Dysembryoplastic neuroepithelial tumor and probable sudden unexplained death in epilepsy: a case report

Abstract

Introduction: This is the first report of the case of a patient with a natural history of dysembryoplastic neuroepithelial tumor associated with probable sudden unexplained death in epilepsy. These tumors are benign, arising within the supratentorial cortex. Over 100 cases have been reported in the literature since the first description by Daumas-Duport in 1988.

Case presentation: A 24- year-old Caucasian woman had a long period of intractable complex partial seizures, sometimes with tonic-clonic generalization and neuropsychological abnormalities. Magnetic resonance imaging showed a cortico-subcortical parietal tumor with all the characteristics of these types of tumors. After 14 years of evolution, our patient died suddenly during sleep.

Conclusion: To the best of our knowledge, this is the first case of probable sudden unexplained death in symptomatic epilepsy due to dysembryoplastic neuroepithelial tumor with natural history. Early and complete excision, with functional studies before and during the surgery, leads to better control of seizures, avoiding neuropsychological changes and the risk of death. Patients with refractory epilepsy should be evaluated for any sleep disorders and should have complete cardiology assessments including electrocardiographic evaluation of cardiac rhythm disturbances.

Figure 1

Imaging results. (A) First CT scan show a left temporoparietal diffuse hypodense area, quite inhomogeneous without mass effect. (B- D) MRI performed 13 years after seizure onset revealed a multicystic cortico-subcortical parietal lesion, without edema, mass effect, and enhancement.

Figure 2

EEG showing interictal spikes and polyspikes. One minute of hyperventilation activated a tonic-clonic generalized seizure.