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)

Thirty-four patients have been admitted to our palliative care program since its institution in March 1986. Five were unsuitable and were withdrawn soon after admission. Of the remainder, 22 (75.9%) had central nervous system (CNS) tumors, 5 (17.2%) had myelomeningocoele, 1 (3.45%) had an arteriovenous (AV) malformation, and 1 (3.45%) had a storage disease. Twenty-five (86.2%) have since died and 17 (68%) of these have died at home. In comparison with a similar group of 30 patients studied in a feasibility study prior to the institution of our program, patients admitted to our palliative care program were hospitalized for significantly fewer days during the terminal phase of their illness (p less than 0.05) and a significantly higher proportion died at home (p less than 0.001). Following a survey of the parents of 14 children diagnosed with CNS tumors who died while under our care, we determined that the overall level of satisfaction with the program was high compared to care provided prior to admission to the program (p less than 0.01). Components of the program deemed most satisfactory were (1) being able to care for the child at home (p less than 0.01), (2) having access to a palliative care nurse (p less than 0.05), and (3) having access to a pediatric clinical pharmacologist (p less than 0.05). The most troublesome symptoms occurring in this group of patients were pain, gastrointestinal symptoms, and seizures. Most problems were adequately managed by the parents under the supervision of the team. Eighty percent of the terminal care for these patients was provided at home.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Home-based palliative care for children--Part 2: The benefits of an established program. 169 51

Of 29 consecutive children treated for malignant primary tumors of the central nervous system (CNS) at this institution, postoperative examination showed radiographic or cytologic evidence of neuraxis dissemination in 10 (34%). Given the historically poor results in disseminated CNS tumors treated with surgery and radiation therapy alone, these ten patients were treated prospectively with an investigational Phase II protocol consisting of preirradiation cisplatin (90 mg/m2 on day 1) and etoposide (150 mg/m2 on days 3 and 4). The diagnoses included medulloblastoma (n = 4), malignant glioma (n = 3), cerebral primitive neuroectodermal tumor (n = 1), pineoblastoma (n = 1), and mixed glioma of the brainstem (n = 1). Postoperative neuraxis scanning with computed tomography, magnetic resonance imaging, or spinal myelography showed measurable intracranial or spinal metastases in all children. The cerebrospinal fluid (CSF) cytologic examination was positive for tumor cells in five. The best responses, based on serial imaging of neuraxis metastases, included two complete responses, four partial responses, and three stable disease states. One patient had progressive disease at the primary site despite stable disease in the spine; progressive neuraxis disease was documented in only one patient during chemotherapy. Clearance of tumor cells from the CSF was documented in three patients. The adverse effects of chemotherapy, consisting of transient myelosuppression and mild ototoxicity, were minimal. Reversible neurologic deterioration occurred in two patients; one patient became acutely quadriplegic after a prolonged convulsive seizure without radiographic evidence of tumor progression.
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PMID:Neuraxis dissemination in pediatric brain tumors. Response to preirradiation chemotherapy. 173 73

From the medical records of 238 intensive care unit (ICU) patients who had infections with gram-negative pathogens commonly associated with serious illness, we developed a predictive score of clinical risk factors for seizures. To evaluate the predictive ability of this score, we applied it to a separate population of 645 seriously ill hospitalized patients with similar gram-negative infections who were in antibiotic clinical trials. The patients at highest risk were classified into one of the following three categories: (a) patients with major central nervous system (CNS) insults (CNS surgery, hemorrhage, infection, or other lesion within 1 month before hospital admission or any history of CNS neoplasia), (b) patients with a predisposing factor (renal impairment or a history of seizures) plus a precipitating factor (anoxic encephalopathy/coma or an acute hypotensive episode), and (c) patients with both renal impairment and a history of seizures. Receiver operating characteristic (ROC) curves were calculated in each of the two populations. The area under the ROC curve (AUC) represents the probability that the score would rank a randomly chosen patient who subsequently had a seizure as having had a greater prior level of seizure risk than a randomly chosen patient who did not experience a seizure. The AUC was 0.87 (SE = 0.05) for the original population used to develop the score and 0.81 (SE = 0.04) for the population used for the validation study. The clinical risk score, based on readily available information, provides a useful means to identify among seriously ill infectious disease service patients, those who are at highest risk for seizures. It also serves as a baseline for evaluating the non-drug-related risk factors for seizures in patients treated with antibiotics.
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PMID:Factors predictive of seizures among intensive care unit patients with gram-negative infections. 240 Dec 48

We conducted a population-based case-control study with 338 patients, less than 15 years of age, diagnosed with a primary tumor of the central nervous system from January 1968 through December 1977 in 53 New York State counties. The study also included 676 controls selected from the birth certificate files of the New York State Department of Health. We collected information on neurofibromatosis and congenital anomalies in study subjects, their siblings and parents by telephone interview with the mother of each case and control. We obtained supplemental information on neurofibromatosis in the patients and their families from hospital medical records. This study confirmed the strong association of neurofibromatosis with risk of CNS tumors. Thirteen cases and no controls had neurofibromatosis. Two fathers and 3 mothers of cases had neurofibromatosis. Five cases had siblings with neurofibromatosis. None of the first-degree relatives of controls had neurofibromatosis. We observed a relative risk of 4.49 for history of seizures. Seizures are often among the presenting symptoms for CNS tumors. We observed no difference between cases and controls in the occurrence of congenital anomalies. There was a nonsignificant excess of congenital anomalies among siblings of cases compared with controls. This decreased to 1.13 when adjusted for number of siblings.
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PMID:Neurofibromatosis and other disorders among children with CNS tumors and their families. 249 66

Plasminogen activator inhibitor-1 (PAI-1) is a serpin proteinase inhibitor which regulates fibrinolysis and the proteinase cascade of tumour invasion. In this study, PAI-1 was identified in the cerebrospinal fluid (CSF) from patients without neurological disease and patients with various neurological disorders. The mean level of PAI-1 in the CSF of 28 patients without central nervous system (CNS) disease was 0.28 +/- 0.03 (SEM) ng/ml. CSF PAI-1 was significantly increased in the following diagnostic categories:dementia (Alzheimer's disease), cerebral infarction, CNS infection, alcohol withdrawal seizures and CNS neoplasia. In all these disorders, with the exception of CNS infection, PAI-1 was also increased as a fraction of total CSF protein. CSF PAI-1 was not increased in patients with hydrocephalus or idiopathic seizure disorders. Complementary plasma samples were available for 18 of the 128 CSF specimens studied. For these cases, there was no correlation between plasma PAI-1 and CSF PAI-1 levels. PAI-1 may represent a non-specific marker of disease in the central nervous system.
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PMID:Plasminogen activator inhibitor-1 in the cerebrospinal fluid as an index of neurological disease. 805 48

Gangliogliomas are relatively rare tumors of the CNS in which ganglion cells and neuroglia coexist. 13 cases are reported, representing 0.61% of a series of 2,121 patients undergoing biopsy for CNS tumors. The clinical, radiological, pathological, electron microscopical and immunohistochemical features were evaluated with clinical follow-up. The age of the patients ranged from 3 to 51 years, including 8 males and 5 females. Duration of symptoms prior to surgery varied from 15 days to 12 years, the most common symptoms being seizures, headache, vomiting and motor disturbances. Computed tomographic scans found high density space-occupying mass in 6 patients and low density space-occupying mass in 3. Ten of the tumors originated form the cerebral hemispheres and three from the midline. The tumors were all sharply delineated and well defined from surrounding tissues. The tumor is generally firm. 8 patients are survived operation for 2-10 years. Neurons and glial cells in the tumor could be demonstrated by electron microscopy and immunohistochemical examination. Anaplastic transformation in the gliomatous component was found in one case. This series suggest that surgical removal of this neoplasm carries a good prognosis.
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PMID:[Clinicopathology of gangliogliomas]. 816 79

Primary CNS malignancies are responsible for approximately 12,000 deaths annually in the United States. There has been little change in the outcome for adults with malignant brain tumors over the past few decades, despite improvements in surgical techniques and advances in radiation therapy. These tumors are uniformly fatal one to two years after diagnosis. The morbidity and mortality of this disease arise from the effects of a locally invasive, non-metastasizing lesion. The patients may suffer from seizures, paralysis, incoordination, aphasia, confusion, memory loss, sensory deficits or visual loss, depending on the regions of the brain affected. In addition, they usually require large doses of corticosteroids early and late in their illness, and may experience disabling side effects of this treatment, such as edema, proximal myopathy, diabetes, fungal infections or deep vein thrombosis. Few patients in the older age group are able to work after the diagnosis. Most of the patients are incapable of self-care for several months before death. The localized transfer of new genes into cancer cells potentially permits the expression of proteins with specific biologic functions that may provide a means to alter the biology of tumor growth through a variety of mechanisms including increasing tumor immunogenicity, inducing the local expression of toxic agents, and sensitization of tumors to chemotherapeutic agents. Gene therapy with the transfer of the drug susceptibility gene Herpes virus thymidine kinase (HSV-TK) has shown promise in a number of animal models, including CNS tumors. This study will evaluate the use of adenovirus-mediated transfer of the HSV-TK gene into primary human brain tumors followed by systemic treatment with ganciclovir. The goals of this phase I study are to evaluate the overall safety and efficacy of this treatment and to gain insight into the parameters that may limit the general applicability of this approach. In this phase I study, patients with recurrent gliomas will receive stereotactic-guided injections of the virus into the brain tumor, followed by intravenous ganciclovir for 14 days. Patients eligible to undergo a palliative debulking procedure will receive the same treatment followed by resection on day 7. At the time of resection a second dose of virus will be administered intra-operatively into the residual, unresectable portion of the tumor, and intravenous ganciclovir will be continued for additional 14 days. Tissue removed at the time of resection will be analyzed for evidence of adenovirus infection, thymidine kinase expression and signs of inflammation. The size and metabolic activity of all tumors will be followed by volumetric MRI scans and Position Emission Tomography Scans, respectively. Patients will be enrolled in groups of three, with each group receiving successively larger doses of adenovirus. This study will quantify the toxicity of this therapy, and provide evidence as to the duration of transgene expression and virus induced inflammation.
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PMID:Treatment of advanced CNS malignancies with the recombinant adenovirus H5.010RSVTK: a phase I trial. 884 6

Gangliogliomas account for 1-4% of all pediatric CNS tumors. We reviewed the records of 123 patients treated at the Children's Hospital of Philadelphia between 1974 and 1995. Ninety-nine patients were eligible for review with complete medical records. The mean age was 9.5 years with 52% females. The mean follow-up was 3.7 years. The most common presenting symptom was seizures (49%). The mean length of symptoms to diagnosis was 24.4 months. Complex partial seizure was the most frequent seizure type (60%). Electroencephalograms were abnormal in 36%. The majority of gangliogliomas were in the temporal lobes (38%), with other sites in the parietal (30%) and frontal lobes (18%). All patients had abnormal nonspecific neuroimaging studies. Nine received chemotherapy, and 21 were treated with radiotherapy. Postoperatively, 78% were seizure free on no medication, 18% had improvement of seizure control, and 4% had stable seizure events. Nine died of disease. Gangliogliomas are benign tumors that frequently present with seizure disorders and nonspecific electroencephalographic and neuroimaging studies. Complete surgical extirpation of these tumors provides improved seizure control in most patients and a short course of antiepileptic medical therapy. Adjuvant therapy including radiotherapy and chemotherapy is limited to recurrent disease not amenable to reoperation.
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PMID:Clinical outcome of pediatric gangliogliomas: ninety-nine cases over 20 years. 957 74

An association between central nervous system neoplasm and pregnancy is uncommon although its frequency is underestimated. The most commonly observed tumors are acoustic neuromas, meningiomas and gliomas. The interactions between these tumors and pregnancy is not well known : Pregnancy could affect tumor growth by : - activating tumor growth by stimulating receptors; - increasing the peritumoral edema; - favoring immunotolerance due to pregnancy. Steroid hormone receptors and their therapeutic significance are described here, focusing particularly in meningiomas. Central nervous system tumors do not effect the progress of pregnancy per se but do the therapeutic implications. The clinical aspects are the same as in non-pregnant women. MRI appears to be the most adapted imaging technique. Neurosurgical guidelines are also described, with emphasis on symptomatic therapeutics, especially for seizure. Attitudes concerning the nature of the tumor and time of pregnancy are then discussed. The obstetrical attitude should be therapeutic abortion or delivery, with cesarean if necessary. Prognosis is getting better for the child and mother alike, but still remains poor.
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PMID:[Tumors of the neuraxis and neuraxial sheaths during pregnancy (excluding tumors of the sellar region)]. 1084 48

We report a case of a 44-years-old woman with relapsing-remitting and secondarily progressive form of multiple sclerosis (MS) since aged 24 years, who developed an anaplastic astrocytoma. The neurological manifestations of the tumor were misinterpreted as resulting from MS. Sequential MRI examination and seizures raised the possibility of another nature of her symptoms, besides MS. Her initial good response to high doses corticosteroids led to the initial assumption her symptoms were only exclusively due to the demyelinating process. She underwent craniotomy with radical excision of the lesion. Pathological examination disclosed anaplastic astrocytoma. Other cases of coincidental MS and primary CNS tumors are reviewed, as well as their possible relation.
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PMID:Glioma and multiple sclerosis: case report. 1213 52


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