Sîrbu Journal of Medical Case Reports 2011, 5:441 http://www.jmedicalcasereports.com/content/5/1/441 C A S E R E P O R T JOURNAL OF MEDICAL CASE REPORTS Open Access Dysembryoplastic neuroepithelial tumor and probable sudden unexplained death in epilepsy: a case report Carmen-Adella Sîrbu 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. Introduction Dysembryoplastic neuroepithelial tumor (DNT) is a rare low-grade, mixed neuronal and glial tumor, usually asso- ciated with pharmacologically intractable, complex par- tial or generalized seizures which date from childhood. DNTs are heterogenous lesions composed of multiple, mature cell types. Features include a multinodular and multicystic appearance, the presence of both neuronal and glial (oligodendrocytic and astrocytic) components with little if any cytologic atypia, the presence of accom- panying cortical dysplasia, and the lack of an arcuate vascular pattern. The prognosis after surgery is favour- able. Differentiation of DNT from gangliogliomas or other low grade gliomas is possible using magnetic reso- nance imaging (MRI) features and is important because DNT does not recur after epilepsy surgery. Therefore postoperative radiation and chemotherapy are not Correspondence: sircar13@yahoo.com Central Military Emergency University Hospital, “Dr Carol Davila” Department of Neurology, Calea Plevnei 134, Bucharest, Romania needed, and in infancy and childhood they may be dele- terious, so the recognition of surgically curable clinico- pathological entities is mandatory. The presence of secondary generalized seizures, an extratemporal irrita- tive zone and a structural lesion in extratemporal regions correlate with sudden unexplained death in epi- lepsy (SUDEP). Thus, all efforts should be undertaken to eliminate this seizure including abstract epilepsy surgery. Case presentation A 24- year-old Caucasian woman was admitted to our department with refractory epilepsy. Her history included a normal birth and normal psychomotor devel- opment. The seizures started at the age of 11, and were of the complex partial atonic type. The moment of men- tal decline and change of behavior appeared a few months after the onset of seizures. Computer tomogra- phy (CT) showed a left temporoparietal diffuse hypo- dense area, quite inhomogeneous without mass effect © 2011 Sîrbu; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.