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 examined potential clinical and pathologic correlates of seizures among the 3,291 children in the Childhood Brain Tumor Consortium database. Fourteen percent had seizures prior to their hospitalization for a brain tumor. Among children who had a supratentorial tumor, seizures occurred in 22% of those less than 14 years of age. The prevalence of seizures increased to 68% of older teenagers. Among children with an infratentorial tumor, the prevalence of seizures was relatively constant at 6% over all age groups. The onset of seizures began more than one year prior to surgical tumor removal in over half of the children aged five or more with supratentorial tumors, significantly longer than for those of the same age with infratentorial tumors. Almost all children (98.9%) with an infratentorial tumor and seizures had at least one other symptom and more than three-fourths of them had at least three. Eighty-nine percent of children with a supratentorial tumor and seizures had at least one other symptom and more than one-half had at least three symptoms. Regardless of whether the tumor was above or below the tentorium, confusion or stupor and coma were more common in children with seizures than in children without seizures. Among children with supratentorial tumors, symptoms of a declining academic performance or an abnormality of personality, speech, walking, or sensation were significantly more frequent in children with seizures, while visual symptoms (other than visual loss or diplopia) and nausea or vomiting were less frequent. Among children with supratentorial tumors, those who had seizures were more likely to have paralysis of an arm, hand, or face, confusion or stupor, or coma and less likely to exhibit irritability, papilledema, optic atrophy, decreased visual acuity, pupillary abnormalities, or abducens paresis. Among children with infratentorial tumors, those with seizures were significantly less likely to have truncal ataxia, but more likely to experience confusion, stupor, or coma. In the supratentorial compartment, astrocytoma (nos), protoplasmic astrocytoma, anaplastic astrocytoma, and ependymoma were more frequently associated with seizures than was craniopharyngioma. No infratentorial tumor type was more or less likely to be associated with seizures. All common tumor types that were represented in both the supratentorial and the infratentorial compartment except astrocytoma (nos) were associated with significantly greater rates of seizures when located in the supratentorial compartment. The tumor location with the highest incidence of seizures was, as expected, the superficial cerebrum. More than 40% of the children with such tumors had seizures.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Epidemiology of seizures in children with brain tumors. The Childhood Brain Tumor Consortium. 154 79

The authors retrospectively reviewed ten pediatric brain tumor patients with intractable seizures who underwent lesionectomy without intentional identification and resection of the epileptogenic region to assess the clinical features and seizure outcome after lesionectomy in such patients. Seizures were complex partial in seven cases and simple partial, absence, and generalized tonic-clonic in one case each. Tumors were located at the medial temporal lobe in four cases, at the frontal lobe in four cases, at the parietooccipital and the suprasellar areas in one case each. The most common pathology was benign oligodendroglioma (five cases) followed by ganglioglioma (two cases). Others were pleomorphic xanthoastrocytoma, hamartoma, and primitive neuroectodermal tumor (one case each). In four cases, complete removal of the tumor was feasible. Postoperatively nine of the ten patients showed favorable seizure control (Engel's classification 1 and 2) and of these, six were seizure-free during the follow-up period (mean duration: 40 months). Therefore, lesionectomy can be an appropriate initial treatment for patients with brain tumor and medically intractable seizures.
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PMID:Intractable seizures associated with brain tumor in childhood: lesionectomy and seizure outcome. 860 79

Antiepileptic drugs are frequently used in children with brain tumors. This retrospective study reviewed chronic use of antiepileptic drugs in children with brain tumors at two children's hospitals between 2000 and 2007. Antiepileptic drugs were used in 32/334 pediatric brain tumor patients (10%). Almost all (94%) had supratentorial tumors, of which 78% were glial tumors. The most common localization was temporal (70%). The most frequently used initial antiepileptic drugs were phenytoin (n = 14) and oxcarbazepine (n = 7). Initial antiepileptic drugs were frequently changed, because of lack of efficacy and adverse effects, as well as concerns about possible drug interactions. At last follow-up, the most common antiepileptic drugs were oxcarbazepine (n = 11) and levetiracetam (n = 10). Levetiracetam was more likely to be used in children who received chemotherapy or radiation therapy (8/14, or 57%) than in those who did not receive adjuvant therapies (3/18, or 17%) (P = 0.03). The patients started on newer-generation antiepileptic drugs (levetiracetam, oxcarbazepine, lamotrigine) tended to remain on the same antiepileptic drugs more than did patients on older-generation antiepileptic drugs (valproic acid, phenytoin, phenobarbital) (73% vs 28%) (P = 0.04). Newer antiepileptic drugs, especially those without significant drug-drug interactions, may be a more appropriate first choice in children with brain tumors and seizures.
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PMID:The use of antiepileptic drugs in pediatric brain tumor patients. 1966 35

Craniopharyngiomas are the third most common pediatric brain tumor and most common pediatric suprasellar tumor. Contemporary treatment of craniopharyngiomas uses limited surgery and radiation in an effort to minimize morbidity, but the long-term health status of patients treated in this fashion has not been well described. The purpose of this study was to analyze the health status of long-term survivors of pediatric craniopharyngioma treated primarily with radiation and conservative surgical resection. Medical records of all long-term survivors of craniopharyngioma treated at St. Jude Children's Research Hospital and then transferred to the long-term follow-up clinic were reviewed. The initial cohort comprised 55 patients. Of these, 51 (93%) were alive at the time of this analysis. The median age at diagnosis was 7.1 years (range, 1.2-17.6 years), and 29 (57%) were male. At the time of analysis, the median survival was 7.6 years (range, 5.0-21.3 years). Diagnosis and treatment included surgical biopsy, resection (n = 50), and radiation therapy (n=48). Only 1 patient received chemotherapy. Polyendocrinopathy was the most common morbidity, with hypothyroidism (96%), adrenocorticotropic hormone deficiency (84%), and diabetes insipidus (53%) occurring most frequently. Half of the patients were hypogonadal, and 33 (65%) were overweight or obese. The most common neurologic problems included shunt dependence (37%), seizures (28%), and headaches (39%). Psychological and educational deficits were also identified in a significant number of these individuals. Despite efforts to reduce morbidity in these patients, many survivors remain burdened with significant medical complications. In a small percentage of patients, complications may result in death even during extended remission of craniopharyngioma. Because of the broad spectrum or morbidities experienced, survivors of craniopharyngioma continue to benefit from multidisciplinary care.
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PMID:Health status in long-term survivors of pediatric craniopharyngiomas. 2120 70

Survivors of pediatric brain tumors are at risk for long-term psychological morbidities. The current study investigated the prevalence and predictors of suicide ideation (SI) in a clinical sample of youth and adult survivors. Retrospective chart reviews were completed for 319 survivors of pediatric brain tumors who were assessed via clinical interview during routine neuro-oncology clinic visits between 2003 and 2007. Survivors were, on average, 18.0 years of age (SD = 4.9) and 10 years from diagnosis (SD = 5.0) at their most recent follow-up. The most common diagnosis was low-grade glioma (n = 162) followed by embryonal tumors (PNET/medulloblastoma; n = 64). Multivariable logistic regression was used to calculate odds ratios (OR) and 95 % confidence intervals (CI) for SI. Nearly 12 % of survivors (11.7 %, n = 37) reported SI. Five survivors (1.5 %) had documented suicide attempts, though none were fatal. In a multivariable model, adjusting for sex and age, history of depression (OR = 20.6, 95 % CI = 4.2-101.1), psychoactive medication treatment (OR = 4.5, 95 % CI = 1.8-11.2), observation or surgery only treatment (OR = 3.7, 95 % CI = 1.5-9.1), and seizures (OR = 3.6, 95 % CI = 1.1-11.1) were significantly associated with SI in survivors. Survivors of pediatric brain tumors appear to be at risk for experiencing SI. Our results underscore the importance of a multidisciplinary approach to providing follow-up care for childhood brain tumor survivors, including routine psychological screenings.
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PMID:Suicide ideation in pediatric and adult survivors of childhood brain tumors. 2362 16

Ganglioglioma (GG) is a rare pediatric brain tumor (1-4 %) with neoplastic glial and neuronal cells. Posterior fossa GGs (PF GGs) occur less frequently than supratentorial GGs (ST GGs). The BRAF V600E mutation has been reported in GGs and carries therapeutic implications. We compare the presenting symptoms, magnetic resonance imaging, BRAF V600E mutation status, treatment, and prognosis in children with ST and PF GGs. The neuro-oncology database at a tertiary care Children's Hospital was retrospectively reviewed from 1995 to 2010 for patients with ST and PF GG. All available imaging was reviewed. Symptoms, BRAF V600E mutation status, treatment, and survival data were collected from the electronic medical record and analyzed. Our series consisted of 11 PF GG and 20 ST GG. Children with PF GG presented with ataxia, cranial nerve deficits and long tract signs whereas the majority with ST GGs presented with seizures. On imaging, PF GGs were infiltrative and expansile solid masses with dorsal predominant "paintbrush" enhancement whereas ST GGs were well circumscribed mixed solid and cystic masses with heterogeneous enhancement. Five of 11 (45%) PF GGs and 6 of 9 (67%) ST GGs expressed the BRAF V600E mutation. No unique imaging features were identified in BRAF V600E mutation positive tumors. The majority of ST GGs were treated with surgery alone, whereas the majority of PF GGs required multimodality therapy. PF GGs had worse progression-free survival and a higher mortality rate compared with ST GGs. Unlike ST GGs, PF GGs are expansile, infiltrative, show dorsal predominant "paintbrush" enhancement, are not amenable to gross total resection, and have worse progression-free survival and mortality.
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PMID:Pediatric posterior fossa ganglioglioma: unique MRI features and correlation with BRAF V600E mutation status. 2479 87

In pediatric patients, brain tumors have been estimated to be the cause for seizures in only 0.2-0.3% of cases, whereas seizures occurred in about 13% of pediatric brain tumor patients at presentation. This survey was conducted to analyze EEG findings in pediatric tumor patients over the past 14 years to evaluate the diagnostic value of preoperative EEG for diagnosis of brain tumors. Surface EEG was obtained in awake patients using the international 10- to 20-electrode placement in all pediatric patients with intracranial neoplasms between 2000 and 2013 at the University Hospital of Leipzig except for those who needed emergency operative treatment. One hundred forty-two pediatric patients with 80 infratentorial and 62 supratentorial tumors (WHO grades I-II: 91 patients; WHO grades III-IV: 46 patients). Symptomatic hydrocephalus was found in 37. Sensitivity and specificity of ophthalmologic examination for predicting hydrocephalus was 0.39 and 0.72. Preoperative EEG has been conducted in 116 patients, showing normal activity in 54 patients (47%). Out of 62 pathologic EEGs, 40 indicated correctly to the site of the lesion, 15 were pathologic despite of infratentorial location of the tumor. Nineteen patients had a history of seizures of which six had normal EEGs. Sensitivity for and specificity of EEG examination for symptomatic epilepsy was 0.68 and 0.7. Conclusion Preoperative routine EEG provides no additional value in the diagnostic algorithm of pediatric train tumors. The low specificity and sensitivity of EEG (even in patients with clinical seizures as primary symptom of a brain tumor) underline that EEG does not contribute to diagnosis and a normal EEG might even delay correct diagnosis.
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PMID:Signs and symptoms of pediatric brain tumors and diagnostic value of preoperative EEG. 2624 70

Astrocytoma is the most common childhood brain tumor. Anaplastic astrocytoma (AA) are high grade gliomas (HGG), found very rarely in pediatric patients. AA mainly results from a dedifferentiation of a low grade astrocytoma. Clinical features of supra-tentorial tumors vary according to their anatomic location, biologic aggressiveness and age of the patient. They can be either completely asymptomatic or present with signs of raised intracranial pressure, seizures (about 40% of cases), behavior changes, speech disorders, declining school performance, or hemiparesis. There have been published adult cases of brain tumor misdiagnosed as viral encephalitis. Due to variety of clinical presentations, diagnosis of AA can be challenging. Here we report a case of a 13 year old girl who presented with clinical features suggestive of viral encephalitis, such as fever, headache, dizziness, and first seizure with postictal sleep and prolonged drowsiness. However, her brain MRI findings were consistent with long standing mass effect from the underlying intracranial contents and that coupled with her history of unusual taste led to further investigations and the diagnosis of the AA. In retrospect, this presentation could have been a temporal epileptic aura. High grade astrocytomas are particularly difficult to treat with a two-year survival rates range from 10% to 30%. The treatment is multimodal with gross total surgical resection of the tumor, followed by radiotherapy with or without nitrosourea-containing chemotherapy regimen. Recent promising results seen with the use of temozolamide in adults has not been yet demonstrated in the pediatric patients. The extent of tumor resection remains the most significant indicator of survival and early recognition of this tumor is essential. This case report emphasizes the fact that mass lesions in the temporal lobe, including high-grade astrocytoma, should be considered in the differential diagnosis of suspected herpes simplex encephalitis, especially those not responding to therapy. Remodeling of the calvarium adjacent to an intracranial lesion suggests a long standing process.
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PMID:Anaplastic astrocytoma mimicking herpes simplex encephalitis in 13-year old girl. 2627 84

Neurological complications resulting from childhood cancer treatments are common. Treatment for childhood neoplastic disorders is often multimodal and may include procedures, cranial irradiation, chemotherapy, transplant, and immunotherapy, each of which carries distinct neurological risks. Procedures, such as lumbar punctures, are commonly used in this population for diagnostic purposes as well as intrathecal medication administration. Surgery is associated with an array of potential neurological complications, with posterior fossa syndrome being a common cause of morbidity in pediatric brain tumor patients after neurosurgical resection. Cranial irradiation can cause late neurological sequelae such as stroke, cerebral vasculopathy, secondary malignancy, and cognitive dysfunction. Neurotoxic effects of chemotherapeutic agents are common and include neuropathy, coagulopathy causing stroke or cerebral sinovenous thrombosis, encephalopathy, seizures, cerebellar dysfunction, myelopathy, and neuropsychologic difficulties. Hematopoietic stem cell transplant has a high risk of neurological complications including central nervous system infection, seizures, and stroke. Immunotherapies, including chimeric antigen receptor-modified T-cells (CAR T-cells) and immune checkpoint inhibitors, are emerging as potentially effective strategies to treat some types of childhood cancer, but may carry with them substantial neurotoxicity which is just beginning to be recognized and studied. With evolving treatment protocols, childhood cancer survivorship is increasing, and the role of the neurologist in managing both the acute and chronic neurological consequences of treatment is becoming more important. Prevention, early recognition, and treatment of therapy-associated neurotoxicity are imperative to ensuring children can remain on the most effective therapeutic regimens and to improve the neurological function and quality of life of childhood cancer survivors.
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PMID:Neurological Complications of the Treatment of Pediatric Neoplastic Disorders. 3012 55

Primary central nervous system lymphoma (PCNSL) is a rare pediatric brain tumor. A 16-year-old female patient presented to the clinic with complaints of multiple episodes of generalized tonic clonic seizures, nystagmus, and weakness on the left side of the body for 3 weeks. She had similar symptoms, waxing and waning for the last 2 years, responding to corticosteroids. Repeat magnetic resonance imaging (MRI) of the brain showed multiple areas of signal abnormalities involving the left temporal lobe, the basal ganglion, the thalamus, and the right frontal and occipital lobes with contrast enhancement in bitemporal lesions. With a clinico- radiological diagnosis of demyelinating disorder, she underwent an image-guided right frontal lobe biopsy, which revealed sheets of atypical lymphoid cells diffusely immunopositive for CD20 but negative for CD3, CD10, BCL-6, and MUM-1, suggesting diffuse large B-cell lymphoma, germinal center B-cell subtype. The systemic lymphoma workup was essentially normal. She received 5 cycles of chemoimmunotherapy with rituximab, high-dose methotrexate (HDMTX), vincristine, and procarbazine and had a complete radiological response (CR). This was followed by whole brain radiotherapy (WBRT) to a dose of 36 Gy in 20 fractions over 4 weeks. Subsequently she received 2 cycles of consolidation chemoimmunotherapy with rituximab and high-dose cytarabine. Serial brain MRI done 1, 4, and 8 months after completion of treatment showed persistence of the CR. At the last follow-up visit, 15 months from the date of diagnosis, she was disease free and asymptomatic. This report underlines the fact that PCNSL in adolescents may be effectively treated with a combination of HDMTX- and rituximab-based chemoimmunotherapy followed by consolidation with WBRT.
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PMID:A Rare Case of Primary Central Nervous System Lymphoma in an Adolescent Female Treated with High-Dose Methotrexate and Rituximab-Based Chemoimmunotherapy and Consolidation Whole Brain Radiotherapy. 3066 45


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