Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: 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)

In order to investigate the clinical characteristics, neuroimaging findings, pathological features, treatment, and outcomes, and to provide valuable guidance for the diagnosis and management of intracranial gangliogliomas, 34 cases of intracranial gangliogliomas were retrospectively analyzed. This study included 23 males and 11 females. Age at operation ranged from 17 days to 50 years. All patients had preoperative magnetic resonance imaging (MRI). Computed tomography (CT), magnetic resonance spectroscopy (MRS), 18F-FDG-PET (positron emission tomography), and 99Tc-HMPAO-SPECT (single photon emission computed tomography) were also performed in some cases. All pathological specimens and all available neuroimages were re-evaluated. The follow-up period varied from 12 to 89 months (mean 36 months). Seizure was the most common presenting symptom (85%). Tumor calcification was detected by CT scan in six of 11 cases. Seventeen gangliogliomas (50%) showed cystic components and 18 tumors (53%) were enhanced on MRI. All temporal lobe gangliogliomas were located intracortically and most of them had poor demarcation on MRI. In comparison with the contralateral normal area, the gangliogliomas showed a reduced Cho/Cr and NAA/Cr ratio, and an increased Cho/NAA ratio. On 18F-FDG-PET, gangliogliomas were usually hypometabolic. HMPAO-SPECT indicated tumor hypoperfusion or isoperfusion. A gross total resection was achieved in 25 patients. Tumor progression was observed in three patients who underwent an incomplete tumor resection. Two of them underwent a malignant transformation to a glioblastoma. Twenty-seven patients could carry on their normal life activity with the Karnofsky Performance Scale (KPS) of more than 80. Even though ganglioglioma is a slowly growing benign tumor, which could be demonstrated by magnetic resonance spectroscopy (MRS), PET, and SPECT, there is a chance of malignant transformation, especially in cases of incomplete tumor resection. Gangliogliomas should be resected gross totally, if feasible, to achieve the best long-term outcomes.
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PMID:Intracranial ganglioglioma: preoperative characteristics and oncologic outcome after surgery. 1224 Nov 12

This study reports the University of Florida experience treating oligodendrogliomas with the goal of identifying the factors that predict favorable outcome. Between 1958 and June 1998, 51 patients (aged 5-75 years) were diagnosed with pure oligodendroglioma at the University of Florida. Histologic grade was anaplastic in 19 patients, low-grade in 30 patients, and unknown in one patient. Twelve patients were treated with surgery alone (10 with low-grade tumors). Thirty-nine patients underwent postoperative radiotherapy after surgery (plus chemotherapy in four patients). A biopsy was performed in 10 patients, 40 underwent subtotal or gross total resection, and one had surgery of unknown extent. Thirteen patients with a radiographic abnormality consistent with a low-grade glioma had the initiation of definitive treatment (i.e., surgery with or without radiotherapy) deferred for a median of 4.5 years before undergoing treatment for worsening symptoms or radiographic progression. Absolute survival for the entire group (51 patients) at 5, 10, and 20 years from the date of initial treatment was 61%, 37%, and 31%, respectively, and at 5, 10, and 20 years from the date of initial diagnosis was 65%, 43%, and 34%, respectively. No patient died of intercurrent disease. Multivariate analysis for absolute survival from the time of initial treatment demonstrated young age (young patients fared better than older patients) and size (<5 cm better than > or =5 cm) to be significant variables. No patient under the age of 21 years (n = 8) at the time of diagnosis has died in this series. Deferring aggressive treatment did not alter survival. Multivariate analysis for favorable absolute survival based on the time from initial diagnosis demonstrated young age and presentation with seizures alone to be statistically significant for favorable outcomes. Approximately one third of patients with oligodendroglioma appear to be cured with aggressive treatment. Age is the most important predictor of long-term progression-free and absolute survival with young patients (especially aged <21 years) doing much better than older patients. Deferring aggressive treatment (surgery and/or radiation) until symptoms clearly indicate tumor progression does not compromise outcome for a select group of patients who are diagnosed at a young age with a nonenhancing mass on MR and seizures as the lone presenting symptom.
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PMID:Oligodendroglioma: does deferring treatment compromise outcome? 1279 17

Low-grade gliomas are a heterogenous group of diseases characterized by relatively slow-growing primary brain tumors of astrocytic and/or oligodendroglial origin. Many patients present with easily controlled seizures and remain stable for years, whereas others progress rapidly to higher-grade tumors. Several studies have retrospectively investigated tumor-, patient-, and treatment-related prognostic factors in this patient population. Tumor histology, grade, location, contrast enhancement, and molecular markers have been identified as prognostic factors for survival. Likewise, patient age, performance status, and seizure history are patient-dependent prognostic factors. However, although patients who undergo surgical resection and receive adjuvant radiotherapy tend to have improved survival, treatment-dependent prognostic factors have yet to be definitively identified. Recursive partitioning and multivariant analyses have identified a class of patients with good prognosis. Younger patients with good performance status, non-contrast-enhancing tumors (<5 cm), and tumors of oligodendroglial or mixed-oligoastrocytic subtype have improved survival. The European Organisation for Research and Treatment of Cancer has recently developed a prognostic score based on identified prognostic factors to assist in the management of low-grade gliomas. For patients with a favorable (low-risk) score, treatment with radiotherapy or chemotherapy treatment should be withheld until tumor progression. For patients with a high-risk score, treatment at diagnosis may be indicated. However, other than surgery, the optimal types and sequence of therapies are not yet established. Improvements in defining prognostic factors will assist in low-grade glioma management.
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PMID:Prognostic factors for low-grade gliomas. 1476 81

The semi-benign nature of diffuse astrocytomas is characterized by an increased risk for tumor recurrence and malignant transformation. In patients with intractable seizures, however, length of history and clinical follow-up studies indicate a better prognosis of this tumor entity. Here, we present a clinico-neuropathological study of 19 patients with chronic seizures and diffuse astrocytomas. In 6 patients, long-term survival and lack of tumor progression after a maximal follow-up time of 13 years appeared to correlate with a histologically isomorphic phenotype. Cytological hallmarks comprise low cellularity, lack of mitotic activity and highly differentiated astroglial elements infiltrating into adjacent brain parenchyma. Compared to "classical" variants of diffuse astrocytomas (WHO grade II), immunohistochemical reactions revealed a cellular proliferation below 1%, absence of nuclear p53 accumulation, and a lack of glial MAP2 and CD34 expression. Histopathologically, the isomorphic astrocytoma subtype can be distinguished from gangliogliomas, pilocytic astrocytomas and dysembryoplastic neuroepithelial tumors as well as from cortical dysplasia or reactive gliosis. Our data support the concept of a rare variant of diffuse astrocytomas occurring in young adults with long-term epilepsy and a favorable prognosis, which corresponds to WHO grade I.
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PMID:An isomorphic subtype of long-term epilepsy-associated astrocytomas associated with benign prognosis. 1503 26

A prospective Phase II study of temozolomide (TMZ) was conducted in 16 patients with refractory meningioma. All patients had previously been treated with surgery and involved-field radiotherapy; however, no patient had prior chemotherapy. TMZ was administered orally for 42 consecutive days every 10 weeks. Grade 3 or greater TMZ-related toxicity included anemia (25%), fatigue (18.7%), neutropenia (37.5%), seizures (6.3%), and thrombocytopenia (18.7%). No patient demonstrated a neuroradiographic complete or partial response. Time to tumor progression ranged from 2.5 to 5.0 months (median 5.0 months); survival ranged from 4 to 9 months (median 7.5 months).
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PMID:Temozolomide for treatment-resistant recurrent meningioma. 1507 29

Adult patients with a magnetic resonance scan suggestive of a supratentorial low-grade glioma should generally undergo at least a stereotactic biopsy to confirm the diagnosis and rule out an anaplastic glioma or a non-neoplastic lesion. Early tumor treatment should be given to patients with newly diagnosed low-grade gliomas who are over age 50 years, those who have headaches or neurologic deficits other than seizures, or those whose neuroimaging studies show tumor growth or mass effect. For younger patients presenting with seizures and no other neurologic symptoms, it is reasonable to defer therapy until there is clinical or radiographic tumor progression. When it is judged that intervention is necessary, patients should undergo the maximal surgical tumor resection, which preserves or improves neurologic function. For younger (<50 years) astrocytoma patients with a good tumor resection, radiation may be deferred until tumor progression. Early radiation should be given to astrocytoma patients who are older than 50 years of age at diagnosis (regardless of the type of surgery) or to younger patients who are judged to require early intervention but who are not candidates for aggressive surgical resection. The radiation dose for low-grade glioma should be 4500 to 5000 cGy, preferably with three-dimensional conformal ports. The same guidelines for management apply to patients with low-grade oligodendroglioma or oligoastrocytoma, except that chemotherapy is a reasonable alternative to radiation when it is judged that treatment other than surgical resection is required.
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PMID:Low-Grade Gliomas in Adults. 1515 4

At the present time, there are no proven beneficial effects of chemotherapy (CT) for the treatment of pure low-grade astrocytomas. Brain radiotherapy (RT) still remains the standard treatment in order to reduce or delay tumor progression or symptoms, despite possible long-term neurologic complications. We report 10 patients, with histologically proven pure low-grade fibrillary astrocytomas, to which we administered a first-line nitrosourea-based CT. All patients were symptomatic with pharmaco-resistant epilepsy or neurologic symptoms, and had been rejected for neurosurgical resection. All patients with epilepsy had a clinical improvement with reduction in seizure frequency and 60% became seizure-free. CT was well tolerated; all patients developed myelosuppression with 40% of grade III/IV hematotoxicity. Seven were alive at the time of writing with a mean follow-up of 6.5 years (3.5-12) from first recorded symptoms. The three deceased patients died 7.5, 7.5, and 8.5 years from first symptoms. These results demonstrate that some patients with symptomatic non-resectable fibrillary low-grade astrocytomas can be treated with up-front CT to improve their neurologic status. This report suggests that benefits of CT on symptoms, survival, and quality of life should be prospectively compared with RT.
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PMID:First-line nitrosourea-based chemotherapy in symptomatic non-resectable supratentorial pure low-grade astrocytomas. 1612 69

Low-grade gliomas (LGGs) represent a vexing clinical problem. Some patients present with readily controllable seizures and will enjoy years of freedom from tumor progression without intervention, whereas others progress rapidly with eventual neurologic decompensation and death. Both radiation and chemotherapy are helpful to many patients, but the optimal timing and sequencing of these therapies remain unknown. Recent studies have informed our understanding of clinical, histologic, and molecular prognostic factors and help provide guidance as to which patients require early intervention and when observation is feasible or warranted. We review contemporary knowledge regarding prognostic factors, our current evidence-based understanding of the roles and timing of radiation and chemotherapy, and ongoing clinical trials that will further elucidate management of LGGs.
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PMID:Outcome in adult low-grade glioma: the impact of prognostic factors and treatment. 1789 97

Making treatment decisions for patients with infiltrating low-grade gliomas (LGGs) is challenging. Patients frequently present with seizures and usually have little or no neurologic deficit. In this younger and relatively well patient population, despite the potential for significant morbidity, we believe that surgical resection, radiation therapy, and chemotherapy each play an important role in the optimal management of these tumors. Randomized clinical trials have begun to address some of the many questions about prognosis, natural history, and treatment, but most questions have yet to be answered. We believe that, when possible, a maximal surgical resection consistent with preservation of neurologic function should be performed, even though it is likely that no randomized clinical trial will ever be done to demonstrate a survival advantage for this approach. External beam radiation therapy is most often given to a total dose of 50.4 or 54 Gy in 1.8-Gy fractions. The role of chemotherapy is less certain, but a growing body of evidence suggests that temozolomide, a generally well-tolerated drug, is active in the treatment of LGGs. In recent years, loss of heterozygosity of chromosome 1p and 19q, as well as silencing of the MGMT gene, have been identified as promising predictors of response to adjuvant therapy in gliomas. Although randomized trials have not yet shown a survival benefit for early radiation therapy or chemotherapy, one study by the European Organisation for Research and Treatment of Cancer did show an improvement in time to tumor progression with the earlier use of radiation therapy. In addition, a trial by the Radiation Therapy Oncology Group (soon to be analyzed and reported) is comparing radiation alone with radiation followed by a year (six cycles) of standard-dose PCV chemotherapy (procarbazine, CCNU, and vincristine); this trial may shed light on the use of chemotherapy in conjunction with radiation therapy for the initial treatment of LGGs. Because patients remain at risk for tumor progression for the remainder of their lives, we recommend lifelong follow-up with MRI scans, even for patients without documented tumor regrowth over long intervals. To give clinicians a more solid basis for guiding therapy recommendations, we encourage participation in large cooperative group clinical trials.
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PMID:Low-grade gliomas in adults. 1857 14

The early appearance of high grade glioma on magnetic resonance (MR) imaging was retrospectively reviewed in the clinical records and MR images of 52 patients with intracerebral glioma treated in Osaka General Medical Center between 1997 and 2006. Three patients had no abnormal findings, and four patients had only hyperintense areas on T(2)-weighted imaging at initial MR examination. Five of the seven patients presented with generalized seizures. Six of the seven patients developed tumor progression within only 5 months. All patients underwent surgical tumor resection and the histological diagnoses were all high grade gliomas, glioblastomas in five, gliosarcoma in one, and anaplastic astrocytoma in one. Surveillance MR imaging should be performed at short intervals in adult patients presenting with seizures but with no or minimal abnormalities on initial MR imaging to identify progression of high grade glioma at the earliest opportunity.
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PMID:Early appearance of high grade glioma on magnetic resonance imaging. 1916 96


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