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

We report a case of a 30-year-old woman who developed an intraparenchymal cerebral metastasis from a Ewing's sarcoma of the chest wall diagnosed and treated 3 years earlier and in apparent remission at the time of the neurological presentation (seizures). The case was complicated by a spontaneous preoperative intratumoral hemorrhage that occurred the morning of the scheduled surgical resection and caused a left dense hemiparesis. The tumor and the hematoma were removed. The patient improved after surgical intervention and a postoperative course of cranial irradiation followed by systemic chemotherapy was given. Nineteen months after treatment the patient remains disease-free from the clinical and radiological standpoint.
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PMID:Solitary, isolated metastasis from Ewing's sarcoma to the brain: case report. 199 54

Ifosfamide/mesna was given to 97 patients who had malignant solid tumors diagnosed before they were 21 years of age. Patients received 1.6 g/m2 ifosfamide daily x 5, given i.v. over 15 min, followed by 400 mg/m2 i.v. mesna at 15 min and 4 and 6 h after ifosfamide. Responses were noted in patients with osteosarcoma, Ewing's sarcoma, rhabdomyosarcoma and other soft-tissue sarcomas, rhabdoid tumor, neuroblastoma, Wilms' tumor, primitive neuroectodermal tumor, retinoblastoma, germ-cell tumors, and B-cell lymphoma. Toxicity included mild to moderate nausea and vomiting, transient, reversible myelosuppression, transient elevations of serum blood urea nitrogen (BUN) and creatinine and liver enzymes, infections, and self-limiting neurotoxicity characterized by changes in mental status, motor dysfunction, cranial nerve palsy, cerebellar dysfunction, and seizures. Neurotoxic symptoms were generally seen in patients who had previously received cisplatin. Ifosfamide is an important alkylating agent that should be combined with other agents in phase II and III trials. Alternate dose schedules should also be investigated.
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PMID:Ifosfamide in pediatric malignant solid tumors. 250 57

From 1980 to 1987, 162 consecutive children with soft tissue and osseous sarcoma were reviewed to determine the frequency and types of neurologic complications seen. Neurologic complications occurred in 43 of 162 (26.5%) patients. Children with poorly differentiated sarcomas and rhabdomyosarcoma were more likely to have neurologic complications, which occurred in 39% of patients at risk. The types of complications seen included: metastatic spinal cord compression (11%); symptomatic peripheral neuropathy (10%); intracranial metastatic disease (7.5%); seizures (6%); and acute and chronic methotrexate-related neurologic dysfunction (2.5%). Spinal cord compression frequently occurred early in disease whereas brain metastases was almost always a late finding. Symptomatic peripheral neuropathy occurred primarily in children with rhabdomyosarcoma and Ewing's sarcoma. The advent of increasingly successful therapies for children with sarcoma and the frequency of severe neurologic complications indicate that a heightened level of surveillance for neurologic compromise is required.
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PMID:Neurologic complications in children with soft tissue and osseous sarcoma. 255 41

A child with progressive facial hemiatrophy (PFHA) later had Ewing's sarcoma develop. During seizures presumably related to her PFHA, she suffered pathologic fractures on two occasions. The second of these might have been avoided had her condition been better understood.
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PMID:Progressive facial hemiatrophy. Report of a case associated with Ewing's sarcoma. 743 66

The autopsy findings of a 14-year-old Japanese girl with Ewing's sarcoma, who had multiple neurofibrillary tangles and Lewy bodies and hemiatrophy of the central nervous system (CNS), are reported. She had retinoblastoma of her right eye 8 months after birth, which was treated with chemotherapy and irradiation (40 Gy), twice, seizures 1 year and 2 months after birth, and thereafter severe mental retardation. She showed left hemiparesis after a febrile seizure at the age of 7 years and CT disclosed the right cerebral hemiatrophy. For the last 2 years of life she suffered from Ewing's sarcoma. Extrapyramidal signs were absent. Neuropathologically, tangles consisting of paired helical filaments were distributed symmetrically in virtually all the grey matter. They were particularly numerous in the frontal cortex and substantia nigra, but sparse in the nucleus of Meynert, hippocampus, and brainstem. Several Lewy bodies, which were ultrastructurally identical to those seen in Parkinson's disease, were present in the substantia nigra (more on the left than right) and locus coeruleus. Morphometrically, the number and size of substantia nigral neurons were reduced, the reduction in the latter being more marked than the former, but the melanin pigment contents and shapes of the remaining neurons appeared normal. The right cerebral hemiatrophy with contralateral cerebellar hemiatrophy may have been attributable to irradiation. Although our patient did not have parkinsonism, her features resembled those of a 28-year-old autopsy case reported by Popovich et al. [1987].
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PMID:A 14-year-old patient with Ewing's sarcoma presenting at autopsy with multiple neurofibrillary tangles and Lewy bodies in addition to hemiatrophy of the central nervous system. 910 Nov 9

The frequency and severity of neurologic symptoms in children with systemic cancer is unknown. The authors reviewed the records of children with systemic cancer for whom a neurologic consultation was requested between 1993 and 1996. The 157 patients had 161 malignancies and 205 consultations. Leukemia (59) and lymphoma (34) were the most common malignancies. The 68 solid tumors included neuroblastoma (13), Ewing's sarcoma, and rhabdomyosarcoma (10 each). In contrast to adults, in whom back pain and altered mental status are the most common reasons for neurologic consultation, headache (33) and seizures (29) were the most common symptoms in children. Structural lesions were present in 84% of patients with headache and focal deficit and in 14% of patients with isolated headache. Structural disease was identified in 37% of children with seizures. Neurologic signs were caused by complications of cancer therapy in 70 instances and to direct tumor invasion of the nervous system in 60. In 71 consultations, neurologic symptoms could not be attributed to cancer or its treatment. The spectrum of neurologic symptoms in children with cancer differs from adults and requires the consulting neurologist to have a thorough knowledge of childhood cancer and its effects on the nervous system.
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PMID:Neurologic consultations in children with systemic cancer. 1008 40

A 11-year-old girl was admitted for Ewing's sarcoma within the right pelvis. Hematological and biochemical indices were normal. Since this was considered a difficult case for operation, radiotherapy and chemotherapy were performed. Intra-arterial chemotherapy with cisplatin 130 mg, doxorubicin 33 mg and caffeine 1,600 mg was started. Then, 38 hours after the administration of drugs, the girl became drowsy and seizures appeared. Laboratory studies revealed serum sodium 113 mEq/l, urine sodium 125 mEq/l and plasma osmolality of 238 mOsm/kg H2O. The diagnosis was SIADH secondary to cisplatin, and the treatment was fluid restriction. Her serum sodium level rose to 134 mEq/l, and her consciousness became clear. Concurrent administration of cisplatin aimed to prevent renal dysfunction, but hyponatremia developed due to massive fluid administration. Especially for this case, caffeine was administered. In conclusion, early recognition of SIADH is imperative to effectively correct the fluid and electrolyte imbalance.
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PMID:[Syndrome of inappropriate secretion of antidiuretic hormone suddenly occurring in a case following chemotherapy]. 1567 94

Posterior reversible encephalopathy syndrome (PRES) is a clinico-neuroradiologic disease entity represented by characteristic magnetic resonance image (MRI) findings of subcortical/cortical hyperintensity in T2-weighted sequences, more often observed in parieto-occipital lobes, accompanied by clinical neurologic alterations. PRES is a rare central nervous system complication in childhood hematologic-oncologic patients and shows very different neurologic symptoms between patients, from numbness on extremities to generalized seizure. The etiology of PRES was not well known until these days. In this study, 8 patients with PRES were reviewed, retrospectively. There were 4 patients with acute lymphocytic leukemia, 1 with aplastic anemia, and 3 with solid tumors (1 patient each for neuroblastoma, Ewing sarcoma, and osteosarcoma). Allogeneic stem cell transplantation was performed in 2 patients. Immunosuppressive agents such as tacrolimus and cyclosporine A were used in 3 patients. One neuroblastoma patient was in immediate postoperative status. All patients experienced seizure attacks of different types and showed typical MRI findings. Follow-up MRIs revealed significant improvements. From this review, we might consider chemotherapy and surgery as additive causes for PRES other than immunosuppressive agents. Therefore, careful examination of the patients receiving chemotherapy and surgery was needed to find out this uncommon but good prognostic complication.
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PMID:Posterior reversible encephalopathy syndrome in childhood with hematologic/oncologic diseases. 1956 46

Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) is a malignant small, round cell tumor arising from bone and soft tissue in children and young adults. It can occur at osseous and extraosseous sites. Its usual locations are diaphysis of long bones followed by pelvis, ribs, vertebrae, and rarely skull. We reviewed the literature and PubMed advanced search on ES/pPNET occurring at extraosseous sites, mainly involving the central nervous system (CNS). We reported a case of a 22-year-old male presenting with seizure finally diagnosed as a case of ES/pPNET. The challenges in management of this rare CNS tumor and its differential diagnosis are highlighted. We found that most cases of ES involving CNS represent secondary metastases from extracranial sites of ES/pPNET and there are rare case reports of primary intracranial ES-pPNET. Furthermore, among these intracranial tumors, most common tumors occupy an intraaxial location and only a handful of cases of dural-based or extraaxial tumors mimicking meningioma are reported. Differentiation of pPNET from central PNET (cPNET) is important as it has definitive therapeutic and prognostic implications. Awareness of this entity of ES/pPNET, its rare dural presentation, and differentiation from the more common cPNET is needed for appropriate patient management. Meningeal ES/pPNET has to be kept in mind in the differential diagnosis of meningeal tumors eroding bone.
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PMID:Primary intracranial dural-based Ewing sarcoma/peripheral primitive neuroectodermal tumor mimicking a meningioma: A rare tumor with review of literature. 2876 7