Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dysembryoplastic neuroepithelial tumours (DNTs) are a group of supratentorial cortical benignant lesions that superficially resemble mixed oligo-astrocytomas, oligodendrogliomas or astrocytomas. Clinically these tumours are associated with partial seizures beginning before the age of 20 years, with no neurologic deficit and no stigmata of phacomatosis. In the revised WHO classification, DNTs have been incorporated among the category of neuronal and mixed neuronoglial tumours. This classification describes a histologic variant characterized by the following criteria: cortical location, multinodular architecture--the nodule being made of multiple variants looking like astrocytomas, oligodendrogliomas or oligo-astrocytomas, foci of dysplastic cortical disorganization and the presence of a glioneuronal element showing a columnar structure perpendicular to the cortical surface. A study of 14 cases for which only a specific glioneuronal element could be identified demonstrated that this specific element is sufficient for diagnosing DNTs and that the spectrum of DNTs includes a simple form with a unique glioneuronal element. Preoperative imaging follow-up data, in the series of 23 simple and complex forms, indicated that DNTs are perfectly stable. However, these tumours may show a high MIB 1 labeling index.
 ...
PMID:Dysembryoplastic neuroepithelial tumours. 829 88

The authors report an unusual case of a 11-year-old boy whose supratentorial ependymoma showed rapid growth. He had had generalized convulsive seizures when he was 9 years old. On an initial CT scan a small calcified lesion was identified adjacent to the right sensorimotor cortex. Repeated CT scans showed no interval change in the size of the tumor for 16 months. Then, he suffered an acute onset of left hemiparesis. The neuroimaging studies demonstrated a huge tumor with a large cyst in the right parietal region which had not been observed on CT scan 7 months before. Total removal of the tumor was performed and the histopathological diagnosis was ependymoma with no evidence of malignancy. However, MIB-1 staining of the specimen revealed a high index of 1 proliferative potential up to 25% in some area. The high score of MIB-1 staining correlated well with the rapid clinical course of this histologically benign ependymoma. The small calcified lesion demonstrated on the initial CT scan in this case is considered to have been a low grade ependymoma and to have abruptly transformed into a higher grade, one resulting in rapid enlargement. The authors stress that small intracranial calcified lesions should be carefully followed up by repeated neuroimaging studies at short intervals.
 ...
PMID:[Rapidly enlarging supratentorial ependymoma in a child presenting initially with a small calcified lesion: case report]. 926 64

Four examples of a novel glioneuronal neoplasm are presented. All tumors affected adults (including two males and two females aged 25-40 years) as supratentorial, cerebral hemispheric masses with associated seizure activity and, in one case, symptoms of raised intracranial pressure and progressive hemiparesis. CT scans in two cases revealed hypodense masses without calcification. MRI scans at presentation demonstrated, in all cases, solid T1-hypointense and T2-hyperintense tumors with mass effect in one instance but no edema or contrast enhancement. Only one was relatively circumscribed on neuroradiologic study. All were infiltrative in their histologic growth pattern and predominantly glial in appearance, being composed mainly of fibrillary, gemistocytic, or protoplasmic astroglial elements of WHO grade II to III. Their distinguishing feature was their content of sharply delimited, neuropil-like islands of intense synaptophysin reactivity inhabited and rimmed in rosetted fashion by cells demonstrating strong nuclear immunolabeling for the neuronal antigens NeuN and Hu. These cells included small, oligodendrocyte-like ("neurocytic") elements as well as larger, more pleomorphic forms. Two cases contained, in addition, well-differentiated neurons of medium to ganglion-cell size. Proliferative activity was observed principally within the glial compartment; two cases contained mitotic figures and exhibited relatively elevated MIB-1 indices (6.8% and 8.2%). One of the latter progressed and proved fatal at 30 months following subtotal resection and radiotherapy. The three other patients are alive at intervals of 14 to 83 months, two tumor-free and one with extensive disease associated with the appearance of enhancement on MRI. This glioneuronal tumor variant may pursue an unfavorable clinical course.
 ...
PMID:A distinctive glioneuronal tumor of the adult cerebrum with neuropil-like (including "rosetted") islands: report of 4 cases. 1032 80

Bcl-2 and bcl-xL are proteins known to inhibit cell death (apoptosis). Expression of these proteins in gangliogliomas has not been extensively examined. This study retrospectively evaluates bcl-2 and bcl-x immunostaining in paraffin-embedded materials in gangliogliomas. Twenty-nine gangliogliomas in 17 males and 12 females, age 2.5 to 47 years (mean, 20.7 years), were studied. Nineteen tumors were situated primarily in the temporal lobe. All but three patients presented with seizures ranging from 3 months to 28 years' duration (mean, 11.1 years) before surgery. All tumors histologically were comprised of an atypical neuronal component and a glioma component, which most frequently resembled a low-grade astrocytoma. Cortical dysplasia was observed adjacent to eight tumors. MIB-1 (marker of cell proliferation) labeling indices (percentage of positively staining tumor cell nuclei) ranged from 0 to 7.7 (mean, 0.8). bcl-2 staining was observed in 25 tumors (86%); neuronal staining was present in 24 cases (83%), and glial cell staining in 21 tumors (72%). Bcl-xL staining was only observed in eight gangliogliomas (28%); in all eight tumors (28%), neuronal staining was seen, and focal glial cell staining was present in two cases (7%). Four tumors (14%) did not stain with either bcl-2 or bcl-xL. There appeared to be no relationship between MIB-1 immunostaining and staining with bcl-2 or bcl-xL. bcl-2 expression by immunohistochemistry was observed more frequently than bcl-xL in gangliogliomas. Expression of these proteins may reflect abnormalities of apoptosis, which could play a role in the survival of cells that may be involved in the development of gangliogliomas.
 ...
PMID:Bcl-2 and Bcl-X expression in gangliogliomas. 1037 80

We report the case of a middle aged woman who developed refractory complex partial seizures in her early twenties. She had a right coloboma and neurological examination was normal. Neuropsychological assessments revealed mild impairments of non-verbal memory, and visuospatial and constructional difficulties. Serial CT and MRI showed multiple nodular abnormalities in her mid-brain, diencephalon, subependymal region and both mesial temporal lobes. EEG recording revealed a right medial temporal focus. She underwent en bloc temporal lobectomy. The surgical pathology revealed mesial temporal dysembryoplastic neuroepithelial tumour (DNET) in association with extensive, contiguous neocortical dysplasia. The proliferation marker MIB-1 revealed up to 2% labelling in tumour areas resembling conventional oligodendroglioma. In the two years since surgery she has been seizure free and the remaining MR brain abnormalities have not changed. The very unusual centripetal MRI pattern of discrete multifocal lesions with the characteristic T1 and T2 features of DNET would support the dysembyroplastic germinal origin that was proposed by Daumas-Duport et al. (1988) in their original description of the lesion. The neuroradiological, neuropathological and clinical features of this case are compared with the two previous cases of multifocal DNET.
 ...
PMID:Dsyembryoplastic neuroepithelial tumour with discrete bilateral multifocality: further evidence for a germinal origin. 1104 13

Mixed glioneuronal neoplasms are relatively uncommon tumors in the central nervous system. Recently, an unusual glioneuronal tumor arising in adults marked histologically by neuropil-like islands was described. We present a similar case arising in a 23-year-old woman who presented with headaches and seizures and on imaging studies was noted to have a frontal-temporal lobe mass. The patient underwent partial resection of the tumor, which histologically resembled anaplastic astrocytoma, and received a course of radiation therapy and chemotherapy. Increasing seizure frequency and expanding size on neuroimaging prompted a re-excision of the tumor. The second resection was marked by islands of tissue resembling gray matter with slightly atypical neuronal and glial cells situated in the white matter. These islands stained positively with synaptophysin and did not stain with glial fibrillary acid protein. Mild vascular proliferation and moderate nuclear pleomorphism also characterized the tumor. Areas of necrosis were not noted. A MIB-1 labeling index of 18.1% was noted. P53 immunoreactivity was observed in approximately 40% of tumor cell nuclei. This lesion is felt to represent a clinically aggressive glioneuronal neoplasm with an unusual and distinctive histologic phenotype. HUM PATHOL 31:1435-1438.
 ...
PMID:Glioneuronal tumor with neuropil-like islands. 1111 23

Choroid plexus carcinomas in four dogs (three male, one female) aged small middle 2.5 to 10 years, were examined by light microscopy and immunohistochemistry. The dogs showed progressive neurological signs including ataxia, seizures, vestibular disease and cranial nerve deficits, lasting for several months in some cases. Primary tumours were localized in the lateral (one case), third (one case), and fourth (two cases) ventricles. Hydrocephalus was evident at post-mortem examination in one case. In two cases the neoplastic cells closely resembled the structure of normal choroid plexus, with a distinct papillary pattern, composed of well-differentiated columnar epithelium. In the other two cases, cellular pleomorphism, nuclear atypia, increased mitotic activity and necrosis were observed. In all cases, dissemination of neoplastic cell clusters was detected within the subarachnoid space or the ventricular cavity. Immunohistochemical examination showed a multifocal labelling pattern for pankeratin and cytokeratin AE1 and diffuse vimentin positivity in poorly differentiated tumours. Well-differentiated choroid plexus carcinomas showed multifocal immunoreactivity for cytokeratin AE3, multifocal to diffuse immunoreactivity for vimentin and occasional positivity for carcinoembryonic antigen. Epithelial membrane antigen, Ber EP4 and S-100 were negative in all cases. Glial fibrillary acidic protein labelling occurred only in a single, poorly differentiated tumour. Occasional reactions for proliferating cell nuclear antigen and MIB-1 were seen in two cases. It was concluded that at least two morphological and possibly phenotypic subtypes (well-differentiated and anaplastic) of choroid plexus carcinoma of the dog could be identified.
 ...
PMID:Pathological and immunohistochemical studies of choroid plexus carcinoma of the dog. 1194 7

Granular cell astrocytomas (GCAs) are rare, incompletely characterized infiltrative gliomas that contain a prominent component of granular cells. Such tumors can readily be mistaken for reactive conditions. We studied 22 cases to explore their morphologic spectrum, establish features useful in distinguishing GCA from nonneoplastic diseases, and to determine which parameters correlate with biologic behavior. Tumors occurred in 17 men and five women, ranging in age from 29 to 75 years, who presented mainly with seizures, headache, aphasia, or hemiparesis. Radiologically, high-grade GCAs were contrast-enhancing, cerebral hemispheric masses with prominent peritumoral edema. All contained sheets or interspersed large, round cells packed with eosinophilic, PAS-positive granules. Lymphocytic infiltrates, either perivascular or admixed with neoplastic cells, were present in 14 tumors. Transition to typical infiltrating astrocytoma was noted in 16 cases; of these, granular cells comprised 30-95% of cells. Six tumors consisted almost entirely of atypical granular cells. By WHO criteria, four GCA were grade 2, seven were grade 3, and 11 were grade 4. Glial fibrillary acidic protein staining was seen in all but one tumor, and the majority were immunoreactive for S-100 protein, KP-1, ubiquitin, and epithelial membrane antigen. Although MIB-1 proliferation indices increased with tumor grade, granular cells accounted for only a minority of immunoreactive cells. Among 18 cases with follow-up, 15 recurred after surgery and resulted in death (mean survival, 7.6 months). Two patients died postoperatively, and one was alive at 51 months. Granular cell astrocytoma is an uncommon morphologic variant that appears to be rapidly progressive and usually fatal.
 ...
PMID:Infiltrative astrocytomas with granular cell features (granular cell astrocytomas): a study of histopathologic features, grading, and outcome. 1202 79

Gliofibroma is a rare astrocytic tumor, composed of a glial component ranging from benign to high grade of malignancy and a consistently benign mesenchymal component. Its exact biological behavior is not fully known. In addition, histogenesis and prognostic factors are also still debatable. We herein present a rare case of gliofibroma in a 25-yr-old male with seizure. A computed tomographic scan of the brain showed a 1.5 cm-sized, enhancing mass with calcification. Histologically, the tumor consisted of glial fibrillary acidic protein (GFAP)-positive glial cells admixed with a mesenchymal component and extensive collagen lay down. The glial cells displayed variable cellularity, but without mitosis or necrosis. Since the MIB-1 labeling index was up to 35.8% in the cellular areas of the glial component, it could be considered to be a predictor of worse prognosis.
 ...
PMID:Gliofibroma: a case report and review of the literature. 1292 49

Rhabdoid meningioma (RM) is a recently described, aggressive variant of meningioma. The authors report a case of RM occurring in the resection cavity of an unrelated neurosurgical procedure, temporal lobectomy for intractable seizures. The patient presented with intractable headache 10 years after the temporal lobectomy. Imaging revealed a dura-based, uniformly enhancing lesion within the resection cavity. She underwent gross-total resection and the findings of the surgical pathological report were consistent with an RM, with a dramatically elevated MIB-1 index of approximately 50%. The patient's clinical course was complicated by severe pain and communicating hydrocephalus secondary to rapid dissemination of malignant cells throughout the CSF pathways. Despite aggressive measures, including tumor resection, ventriculoperitoneal shunt placement, and the initiation of conventional radiation therapy, the ensuing leptomeningeal carcinomatosis proved to be rapidly fatal.
 ...
PMID:Rhabdoid meningioma occurring in an unrelated resection cavity with leptomeningeal carcinomatosis. Case report. 1573 68


1 2 3 Next >>