Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017638 (glioma)
30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We experienced a rare case of Takayasu disease with low grade glioma. A 36 year-old female had an episode of right hemiconvulsion. On admission, she complained a slight memory disturbance, urination difficulty and motor weakness of right extremities. CT scan demonstrated a low density lesion in the left frontal lobe. MRI showed an inhomogeneous low intense mass on T 1 WI and a homogeneous high intense mass on T2 WI in the same site. No abnormal contrast enhancement was observed in both CT and MRI. Bilateral carotid angiograms demonstrated a filling defect of the left anterior cerebral artery. An aortogram revealed moderate stenosis of the left carotid artery and the brachiocephalic artery, and complete obstruction of the left subclavian artery. CT-guided stereotactic biopsy was performed using BRW stereotactic system and histological diagnosis was astrocytoma grade II. She was followed by interstitial irradiation of 50 Gy at tumor periphery for 10 days. Her neurological symptoms gradually improved, and she is doing well without neurological deficits at the time of this report. The CT scan obtained 13 months after the brachytherapy revealed a decrease in the extent of low density. Brachytherapy seems to be useful for patients with low grade glioma in the eloquent area of the brain.
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PMID:[A case of low grade glioma with Takayasu disease (aortitis syndrome)]. 826 38

We report a patient with selective short-term memory disturbance caused by a glioma in the left temporal-parietal lobe. The patient was a 40-year-old right-handed housewife who complained of difficulty in memorizing series of numbers. She was working as a car dealer. She was well until 6 months prior to the present admission when she noted a difficulty in memorizing series of numbers such when telephone numbers and car registration numbers. She had to write them down as her customers told them to her. On admission, she was alert and oriented to all spheres. She was mentally sound without dementia. She did not show aphasia, apraxia, or agnosia, except for brief periods of seizures in which she became unable to speech. Neuropsychological examination revealed that she had difficulty in repeating and dictating series of numbers and meaningless kana words. However, she could easily pick up the correct series of numbers or kana words among multiple choices presented visually. Thus it was clear that her problem was not the disturbance of auditory input nor expression, but a selective impairment of short-term memory. She could memorize the same stimuli when visually presented. Therefore, her problem was thought to be a disturbance of auditory short-term memory of meaningless words. After resection of her tumor, she developed transient amnesic aphasia, which improved a year later. She was examined again in her memory function. In the task of visual stimuli, we presented her a card in which a series of numbers or a nonsense syllable was written for 5 seconds and asked her to remember them.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of selective short-term memory disturbance due to a glioma in the left temporo-parietal lobe]. 834 99

A 7-year and 11 month-old girl with cerebellar astrocytoma linked to familial ataxia-telangiectasia (AT) is presented. She was born as the 7th girl of a woman with aortic arch syndrome. Two elder sisters of the patient have ataxia telangiectasia. She had immunodeficiency, and cerebellar ataxia, but had no oculocutaneous telangiectasia. The risk of cancer developing in AT patients is about 1,200 times greater than that in age-matched controls. With regard to central nervous system tumours, seven primary tumours have been reported, such as 3 cases of medulloblastoma and 4 cases of glioma. Members of AT families who were under the age of 45 had a risk of dying of a malignant neoplasm five times greater than in the general population. However, there were no reports of glioma in AT families. In this case, it is suggested that IgA deficiency linked to familial AT may have contributed to the development of astrocytoma.
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PMID:Astrocytoma linked to familial ataxia-telangiectasia. 874 98

A 21-year-old woman was diagnosed with Turcot's syndrome (TS) at age 16 years. She had two ependymomas, one was located in the left middle cerebellar peduncle and the other in the low sacral spinal canal. Her mother and brother both had colectomies for colonic polyposis. Her maternal uncle and grandfather also had this disease and both died from cancer of the colon in their fourth decade of life. The patient was found to have hyperpigmented spots in the retina, skull osteomas and normal neurological examinations. The bone scan and CSF were normal and she had a germline mutation in the segment 3 of the adenomatous polyposis coli (APC) gene. Following partial resection of the two ependymomas, she was treated with radiation and chemotherapy. One year after surgery, paraspinal desmoid tumors were found and removed. She is presently 42 months postsurgical resection of the neural tumors and has remained central nervous system tumor-free. The occurrence of multiple ependymoma in TS has not been reported, and the control of this patient's ependymomas is consistent with other reports of long-term survival with TS and glial tumors.
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PMID:Multiple ependymomas in a patient with Turcot's syndrome. 895 Mar 38

An 11-year-old female presented with headache in May 1993. Magnetic resonance (MR) imaging disclosed a small lesion (9 mm in diameter) in the left occipital lobe. No treatment was performed because the lesion was small. She subsequently developed frequent episodes of scintillating scotoma in the right visual field eleven months later. MR imaging eleven months after the first MR imaging showed the lesion had enlarged to 14 mm in diameter. Preoperative surface electroencephalography (EEG) detected no spike waves. The diagnosis was localized glioma. The mass was totally removed by gyrectomy in December 1994. Intraoperative cortical EEG demonstrated spike waves which disappeared after tumor removal. The histological diagnosis was pleomorphic xanthoastrocytoma. No postoperative neurological deficit was recognized, and scintillating scotoma and headache disappeared. Postoperative stereotactic radiosurgery was performed. The scintillating scotoma was caused by the tumor, because the spike wave and phase reversal were detected by the intraoperative cortical EEG. Intraoperative EEG is useful for the diagnosis of epilepsy caused by tumor. Sulcotomy and gyrectomy are the optimal surgical treatments for epilepsy caused by a localized glioma.
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PMID:[Localized glioma in the occipital lobe manifesting as scintillating scotoma: case report]. 905 35

We report a case of a radiation-induced spinal cord glioma. A 20-year-old girl presented with neck pain and new significant neurological deficits 17 years after resection of a posterior fossa medullomyoblastoma and subsequent craniospinal irradiation. She was found to have a cervical intramedullary tumor that was resected using a standard microsurgical technique. The permanent histopathological diagnosis was anaplastic astrocytoma. Her neurological status was worse immediately following the operation. She improved to her pre-operative status, but then had a relentless neurological decline resulting in death 16 weeks following surgery. Because of the high incidence of malignancy in the few radiation-induced spinal cord gliomas reported in the literature, and the poor outcome independent of therapy in patients with malignant spinal cord gliomas, an initial strategy of biopsy only may be more appropriate than attempted resection for the patient with a suspected radiation-induced intramedullary spinal cord tumor.
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PMID:Radiation-induced glioma of the spinal cord. 929 48

We report a 9-year-old girl with tuberous sclerosis presenting intractable adversive seizure. She had been suffering from frequent attacks of consciousness loss since the age of 6 years. Although a considerable amount of antiepileptic drugs had been administered, her epileptic attacks were not controlled, but instead rather increased. She had been suffering from adversive seizure to the right for more than 2 years. CT scan failed to show any abnormal density area. MRI showed a small lesion in the left frontal subcortical area. The electroencephalogram showed relatively mild abnormal waves in the left hemisphere. We undertook surgical removal of the lesion with epileptogenic foci because her epilepsy has not been controlled and the lesion could be a glioma. Abnormal spike waves were detected around the lesion with electrocorticogram. "Gyrectomy" technique was employed and the spike waves totally disappeared. After the surgery, no neurological deterioration was presented. She has suffered no seizure attack since the surgery even though the amount of the antiepileptic drugs has been significantly decreased. Resection of the epileptogenic foci as well as the abnormal lesion using the technique of gyrectomy is useful for the control of the intractable epilepsy, and makes the quality of life of patients much higher.
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PMID:[A case of tuberous sclerosis presenting intractable adversive seizure, successfully resected with the technique of "gyrectomy"]. 933 Apr 2

We report a case of optic glioma with bilateral optic atrophy. A 3-year-old girl presented with vomiting and left hemiparesis. She had hypothalamic dysfunction, right ptosis, right monocular nystagmus, left facial palsy, left hemiparesis, and left pes adductus. Neuroimaging studies showed obstructive hydrocephalus with a large suprasellar calcified tumor with a ring-like enhancement mimicking craniopharyngioma. Visual-evoked potentials showed delayed latency of N75 in the right occipital lead. The tumor, a pilocytic astrocytoma in the right optic tract and chiasma, was partially removed via a right frontotemporal craniotomy. The right optic nerve had shrunk to half the normal diameter and became twisted downwardly. Intracranial pressure (ICP) increased to 40 cm H2O. The fundus had bilateral optic atrophy without disc swelling. To our knowledge, this is the first report of a lamina/dot sign of the optic disc in a small child with a brain tumor and a normal neuroretinal fiber layer. These ocular findings may result from possible interruption of the axonal flow caused by the tumor and not increased ICP.
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PMID:Optic glioma with characteristic bilateral optic atrophy in a 3-year-old girl. 1070 32

A 69-year-old right-handed woman developed a transcortical motor aphasia with hyperlexia following resection of a glioma in the left medial frontal lobe. Neurological examination revealed grasp reflex in the right hand and underutilization of the right upper extremity. An MRI demonstrated lesions in the left medial frontal lobe including the supplementary motor area and the anterior part of the cingulate gyrus, which extended to the anterior part of the body of corpus callosum. Neuropsychologically she was alert and cooperative. She demonstrated transcortical motor aphasia. Her verbal output began with echolalia. Furthermore hyperlexia was observed in daily activities and during examinations. During conversation she suddenly read words written on objects around her which were totally irrelevant to the talk. When she was walking in the ward with an examiner she read words written on a trash bag that passed by and signboards which indicated a name of a room. Her conversation while walking was intermingled with reading words, which was irrelevant to the conversation. She also read time on analog clocks, which were hung on a wall in a watch store. In a naming task, she read words written on objects first and named them upon repeated question about their names. When an examiner opened a newspaper in front of her without any instructions she began reading until the examiner prohibited it. Then she began reading again when an examiner turned the page, although she remembered that she should not read it aloud. She showed mild ideomotor apraxia of a left hand. Utilization behavior, imitation behavior, hypergraphia, or compulsive use of objects was not observed throughout the course. Hyperlexic tendency is a prominent feature of this patient's language output. Hyperlexia was often reported in children with pervasive developmental disorders including autism. There are only a few reports about hyperlexia in adults and some of them were related to diffuse brain dysfunction. Hyperlexia of our patient was associated with echolalia but not with the other "echo" phenomena, which may be because the lesion was unilateral on the left side. Dysfunction of the left supplementary motor area could lead to disinhibition of regulatory mechanism of verbal output in response to auditory and visual stimuli.
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PMID:[Hyperlexia in an adult patient with lesions in the left medial frontal lobe]. 1096 60

An autopsy case of a patient with diffuse brainstem glioma associated with Laurence-Moon-(Bardet-)Biedl syndrome is described. The subject was a 25-year-old woman who had been suffering from mental retardation, pigmented retinopathy, obesity, hexadactyly, amenorrhea and renal cysts. She developed dizziness, headache and consequent consciousness disturbance. Magnetic resonance images disclosed marked swelling of the pons without contrast enhancement. By means of combined chemotherapy and radiation, she survived for 15 months. Histopathological diagnosis for postmortem specimens obtained from the brainstem was glioblastoma multiforme. No pathogenetic association between the syndrome and brainstem gliomas is known, and the literature contains no cases of patients with this coincidence.
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PMID:Diffuse brainstem glioma in a patient with Laurence-Moon-(Bardet-)Biedl syndrome. 1118 44


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