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

We describe the clinical course of a 20-year-old man who suffered generalized convulsive seizures with postictal aphasia and hemiparesis of the right side. Computed tomography (CT) displayed a left postcentral lesion with prominent perifocal edema and only a little contrast medium enhancement. The completely removed tumor proved to be a primary cerebral non-Hodgkin lymphoma consisting of T-cells. Only ten days after the operation the patient once more presented a clinical deterioration. A nuclear magnetic resonance imaging (MRI) displayed an annular structure in the area previously operated upon, suspected to be an abscess. The second operation disclosed a large recurrence of the primary T-cell lymphoma extending diffusely into the white matter. On account of the rapid recurrence, a whole brain irradiation was started twelve days after the second operation. Four cycles of chemotherapy followed. Immunohistochemical studies of the anaplastic large lymphoma cells showed staining with the pan T-cell markers (UCHL1, CD3) and with the CD30 (Ki-1) antibody. The B-cell markers (L26, LN1) were negative. The EMA (epithelial membrane antigen) was only partially expressed. Further investigation excluded the presence of systemic lymphoma manifestation. 24 months after the last operation the patient remained free of symptoms. The last MRI displayed no evidence for the recurrence of a lymphoma. In reference to this unusual clinical course the few previously reported cases of the extremely rare primary cerebral T-cell lymphoma are reviewed.
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PMID:Primary cerebral anaplastic T-cell-lymphoma (type Ki-1): review and case report. 131 14

A case of intracranial fibrous xanthoma (xanthofibroma) is reported. Intracranial fibrous xanthoma in infancy under the age of 1 year is extremely rare. This patient was a 8-month-old boy with a history of convulsive seizure. He had a previously known chest wall tumor which was diagnosed as fibrous xanthoma of the skin. Plain CT scan revealed a well defined high density area in the left temporal lobe. The area was well enhanced with contrast media. At operation, it was found that the tumor did not attach to dura mater and was almost well demarcated. Total removal of the tumor was performed. The patient has been doing well for these 6 months following craniotomy, with no sings of recurrence and no neurological deficits. Histologically, the tumor was composed of fibroblastic cells and foamy phagocytic cells in storiform pattern. Some multinucleated giant cells were found. Immunohistochemistry technique revealed that the tumor cells were negative for GFAP, positive for Vimentin, positive for S-100 protein and negative for EMA. Our studies support the diagnosis of intracranial fibrous xanthoma coexistent with the same tumor found in the subcutaneous space of the chest wall of a boy under 1 year of age. We regard it as a rare incidence. Differential diagnosis and the characteristics of fibrous xanthoma were discussed.
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PMID:[Intracranial fibrous xanthoma (xanthofibroma) in an infant: a case report]. 203 18

EMA (eyelid myoclonia with absences) consists of brief seizures triggered by eyelid closure and characterized by absence and palpebral myoclonia. The EEG shows brief discharges with 3 per second spike and wave complexes. The present report describes the cases of 2 monovular twins who started to have this form of epilepsy at the age of 4 1/2 years. Their seizures, after 2 years of follow-up, are greatly reduced with combined therapy of valproic acid and benzodiazepines (Clobazam).
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PMID:Eyelid myoclonia with absences (EMA) in two monovular twins. 250 35

A case of cerebral meningioangiomatosis in an adult man without any stigmata of neurofibromatosis is reported. A 22 year-old man with no previous neurological history, presented with adversive seizures; clinical examination and electro-encephalogram were normal. CT showed nodular calcified masses in the left frontal lobe and in the left posterior parietal area. A craniotomy was performed and the frontal lesion was excised. Histological examination showed a predominantly intracerebral tumour involving both grey and white matter, with a complex picture of variable cellularity, dense calcification and prominent perivascular arrangements of reticulin-rich spindle cell fascicles with palisade formation widely involving the brain tissue. Immunohistochemistry for S100 protein varied between different parts of the tumour; there was focal positivity for S100 protein and smooth muscle actin in the lesion with no reaction for GFAP or EMA. Immunostaining for Factor 8-related antigen highlighted the perivascular arrangement of lesional cells and demonstrated an increase in the number of small vessels in other areas. Electron microscopy of the main mass showed elongated spindle cells with formation of pericellular basal lamina. The literature on meningioangiomatosis was reviewed. The evidence for meningeal, perivascular neural plexus or pericyte origin does not appear to be well founded. The present case further illustrates the difficulty in identifying an exact histogenetic cell and probably reflects an origin from a primitive perivascular mesenchymal cell.
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PMID:Meningioangiomatosis: a case report and review of the literature. 1061 86

We present 8 examples of a neoplasm with features of both astrocytoma and ependymoma that may represent a distinct clinicopathologic entity. The cerebral hemispheric tumors occurred in patients that were 3, 4, 12, 14, 15, 26, 30, and 37 years of age. All presented with seizures that, with the exception of 2, began in childhood. Magnetic resonance imaging studies showed ill-defined, T2-hyperintense, generally noncontrast-enhancing lesions that, although centered on the cortex or amygdala, extended into the underlying white matter for a short distance. Histologically, the variably infiltrative tumors were distinctively angiocentric with well-developed perivascular pseudorosettes in some cases. Longitudinal and/or circumferential orientations of perivascular cells were common also. The cells were uniform in their cytologic features from case to case and were bipolar in all but one case. A glial nature was inferred from immunoreactivity for GFAP, and ependymal differentiation was suggested by positivity for EMA in three cases and ultrastructural features in one. Overall, the tumors were biologically indolent except for one that recurred and ultimately proved fatal.
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PMID:Monomorphous angiocentric glioma: a distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma. 1621 59

Central nervous system (CNS) involvement is extremely rare in anaplastic large cell lymphoma (ALCL). Primary ALCL of CNS on radiology is often misdiagnosed as tuberculosis. We report a fatal case of primary ALCL of CNS in a 17 year old male. He came with history of headache and left partial seizures. MRI showed a well- circumscribed lesion in the right fronto-parietal lobe eroding the skull bone. Biopsy showed large pleomorphic cells. Immunohistochemical stains showed positivity for CD30, CD43, EMA and ALK-1. In spite of radiotherapy and steroids, patient expired. Hence a high level of suspicion is essential for early diagnosis and for instituting appropriate treatment.
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PMID:Primary anaplastic large cell lymphoma of central nervous system--a case report. 1638 80

We report five cases of sporadic meningioangiomatosis, three males and two females, ranging in age from 12 to 36 years at diagnosis. The lesion was found incidentally by MRI after a head trauma in one case; the other four subjects had a seizure disorders, which improved following surgical resection of the cortical lesions. Grossly, the lesionectomy specimens were of a whitish color and firm consistency. Histological examination revealed that the lesions were confined to the cortex with focal involvement of the overlying leptomeninges, and revealed unifying features of meningioangiomatosis, such as proliferating microvessels with perivascular cuffs of spindle-cell proliferation within the cortex. Two cases had numerous calcifications; one was associated with a prominent fibrocalcifying component. Immunostaining results were variable among the cases. Only vimentin was consistently positive. Some of the spindle cells were weak positive for EMA in two cases. Immunoreactions with anti-CD34 detected within the cytoplasm of the spindle cells were observed in three of the five cases. The Ki-67 proliferation index of all the cases was very low, less than 0.1%. Neurofibrillary tangles were identified in only one of the five cases using the Bodian and immunostaining methods. These findings indicate that meningioangiomatosis lesions show a wide range of clinicopathological features, making diagnosis difficult. A histopathological spectrum and differential diagnoses were discussed with a review of the literature. Since this lesion is a distinct clinicopathological entity and hamartomatous in nature, it is important to make a correct diagnosis in order to avoid further aggressive treatment.
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PMID:Histopathological study of five cases with sporadic meningioangiomatosis. 1677 Nov 83

Dysembryoplastic neuro-epithelial tumors (DNET) are glioneuronal tumors with a common astrocytic or oligodendroglial differentiation. We report a case of DNET with an ependymal differentiation. A 13 years-old girl had refractory focal seizures for 3 years. Successive MRI showed a stable cortical lesion of the right temporal lobe with DNET characteristics. The lesion was resected. Histological examination revealed a pure ependymal differentiation characterized by perivascular pseudorosettes containing EMA immunoreactive cells. Mib1 positive cells were absent. In spite of histologic features of ependymoma, this tumor showed clinical, radiological and behaviour characteristics of DNET. We are not aware of any other case of DNET with ependymal differentiation in the literature. Our observation suggests that the glial component of DNET may show an ependymal differentiation.
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PMID:[Ependymal variant of dysembryoplastic neuro-epithelial tumor]. 1818 61

We report a case of primary dural based osteosarcoma in the right fronto-temporal convexity in a 43-year-old female who presented with a short history of seizure and headache. Radiologic evaluation revealed a well defined brightly enhancing extra-axial lesion in the right fronto-temporal region with a dural tail around the sylvian fissure. The overlying bone was uninvolved. Paraffin section of the tumor showed plump cells with moderate nuclear and cellular pleomorphism with eosinophilic extracellular material (osteiod) between the cells. At a few places, lace like osteiod was seen encasing individual cells signifying osteiod being formed by tumor cells. Immunohistochemistry for EMA was focally positive and negative for S-100 protein and GFAP. A final histopathological diagnosis of dural based primary osteosarcoma of the right fronto-temporal region was rendered. To the best of our knowledge this will be the eighth such case in literature.
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PMID:Dural based primary osteosarcoma in right fronto-temporal region with review of literature. 2022 82

We describe a case of dural angioleiomyoma (ALM) of the middle cranial fossa. A 62-year-old man was referred to our center for fracture of the left clavicle because of a fall, and he had a sudden seizure during admission. The mass was completely resected. The tumor base was located at the bottom of the temporal lobe in the front of the petrous apex and near the cavernous sinus. After 7 months, the postoperative course demonstrated no tumor recurrence. The lesion had the typical appearance of ALM. Mitoses and necrosis were not identified. The lesion contained multifocality of fat in some areas of spindle-shaped cells, and markedly myxoid change was present. The spindle cells were positive for SMA and DES and negative for EMA, HMB-45, p53 and p16. A small focus of fat was positive for S-100. Less than 1% of the tumor cells showed immunoreactivity for Ki-67. EBV-encoded RNA was negative for tumor cells. Stainings for p53, p16, Ki-67 and EBV infection need to be carried out in cases of intracranial ALM because they are correlated with the biological behavior and prognosis of the tumor.
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PMID:Dural angioleiomyoma of the middle cranial fossa: a case report and review of the literature. 2289 40


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