass in the left occipital cortex, which appeared hypointense on T1-weighted images. The tumor was …