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)

TLR-9 agonists are immunostimulating agents that have antitumor effects in animal models. A phase I trial was conducted to define the safety profile of subcutaneous injections, combined with intrathecally administration of CpG-28, a TRL 9 agonist, in patients with neoplastic meningitis (NM). Cohorts of 3-6 patients with NM were treated for 5 weeks with escalating doses of CpG-28. The primary endpoint was tolerance. Secondary endpoints were progression free survival (PFS) and overall survival (OS). Twenty-nine patients were treated with CpG-28. The primary cancers were malignant glioma, lung carcinoma, breast cancer, melanoma or melanocytoma, ependymoma, and colorectal cancer. The median age was 56 years and median Karnovsky Performance status (KPS) was 70%. The treatment was well tolerated. Adverse effects that were possibly or probably related to the studied drug were grade 2 lymphopenia, anemia and neutropenia, local erythema at injection sites, fever and seizure. There were five serious adverse events: two confusions, two infections of ventricular devices and one grade 4 thrombopenia and neutropenia. The median PFS was 7 weeks and median OS was 15 weeks. Interestingly, the median survival was slightly (but not significantly) higher in the eight patients who were concomitantly treated with bevacizumab (19 weeks vs 15 weeks; P = 0.11). CpG-28 was well tolerated at doses up to 0.3 mg/kg subcutaneously and 18 mg intrathecally. Additional trials are warranted.
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PMID:Immunotherapy with CpG-ODN in neoplastic meningitis: A phase I trial. 2609 10

We report a 10-year-old boy with supratentorial cortical ependymoma that rapidly grew in the course of 3 years. He suffered generalized seizures when he was 5 years old; MRI showed a small cortical lesion in the right postcentral gyrus. MRI performed 2 years later revealed no changes. For the next 3 years he was free of seizures. However, at the age of 10 he again suffered generalized seizures and MRI disclosed a large parietal tumor. It was resected totally and he remains free of neurological deficits. The histopathological diagnosis was ependymoma. Pediatric supratentorial cortical ependymomas are extremely rare. We recommend including cortical ependymoma as a differential diagnosis in pediatric patients with cortical mass lesions presenting with seizures and careful follow-up even in the absence of symptoms because these tumors may progress.
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PMID:Rapidly Enlarging Pediatric Cortical Ependymoma. 2618 Jun 22

Cerebral arteriovenous malformations (AVM) are traditionally considered primary congenital lesions that result from embryological aberrations in vasculogenesis. Recent insights, however, suggest that these lesions may be secondary to a vascular insult such as ischemia or trauma. Herein, the authors present a rare case of a secondary cerebral AVM, occurring in a young girl who received prior cranial radiation therapy. At age 3years, she underwent surgical resection, chemotherapy, and photon radiation therapy for treatment of a fourth ventricular ependymoma. At age 19years, she developed new onset seizures and was found to have a left medial temporal lobe AVM. Her seizures were managed successfully with anti-epileptic medications and the AVM was treated with proton radiation therapy. This case highlights a rare but possible vascular sequela of radiation therapy and adds to the growing body of evidence that cerebral AVM may arise as secondary lesions.
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PMID:De novo development of a cerebral arteriovenous malformation following radiation therapy: Case report and an update to classical arteriovenous malformation nomenclature. 2686 Aug 50

Angiocentric glioma (AG) is a low grade glioma, that was first described in 2002. Since this description, 83 patients with AG have been described, including ours. AG typically presents in childhood with medically refractory seizures that are cured with gross surgical resection. Whilst the natural history is that of a benign tumour, there have been reports of recurrence, transformation, and malignant features that suggest that AG is potentially malignant. We add to the literature a case of a 16-year-old girl who presented in May 2011 with a 3-month history of complex partial seizures, with MRI showing a T2-weighted hyperintense lesion in the left insula and inferior frontal lobe. This was confirmed on biopsy as AG and was followed with surveillance imaging. In April 2012, she presented with disease progression and underwent a left temporal lobectomy, with histology showing both AG and grade II astrocytoma. Adjuvant radiotherapy of 50 Gray in 28 fractions was administered. A small area of contrast enhancement appeared in the left parietal lobe in December 2012, which progressed over subsequent months. In June 2013, she underwent a near total excision, with histology showing anaplastic ependymoma. She received six cycles of adjuvant temozolamide. Despite this, the tumour continued to progress, with her seizure control deteriorating, and the development of a right hemiparesis. The patient died in January 2014, aged 19years.
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PMID:Angiocentric glioma transformed into anaplastic ependymoma: Review of the evidence for malignant potential. 2774 74

Narrative Medicine sessions can encourage patients to rediscover personal identity and meaning by telling or writing their stories. We explored this process to improve care and quality of life for brain cancer patients in an academic neuro-oncology program. Brain cancer and its treatments may threaten a patient's quality of life and sense of self in many ways, including impaired cognitive skills, loss of memory, reduced coordination, and limited capacity for self-expression. The impact of symptoms and side effects on quality of life must be evaluated in terms of each patient's identity and may be understood in terms of each patient's story. Insights from Narrative Medicine visits may also be helpful for the treatment team as they seek to assess patient needs, attitudes, and abilities. We provide case-based histories demonstrating applications of Narrative Medicine in the care of patients with brain tumors whose sense of self and quality of life are challenged. The cases include managing frontal lobe syndrome of loss of initiative and pervasive emotional apathy with his wife and young children, regaining a meaningful activity in a patient, re-establishing self-identity in a young woman with ependymoma, and improving spells with coexistent epilepsy and psychogenic non-epileptic seizures (PNES).
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PMID:Narrative Medicine perspectives on patient identity and integrative care in neuro-oncology. 2866 13

This 48-year-old-man who had undergone right thyroid lobectomy for undifferentiated thyroid carcinoma nine years earlier developed generalized seizures. His cerebrospinal fluid was xanthochromic with elevation of total protein. Computed tomography (CT) showed mixed-density bilateral ventricular masses. Magnetic resonance imaging (MRI) revealed multiple nodules in both lateral ventricles; they were heterogeneously enhanced by gadolinium. Diffuse hyperintensity in the right medial temporal lobe and bilateral subependymal area was noted on fluid-attenuated inversion recovery images. Susceptibility-weighted imaging showed low intensity in the masses and cerebellar sulci suggesting hemorrhage and hemosiderin deposition. The preoperative diagnosis was disseminated malignant tumor with recurring hemorrhage. Histological examination of biopsy specimens showed clusters of cells with small uniform nuclei embedded in a dense fibrillary matrix of glial cells and microcystic degeneration. Pseudo-rosettes indicating ependymoma were absent. Microhemorrhages and hemosiderin deposits were noted. Immunohistochemically, the background fibrillary matrix and neoplastic cells were positive for glial fibrillary acidic protein. Mutated isocitrate dehydrogenase-1 was negative. The MIB-1 index was 1.5%. The tumor was pathologically diagnosed as subependymoma containing microhemorrhages and hemosiderin deposits. The extensive multiplicity and hemorrhage encountered in this case have rarely been reported in patients with subependymoma.
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PMID:Bilateral lateral ventricular subependymoma with extensive multiplicity presenting with hemorrhage. 2869 74

Myxopapillary ependymoma (MPE) is a histological variant of ependymoma found in the conus medullaris or filum terminale region. Intracranial occurrence of the tumor is a rarity. The most characteristic histological feature of myxopapillary tumors is the abundance of intercellular and perivascular mucin and the arborizing vasculature, which tends to form papillae. We are reporting a 14-year-old patient presented with seizures caused by the right occipital region intraparenchymal lesion. Histopathology confirmed it to be MPE. Lesion was excised completely. Literature reviews on the topic are discussed regarding the histological findings, natural history, and outcome of surgically treated MPE. This is the fifth reported case of cerebral intraparenchymal primary MPE.
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PMID:Occipital Intraparenchymal Myxopapillary Ependymoma: Case Report and Literature Review. 2911 96

Stroke-like migraine attacks after radiation therapy (SMART) syndrome is a rare, reversible phenomenon that occurs several years after radiotherapy in patients treated for intracranial neoplastic lesions. Patients typically present with symptoms of headache, seizures, and other focal neurologic deficits concerning for stroke or disease recurrence. In this report, we describe SMART syndrome in a 70-year-old male who developed a persistent right temporal headache, right-sided neck pain, and new-onset seizures 12 years after surgical resection of a temporal anaplastic ependymoma followed by irradiation. We present this case to highlight typical disease presentation, imaging characteristics, and important differential radiologic considerations. Recognition of this delayed complication of brain tumor radiation is paramount given its self-limited course and favorable response to conservative therapy and to avoid misinterpreting imaging findings as tumor recurrence.
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PMID:Stroke-Like Migraine Attacks after Radiation Therapy Syndrome: Clinical and Imaging Characteristics. 3144 56

Gliosarcoma is an unusual subtype of glioblastoma multiforme. Its characteristic features are biphasic configuration, constituting a definite, separate glial and sarcomatous differentiation, on histological evaluation. Herein, we present a rare case of Gliosarcoma that had presented only once in our center in last 13 years. A 60 years old, diabetic, hypertensive male patient came to e emergency department with disturbed level of consciousness and right sided hemiplegia which was progressive over four days. On examination he was, conscious, unoriented in time, person or place, his mouth deviated to left and vitally stable. After initial evaluation, CT scan and MRI were advised. These showed a complex left parieto-occipital heterogeneous mass lesion with cystic and solid components, measuring approximately 5.2x4cm. The mass lesion was seen displacing the occipital horn anteriorly and inferiorly with probable extension into the lateral ventricular cavity. There was no associated midline shift or definite herniation. The lesion was diagnosed as highly suggestive of brain tumor with a differential diagnosis of glioblastoma multiforme or ependymoma. Blood picture revealed a rapidly increasing level of anemia. Surgical intervention comprising left parieto-occipital craniotomy and near total resection of the tumor was carried out. On histopathological and immunohistochemical evaluation the diagnosis of GS was established. A plan of a combination of adjuvant chemotherapy and radiation was formulated that was however, declined by the family. On regular follow up, the patients clinical state rapidly deteriorated with persistence of seizures and requirement of repeated blood transfusions. The patient finally passed away after eighth months.
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PMID:Gliosarcoma case report and review of the literature. 3234 47


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