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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To determine the frequency and nature of MRI lesions in children with neurofibromatosis type I (NF1), 50 patients aged 8 to 16 years were evaluated prospectively with cranial MRI. Forty-one children were asymptomatic with respect to central nervous system pathology, and 50% were macrocephalic. Sixteen patients (32%) had normal MRI examinations. Thirty-two patients (64%) had high intensity lesions on T2-weighted images and 16 patients (32%) had hyperintense lesions on T1-weighted images. Seven patients (14%) had ventricular dilatation (associated with increased intracranial pressure in 2) and 11 patients (22%) had optic pathway lesions (optic glioma in 7). MRI was superior to CT in differentiating optic sheath thickening and optic nerve tortuosity from optic glioma in four patients. An intracranial tumour (ependymoma) and sphenoid wing dysplasia were evident in individual patients. Findings previously unreported in NF1 included an aqueductal web resulting in hydrocephalus, intraocular neurofibroma resulting in retinal detachment, and asymptomatic enlargement of the septum pellucidum. T1 and T2 signal abnormalities in isolation were not associated with neurological deficits or the occurrence of macrocephaly, and all lesions that required intervention were suspected clinically. Macrocephaly in the absence of increased intracranial pressure or accelerated head growth is not an indication for neuroimaging in children with NF1. However, the majority of children (68%) had disease-specific abnormalities and thus MRI may provide a useful adjunct to clinical evaluation in the diagnosis of equivocal cases.
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PMID:MRI findings in children with neurofibromatosis type 1: a prospective study. 866 66

This study represents our experience with eight cases (males: 4; females: 4; 13-47 years old, average age 28.5 years) of a "glial tumourette" (minute glioma), which measured less than 15 mm in diameter on an MRI. Four tumours were located in the frontal lobe, one in the rostrum of the corpus callosum, two in the midbrain, and one in the thalamus. The symptoms and signs lasted from two days to 15 months prior to diagnosis, and they consisted of epileptic seizures in five patients and increased intracranial pressure due to hydrocephalus resulting from aqueductal stenosis in three. All patients had a CT scan and an MRI as a part of their initial neuroimaging evaluations. While the CT findings failed to show the lesion in four patients, MRI demonstrated it in all cases. Five tumours were either totally or subtotally removed while the remaining three were biopsied. Histological examinations revealed six tumours to be low-grade gliomas (fibrillary astrocytoma: 4; oligoastrocytoma: 2) and two to be high-grade gliomas (anaplastic astrocytoma: 1; anaplastic oligodendroglioma: 1). Regarding adjuvant therapy, three patients received radiation and/or chemotherapy. One of the patients with midbrain fibrillary astrocytoma died of the disease 38 months after the operation, however, no evidence of progression in the remaining seven has been observed in the follow-up period ranging from five to 65 months after the operation (average: 25.4 months). The histogenesis of benign and malignant gliomas and the importance of surgical exploration in the management of such patients with minute intracerebral tumours are also discussed.
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PMID:Glial tumourettes (glial microtumours): their clinical and histopathological manifestations. 886 9

Two cases of astrocytoma associated with von Recklinghausen's disease (neurofibromatosis type; NF-1) were reported. The first case wes a 60-year-old man who had been diagnosed as von Recklinghausen's disease on the basis of skin findings. Magnetic resonance imaging (MRI) showed a tumor in the left temporal lobe. Partial removal was performed with neuronavigator, and because of the existence of Rosenthal fiber the histological diagnosis was pilocytic astrocytoma. Radiation therapy was performed. The second case was a 6-year-old boy suffering from headache and left hemiparesis including his face. MRI showed a tumor with a cyst in the right thalamus and obstructive hydrocephalus. Initially CT-guided stereotactic biopsy was performed, and the histological diagnosis, on the basis of increased cellularity, pleomorphism and nuclear atypia without necrosis or vascular proliferation, was anaplastic astrocytoma. Radiation and chemo-immuno therapy were carried out after V-P shunt. It is well known that von Recklinghausen's disease (NF-1) is often associated with optic glioma (5-36%). In the literature, the glioma seldom occurs in other parts of the brain, supratentorial glioma especially is rare. Only two familial cases of supratentorial glioma associated with von Recklinghausen's disease have been reported. The prognosis of supratentorial glioma associated with NF-1 was poor in these reports. In this paper, the diagnostic and therapeutic problems are discussed.
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PMID:[Familial astrocytoma associated with von Recklinghausen's disease: report of two cases]. 905 38

A 4-year-old girl with optic chiasm glioma (OCG), nonobstructive hydrocephalus and ventriculoperitoneal shunt is described, in whom marked ascites developed. The ascitic fluid was protein-rich and its amount correlated with cerebrospinal fluid (CSF) protein. The CSF protein level and the amount of ascitic fluid were influenced by chemotherapy. Very unusual hypernatremia, up to 190 mEq/l with no associated alteration in mental status, was also found. It is suggested that altered absorption ability owing to the high protein content was the cause of both the nonobstructive hydrocephalus and the ascites. The unusual well being with very high sodium concentrations may have resulted from osmoreceptor dysfunction, presumably caused by hypothalamic involvement as well as by the high CSF protein. This combination of findings may point toward specific characteristics of OCG. In an effort to reduce the amount of the ascitic fluid, a further chemotherapeutic trial may be done, before converting the shunt to the vetriculoatrial system.
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PMID:Optic chiasm glioma, electrolyte abnormalities, nonobstructive hydrocephalus and ascites. 914 3

A series of 34 patients with tumours of the third ventricle were operated on by a transcallosal route. Basal extrinsic lesions compressing or invading the ventricle as well as tumours located in the pineal area were excluded from this review. Tumours were approached by a transforaminal entry in 16 cases (47%), by an interforniceal route in 11 (32%), by a subchoroidal entry in 4 (14%) and by a combined transforaminal and subchoroidal entry in 3 (9%). Four out of 34 patients were submitted to a second operation, through the same approach corridor: 2 for an incomplete removal of an intrinsic tumour and 2 for a late regrowth. Postoperative mortality rate accounted for 5.8% (2 patients). Major post operative complications were hemiparesis (4 patients) and diabetes insipidus (4 patients), that were transient in 3. Akinetic mutism like status was observed in only 1 patient. Postoperative psychic disturbances were noticed in 5 cases. Nine out of 21 patients (62%) with preoperative hydrocephalus required a permanent CSF shunt. Histology revealed that 21 tumours (62%) were intraaxial (4 pilocitic astrocytoma, 10 low grade glioma, 1 giant cell astrocytoma, 1 subependymoma, 4 ependymoma/ependymoblastoma, 1 neurocitoma) and 13 (38%) were extraaxial (8 colloid cyst, 2 craniopharingioma, 1 ectopic pituitary adenoma, 1 lymphocytic hypophysitis and 1 metastasis). Total excision of third ventricle tumours was achieved in all patients with extraaxial tumours and in 62% and 71% of intraaxial tumours with the first and second surgical procedure respectively. Ten out of 34 patients of this series were submitted to a complete neuropsychological evaluation at an interval of 2-9 years after surgery. Memory tests were pathological in 2. Disconnection signs were constantly absent. Control function were preserved. Transcallosal approach remains the best microsurgical method of third ventricle tumours treatment. This route provides the capability for a superior visualization of the entire cavity of the third ventricle through different corridors. Permanent neurological and neuropsychological deficits are not frequent. Epilepsy, that accounted for 28% in patients submitted to transcortical transventricular approach to third ventricle tumours, was never noticed in this series operated on through a transcallosal route.
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PMID:Transcallosal approach to tumors of the third ventricle. Surgical results and neuropsychological evaluation. 927 58

Analysis of 139 CSF samples from living subjects, using iso-electric focusing in polyacrylamide gels, demonstrated an anomalous molecular form of acetylcholinesterase (AChE). This form was present in 84 of 87 patients with a clinical diagnosis of Alzheimer's disease (AD), 28 of whom have died and in whom histopathological confirmation of AD was obtained. The abnormal AChE form was also present in 22 of 23 patients with clinical dementia not regarded as AD type. In the six patients who died this abnormal AChE form was found in three cases of multi-infarct dementia, one with cerebral glioma with dementia and one with clinical dementia, but no pathology was found based on the Khachaturian criteria for AD. One patient with normal pressure hydrocephalus was negative when tested for the abnormal AChE form. This evidence indicates that the anomalous molecular form of AChE may not be specific for AD, and may possibly be a common indicator for organic dementia. The discovery of this form in 27 of 29 age-matched non-demented controls may indicate that the anomalous molecular form of AChE may not only exist in patients with clinically detectable dementia, but is probably present for a period before the onset of dementia. Recognizing and understanding the existence of pre-clinical dementia would be beneficial in designing a strategy for both the prevention and the treatment of dementia.
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PMID:An CSF anamalous molecular form of acetylcholinesterase in demented and non-demented subjects. 935 48

Intracranial tumours, often presenting with progressive hydrocephalus, are rare congenital diseases accounting for 0.5-1.5 per cent of all cases of brain tumours diagnosed during childhood. The differential diagnosis includes vascular malformations, infarctions, and haemorrhages. Sonographic signs suggestive of glioblastoma, teratoma, and astrocytoma do not establish the histological diagnosis, however. We report a case of an undifferentiated fetal glioma detected at 29 weeks' gestation. The diagnosis of an undifferentiated brain tumour was suspected by sonography because of the lack of normal brain structures in conjunction with a diffuse echogenic central lesion and an external hydrocephalus. Because of the very poor prognosis, we induced labour by intravaginal and intravenous administration of prostaglandin E2 and achieved the vaginal delivery of a stillborn child whose head circumference corresponded to 38 weeks of pregnancy. Histological and immunochemical features of this undifferentiated congenital glioma (glioblastoma) are presented.
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PMID:Prenatal diagnosis of a highly undifferentiated brain tumour--a case report and review of the literature. 935 78

Brain-stem gliomas occur mainly in childhood and are localized in the mesencephalon, pons and medulla oblongata. Diagnosis is a domain of MRI, requiring T2, T1 and KM. CT shows hemorrhage and calcification well. The criteria are the primary site, size, tumor growth, brain-stem enlargement, delineation, intralesional structure, exophytic components and enhancement. Secondary criteria are herniation, hydrocephalus and liquorgenic seeding. In CT glioma are hypodense, in MRI hyperintense in T2 and hypointense in T1. Enhancement is seen in 25-60% and does not allow differentiation of tumor vs nontumor or gradings. Factors influencing poor outcome are high grade, a short history, cranial nerve involvement, severe brain-stem enlargement, pontine site, diffuse growth and recurrency. The 5-year-survival rate is 30% (after radiation: focal tumors 85%, diffuse 20%). Most frequent are symptoms of brain pressure, cerebellum, cranial nerves and pyramidal tract. There is no agreement on whether biopsy is necessary or not. A diagnosis of tumor is highly suggestive if classical MRI findings fit the clinical history.
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PMID:[Brain stem glioma]. 986 50

A patient is described in whom a large diffuse glioma of the pons extending into the midbrain was diagnosed at the age of 2 years. Biopsy showed a fibrillary astrocytoma. After shunting of a hydrocephalus, the clinical symptoms abated without conventional therapy. Repeated MRI studies showed a continuous decrease of the tumour which was no longer visible when the patient was 6.6 years old. In reviews on spontaneous remissions of oncologic disorders we were unable to find a case of a biologically benign brain stem tumour. There is one isolated report on a similar case, though without histologic documentation.
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PMID:Complete remission of a diffuse pontine glioma. 1002 44

Neurofibromatosis type 1 (NF1) is a genetic disease with a wide range of neurological manifestations. To examine these, and to evaluate neurological morbidity in adulthood of patients with NF1, we studied a hospital-based series of 158 patients that included 138 adult patients aged >18 years and 20 children. NF1 evaluation included a multidisciplinary clinical and a clinically oriented radiological investigation. Neurological events occurring during childhood (in both children and adults of the series) and adulthood were recorded. One or several neurological manifestations have been observed in 55% of patients (adults and children) (n = 87). These included: headache (28 patients); hydrocephalus (7); epilepsy (5); lacunar stroke (1); white matter disease (1); intraspinal neurofibroma (3); facial palsy (1); radiculopathy (5); and polyneuropathy (2). Tumours included: optic pathway tumours (20); meningioma (2); cerebral glioma (3); and malignant peripheral nerve sheath tumours (6). Life-threatening complications were observed in five adults and included four malignant peripheral nerve sheath tumours and one meningioma. Pain was the leading symptom in 11 adults and was related to malignant peripheral nerve sheath tumours, complications of intraspinal neurofibromas, subcutaneous neurofibromas and peripheral nerve neurofibromas. NF1 in adults was not associated with other disabling or life-threatening neurological complications. Symptomatic optic pathway tumours, cerebral gliomas, symptomatic aqueductal stenosis and spinal compression due to intraspinal NF were observed exclusively during childhood. In this series, the predominant neurological features of adults with NF1 were chronic pain and malignant peripheral nerve sheath tumours.
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PMID:Neurological complications of neurofibromatosis type 1 in adulthood. 1009 56


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