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Focal epilepsy associated with dysembryoplastic neuroepithelial tumor in the area of the caudate nucleus

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Abstract

Dysembryoplastic neuroepithelial tumors (DNTs) are usually located within the supratentorial cortex, often in the temporal lobe and they are frequently associated with intractable complex partial seizures. DNTs in extracortical sites are rare. Thus far, 21 cases of 36 DNT-lesions occurring in these areas have been reported; only 8 out of them had epilepsy. We report a case of a 39-year-old woman who had pharmacoresistant epilepsy associated to a DNT in the caudate nucleus–periventricular area treated by lesionectomy. During a 4-year follow-up period, the patient was seizure free and the tumor did not recur. We discuss the hypothetical epileptogenic mechanism involved and we review the pertinent literature.

Introduction

Dysembryoplastic neuroepithelial tumors (DNTs) were first described by Daumas-Duport et al. in 1988 as a typically cortical tumor affecting young patients with long-standing and drug-resistant epilepsy [1].

Usually DNTs are located in the cerebral cortex, most frequently in the temporal lobe [2] whereas extracortical locations are rare. The 4th edition of the WHO classification (2007) reported only 25 cases of extracortical localization and, since then, two further reports have been published [3], [4]. The ectopic DNTs are most frequently found in the septum pellucidum, caudate nucleus, or pericallosal and peri-intraventricular area. Thus far, 21 cases of DNT-lesions occurring in these areas have been reported [3], [4], [5], [6], [7], [8], [9], [10]. Only 8 out of them had epilepsy [3], [5], [6], [7], [9].

Section snippets

Case report

A 39 years old right-handed woman presented with a history of drug resistant focal epilepsy with seizure onset at the age of 33. Seizures occurred in clusters of 3–4 episodes per day every 10–12 days. Interictal EEG and ictal video-EEG recording of seizures were consistent with a right fronto-temporal seizure onset. Antiepileptic treatment with phenytoin, gabapentin, topiramate, oxcarbazepine did not control seizures.

The brain 3 T MRI (Fig. 1) showed a lesion with a pseudo-cystic component

Discussion

DNTs are benign, solid tumors with cystic/microcystic components, located in the cerebral cortex, mostly observed in children and young adults [2]. Only a minority of the reported DNTs occurred outside of the cerebral cortex, DNTs have been reported in the basal ganglia [6], [7], [11], thalamus [11], [12], pons [11], cerebellum [11], [13], [14], and brainstem [15], [16] and midbrain tectum [17].

DNTs may also appear as multifocal lesions with atypical localizations [11], [12], [18]. However, the

Conclusions

In cases of epilepsy associated to a lesion in the caudate nucleus area, extracortical DNT, albeit extremely rare, should be taken into consideration, just in the presurgical and histopathologic time especially in view of the crucial need for avoiding either an invasive presurgical study or an unnecessary and harmful aggressive therapy.

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