Subcortical DNET in a Patient With an Enzymatic Deficiency: A Rare Case and Review of the Literature.

Purpose: This case report describes a toddler with a medical history of biotinidase deficiency who presented with atypical seizures due to a brain tumor.

Methods: This is a case report.

Results: Electroencephalogram revealed a frontal lobe mass, with magnetic resonance imaging confirmation of a mass extending from the frontal lobe into the genu and anterior corpus callosum. She underwent a near-total resection, and pathology identified a dysembryoplastic neuroepithelial tumor. The patient is now seizure free and clinically doing well.

Conclusions: Children with biotinidase deficiency and atypical seizures should receive a full electroencephalogram evaluation, as brain tumors continue to be on the differential for seizures in this patient population.