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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dysembryoplastic neuroepithelial tumor is a recently described but rare tumor that occurs in children and characterized by long-standing, intractable partial complex seizures. Due to a paucity of literature on this condition and its heterogeneous cellular composition, dysembryoplastic neuroepithelial tumors can present difficulties in diagnosis. The authors describe two cases of dysembryoplastic neuroepithelial tumor occurring in young patients (ages 8 and 19 years). Both tumors were located in the temporal lobe. Temporal lobectomy with excision of mesial structures resulted in resolution of the seizures. Differential diagnosis includes oligodendrogliomas, mixed gliomas, and gangliogliomas. Features of the dysembryoplastic neuroepithelial tumor that are useful in making the distinction 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 accompanying cortical dysplasia, and the lack of an arcuate vascular pattern. Because dysembryoplastic neuroepithelial tumors are curable by excision, the recognition and correct diagnosis of this tumor is important.
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PMID:Dysembryoplastic neuroepithelial tumor. 154 64

The authors report a case of dysembryoplastic neuroepithelial tumor which is a new entity of glial tumor proposed by Daumas-Duport et al. A 16-year-old male was admitted to our hospital with a 5-year history of uncontrollable complex partial seizure. CT scan showed a non-enhanced homogeneous low density area without mass effect, simulating old infarction or porencephalic cyst in the right posterior temporal lobe. The inner table of the skull over the lesion was eroded. The lesion showed low signal intensity in T1 weighted MR image and high signal intensity in T2 image. Craniotomy disclosed greyish soft solid tumor without cyst. Histologically, the tumor contained multiple cellular nodules in the microcystic astrocytic part which contained neurons. After the surgery the patient was free from the seizure. Dysembryoplastic neuroepithelial tumor is found in young patients with intractable partial seizures. It is characterized by pseudocystic well-demarcated low density appearance on CT scan. Histologically, it is an intracortical multinodular heterogeneous tumor which, is surgically treatable with favorable prognosis. For differential diagnosis, this tumor must be recognized in the list of low-density intracranial lesions found during CT scan.
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PMID:[Dysembryoplastic neuroepithelial tumor; a case report]. 165 3

Dysembryoplastic neuroepithelial tumour (DNT) is a newly recognized brain mass lesion with distinctive pathological features and a favourable prognosis. We reviewed the clinical, electroencephalographic, neuroimaging and pathological features of 16 patients with DNT who underwent surgery; only one patient did not have epilepsy. Mean age at seizure onset was 9.5 years (range: 1 week to 30 years) and surgery 17 years (range: 7 months to 37 years). The mean verbal IQ was 94.6 (range: 79-110) and performance IQ 105 (range: 79-130) (n = 10). The EEG was abnormal in all cases reviewed (n = 13): localized slow activity was seen in 12 and interictal spiking in 10 patients, being less extensive than or concordant with the lesion in three and more extensive than or distant to the lesion in seven. X-ray CT was normal in three out of 11 patients. Magnetic resonance imaging provided detailed anatomical information: the lesion was predominantly intracortical, although in six patients, there was also white matter involvement. The lesion involved the temporal lobe in all but one patient where it was in the cingulate gyrus. Of the temporal lobe cases, MRI showed that the lesion involved, or was in close proximity to, mesial temporal structures in 11 out of 14 patients. Other magnetic resonance features included: circumscribed hyperintensity on long TE/TR images (10 patients), hypointensity on short TR images (12 patients), and cyst formation (five patients). Calcification was seen on CT in four patients. Post surgical follow-up ranged from 8 to 30 months (mean 16.2 months): 12 patients are seizure free and two have a > 80% reduction in seizure frequency (n = 14). Histopathological characteristics included a heterogeneous composition in all cases, calcification (13 cases), dysplastic features (12 cases) and isolated foci of subpial spread (five cases). The presence of occasional mitoses in 12 cases and immunoreactivity to the proliferating cell nuclear antigen in six cases indicate that these lesions have cellular proliferative activity and that there may be a need to follow these patients postoperatively.
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PMID:Dysembryoplastic neuroepithelial tumor. Features in 16 patients. 803 57

Dysembryoplastic neuroepithelial tumour (DNT) represents a morphologically unique and surgically treatable benign lesion typically associated with complex partial seizures (CPS). Although the radiological features are not pathognomonic the histology is quite distinct. They may account for a significant minority of children with intractable CPS. We present four histologically proven cases to demonstrate and discuss the range of radiological features. Recent recognition that CPS may be caused by a number of conditions including DNT emphasizes that all children with partial seizures should have radiological investigations early in their course.
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PMID:Dysembryoplastic neuroepithelial tumour as a potentially treatable cause of intractable epilepsy in children. 849 72

Dysembryoplastic neuroepithelial tumor (DNT) in a newly proposed mixed neuroglial tumor in the cerebral cortex. However, DNT associated with phacomatosis has mostly been considered exceptional. In this paper, a case of DNT associated with neurofibromatosis type 1 is reported. A 23-year-old male was admitted to our hospital complaining of intractable complex-partial seizure. He had a history of neurofibromatosis type 1 (NF - 1) and pituitary dwarfism. On general physiological examination, many cafe au lait spots and freckling could be noted, showing that the case was neurofibromatosis type 1. In addition, neurological examination showed no abnormal findings. MR images revealed a small area of abnormal intensity on the right temporal. This region showed high intensity on T2 weighted image and low intensity without enhancement on T1 weighted image. On electroencephalography (EEG), an epileptic spike focus was demonstrated in the right temporal lobe. So, lobectomy was performed for control of epilepsy. Postoperative course was uneventful and without seizure. On histological examination, the tumor was composed of three different components : specific glioneural element, foci of oligodendrocyte-like cell, and cortical dysplasia. All of these findings were consistent with the definition of DNT by Daumas-Duport et al, except for the association with NF - 1. Although reported cases of DNT associated with FN - 1 are exceptionally rare, both DNT and NF - 1 originate from maldevelopment of the fetal central nervous system. It is very interesting that our case indicated the possibility of co-existence of both diseases.
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PMID:[A case report of dysembryoplastic neuroepithelial tumor associated with neurofibromatosis type 1]. 884 80

Dysembryoplastic neuroepithelial tumor (DNT) is a newly recognized brain lesion first reported in 1988 by Daumas-Duport et al. The authors described five cases of DNT, that occurred in young people and were characterized by partial seizures. Seizures could become intractable and secondary generalised. Usually, the interictal neurological examination was normal. In most cases, computed tomography showed a supratentorial, "pseudocystic" low density appearance associated in some cases with calcific hyperdensity or focal contrast enhancement. Magnetic resonance imaging demonstrated a predominantly intracortical lesion. Common features included low signal intensity on T1-weighted images and high signal on T2-weighted images. Temporal and frontal lobes were mainly involved. Dysembryoplastic origin explained the clinical and radiological stability and the benign evolution of this tumor. Tumor resection was required only when epilepsy was intractable or when there was intracranial hypertension. Earlier intervention can prevent the physical and psychosocial damage resulting from chronic seizures and can improve the prognosis for these young patient. Histologically, DNT have been incorporated amond the category of neuronal and mixed neuronoglial tumors. Three patterns are described: a simple form with a unique glioneuronal element, a complex form with specific glioneuronal element, nodules being made of multiple variants looking like astrocytomas, oligodendrogliomas or oligo-astrocytomas, foci of dysplastic cortical disorganisation, and a non specific form. When specific glioneuronal composant is absent (50% of cases), the identification of DNT has therapeutic and prognostic implications because aggressive therapy may be avoided, sparing these young patients the long term effects of radio-or chemotherapy.
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PMID:[Dysembryoplastic neuroepithelial tumors. A benign tumor cause of partial epilepsy in young adults]. 894 42

Dysembryoplastic neuroepithelial tumor (DNT) is an uncommon congenital tumor occurring in children. We report a 12-year-old boy who had a focal motor seizure involving his left face 6 months prior to admission. Preoperative computed tomography of the brain demonstrated a focal calcified tumor measuring 2.5 cm in diameter in the right temporal lobe. Initial pathological diagnosis was oligodendroglioma, and postoperative radiotherapy with a total dose of 5000 cGy in 28 fractions over 5&1/2 weeks was given. At follow-up 8 months later, neither clinical nor radiological recurrence was found. Because of the benign clinical course, a review of the surgical specimen was made, which led to the diagnosis of DNT. It is important to distinguish DNT from other tumors that have a worse prognosis. Correct diagnosis may prevent unnecessary radiotherapy or chemotherapy. The pathologic and radiologic diagnostic criteria of DNT are discussed, with a review of the literature.
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PMID:Mesial temporal dysembryoplastic neuroepithelial tumor mimicking oligodendroglioma: a case report and review of the literature. 904 72

Dysembryoplastic neuroepithelial tumor (DNT) is a relatively new neuroepithelial neoplasm that was first described by Daumas-Duport et al. in 1988. It was incorporated in the revised World Health Organization classification of brain tumors in 1993. The authors present the first case of DNT reported in the Latin-American literature. A 16-year-old woman had been found at age of five years to have complex partial seizures accompanied by secondary generalization. She was treated with various anti-epileptic drugs but always with incomplete control of seizures activity. Neurological examination was normal. Magnetic resonance imaging demonstrated a lesion in the left temporal lobe. She underwent a craniotomy with complete resection of the tumor. Histological study showed atypical neuroglial proliferation characteristic of DNT. Monoclonal antibody immunostaining for glial fibrillary acidic protein and S-100 protein confirmed the glial component, and neuronal specific enolase and synaptophysin emphasized the neuronal component.
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PMID:[Dysembryoplastic neuroepithelial tumor. Case report]. 962 69

Dysembryoplastic neuroepithelial tumor (DNT) is a recently described rare brain neoplasm with characteristic clinical and morphological features and favorable prognosis. We report here two cases of DNT. The first concerned a 12 years old girl who presented complex seizures preceded by acoustic aura (melodies). Computed tomography revealed a hypodense tumor measuring 2 x 2.5 cm in diameter, located paracortically in the left temporal lobe. The second tumor was removed from a 21-year-old man with partial complex seizures. Nine years earlier patient underwent neurosurgery with partial removal of the tumor The tumor's histopathologic diagnosis is unfortunately lacking. Computed and magnetic resonance imaging showed a mass occupying the cortex and paracortical areas of the anterior pole of the temporal lobe. Histologically, both tumors consisted of small, S-100 protein immunopositive oligodendrocyte-like cells (OLCs) arranged between synaptophysin- and, to a lesser degree, NFP-immunopositive axons (glioneuronal element). In the second case, an area of pilocytic astrocytoma-like appearance was also found, these cells were immunopositive for GFAP. The present study provides clinical, radiological and histological data, which may be helpful in differential diagnosis of this newly recognised brain tumor.
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PMID:Clinical, radiological and histological presentations of dysembryoplastic neuroepithelial tumors (DNT). Report of two cases. 981 23

Dysembryoplastic neuroepithelial tumors (DNTs) were first described in 1988. A DNT is a cortical tumor that produces enlargement of a gyrus, forming a megagyrus that exceeds the normal thickness of the cortex. This tumor is generally seen in young patients with a long-standing history of drug-resistant and disabling seizures mostly of the complex partial type. No neurological deficits are found between the interictal periods in the majority of the reported cases. A general agreement exists over the clinical and radiological manifestations of a DNT as well as the benign biological behavior. Two pathological variants of DNT have been recognized: the single form, composed only of the so-called specific glioneuronal element, and the complex form that additionally shows glial nodules and foci of cortical dysplasia. Nevertheless, and despite the benign biological course, nuclear atypias, cellular monstruosities, foci of necrosis and mitosis can also be found. Surgical eradication of the tumor will usuallly have a good prognosis, without recurrences and with a positive control to seizure-free clinical outcome. Controversy continues to exist over a hamartomatous or a neoplastic origin of this lesion. The DNT has been placed among the neuronal and mixed neuronal-glial neoplasms in the revised World Health Organization (WHO) brain tumor classification, a category that includes the gangliogliomas and the central neurocytomas. Ultrastructural studies and immunostaining techniques may suggest that these three lesions represent different spectrums of the same condition. This paper reviews the most recent publications to offer a better understanding of DNTs and their implications in diagnosis and management.
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PMID:Dysembryoplastic neuroepithelial tumor. 1008 2


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