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)

There are four previously reported cases of post-irradiation calcification in the basal ganglia as demonstrated on the conventional skull roentgenograms. We have described two additional cases with grey matter calcification, which were demonstrated 10 and 14 yr after radiation therapy for an optic glioma and a medulloblastoma, respectively. The calcification was clearly demonstrated on CT scan, although it was not apparent on the skull roentgenogram. The extract pathogenesis of this condition is not clear. It appears, however, to be related to radiation vasculitis of the small vessels of the brain with resultant hyalinization and calcification. A long-term follow up study would be necessary to evaluate the significance and implication of post-irradiation calcification of the grey matter. CT is the most sensitive method of demonstrating the intracranial calcification in vivo.
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PMID:CT evidence of grey matter calcification secondary to radiation therapy. 61 95

Cerebral vasculitis is an unusual disorder with numerous causes. One such entity, noninfectious granulomatous angiitis of the nervous system (GANS), is an extremely rare disease with a predilection for leptomeningeal and parenchymal arteries and veins. Isolated involvement of the central nervous system is characteristic of GANS, which has also been referred to as primary angiitis of the central nervous system (PACNS). The results of magnetic resonance (MR) imaging and angiography in seven patients with presumed PACNS were retrospectively analyzed and correlated. MR images were positive in every case. Characteristically, lesions were multiple, bilateral, and supratentorial. Both gray- and white-matter infarcts were identified in four of seven patients; infarcts were most common in the deep white matter. PACNS can also appear as primary parenchymal hemorrhage or simulate low-grade glioma. All lesions identified on MR images were associated with positive angiographic findings of cerebral vasculitis in the corresponding vascular distribution. However, for 12 of 33 vascular distributions with angiographic evidence of cerebral vasculitis, no lesions were identified on MR images. These correlative observations suggest that some patients with proved PACNS may have normal MR imaging results.
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PMID:Cerebral vasculitis: MR imaging and angiographic correlation. 172 11

In this report we describe a patient with a benign glioma treated with surgery and radiation. After a period of stability he developed subacute bacterial endocarditis, and deteriorated neurologically. Computed tomographic scans did not show recurrent tumor. An angiogram showed vasculitis restricted to the previously irradiated area. Secondary to subacute bacterial endocarditis was the presence of high levels of circulating immune complexes. His neurological status was unchanged after antibiotics, but improved after treatment with dexamethasone. We interpret the clinical course as an immune-complex-mediated vasculitis superimposed on a subclinical radiation vasculitis. This case supports the hypothesis that immune mechanisms may be involved in delayed radiation injury to the nervous system.
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PMID:Focal cerebral vasculitis associated with circulating immune complexes and brain irradiation. 372 12

A case of cerebral granulomatous angiitis of the left temporal lobe mimicking a brain tumour is presented. Following surgical removal of a glioma-imitating mass, histological examination disclosed a granulomatous vasculitis. Treatment consisted of dexamethasone given in tapering doses for 5 days after surgery. Clinical and morphological differences from other reported cases of granulomatous angiitis of the nervous system are described.
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PMID:Cerebral granulomatous angiitis with atypical features. 671 9

Erythema elevatum diutinum (EED) is a rare disease presenting with persistent red to yellow-brown papules or plaques which are mainly localized symmetrically on the extensor aspects of the hands and fingers, the elbows and the knees. The histology shows a leucocytoclastic vasculitis in early lesions and fibrosis of the dermis later on. Dapsone is the treatment of choice. Today, EED is usually assigned to the neutrophilic dermatoses in which an association with hematological disturbances is well documented. We report on a patient with EED and glioma WHO grade IV, a coincidence unreported hitherto. Additionally, we review the literature on diseases associated with EED.
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PMID:[Erythema elevatum diutinum. A rare dermatosis with a broad spectrum of associated illnesses]. 917 57

A 50-year-old Chinese woman with a chronic 20-year history of ataxic gait associated with dry eyes and mouth, was admitted to hospital after a single episode of syncope. Magnetic resonance imaging scans showed a large left frontal hypodense lesion suggestive of a glioma. Craniotomy was performed and the lesion excised, with histology showing only infarcted tissue and no malignant cells. Further diagnostic evaluation revealed that the patient had primary Sjogren's syndrome, with demyelinating polyneuropathy. In the absence of risk factors for stroke, it was considered likely that the cerebral infarct was secondary to autoimmune-related vasculitis. Functional neuroimaging, such as magnetic resonance spectroscopy, should be considered in evaluating doubtful or unusual brain lesions in patients with autoimmune disease.
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PMID:Cerebral infarct mimicking glioma in Sjogren's syndrome. 1216 35

A 27-year-old woman was referred to our hospital with mild disorientation, bilateral abducens nerve palsy, and mild left hemiparesis. Magnetic resonance (MR) imaging revealed diffuse mass lesions resembling malignant glioma in the right frontal intraparenchymal region, with enhancement of multiple meningeal and intraparenchymal nodules. Partial resection of the frontal lesion was performed. Histological examination revealed that the specimens consisted of brain tissue, with marked perivascular infiltration of histiocytes and sheets of xanthomatous cells. The diagnosis was primary cerebral angiitis containing marked xanthoma cells. Steroid therapy was administered over 1 week. MR imaging showed that the remaining lesions resolved gradually, and had disappeared 2 years after surgery. No neurological symptoms or recurrence of the tumor has been observed during the 6-year period since the operation.
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PMID:Primary cerebral angiitis containing marked xanthoma cells with massive intraparenchymal involvement--case report--. 1578 8

Primary granulomatous angiitis of the central nervous system (CNS) is extremely rare. Its preoperative diagnosis is difficult as the condition displays nonspecific features on routine neuroimaging investigations. In this paper, the authors report findings of magnetic resonance (MR) spectroscopy and fractional anisotropy (FA) with diffusion tensor MR imaging in a case of granulomatous angiitis of the CNS. A 30-year-old man presented with morning headaches and grand mal seizures. An MR image revealed a mass resembling glioblastoma in the right temporal lobe. Magnetic resonance spectroscopy showed a high choline/creatine (Cho/Cr) ratio indicative of a malignant neoplasm, accompanied by a slight elevation of glutamate and glutamine. The FA value was very low, which is inconsistent with malignant glioma. The mass was totally removed surgically. Histologically, the peripheral lesion of the mass consisted of a rough accumulation of fat granule cells, infiltration of inflammatory cells, and distribution of capillary vessels. Some vessels within the lesion were replaced by granulomas. The histological diagnosis was granulomatous angiitis of the CNS. The MIB-1-positive rate of the granuloma was approximately 5%. Both MR spectroscopy and FA were unable to accurately diagnose granulomatous angiitis of the CNS prior to surgery; however, elevated Cho/Cr and glutamate and glutamine shown by MR spectroscopy may indicate the moderate proliferation potential of the granuloma and the inflammatory process, respectively, in this condition. Although the low FA value in the present case enabled the authors to rule out a diagnosis of glioblastoma, FA values in inflammatory lesions require careful interpretation.
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PMID:Primary granulomatous angiitis of the central nervous system: findings of magnetic resonance spectroscopy and fractional anisotropy in diffusion tensor imaging prior to surgery. Case report. 1793 38

We report four cases of biopsy-proven B-cell-rich primary angiitis of the central nervous system (PACNS). The mean age of the patients was 29 years (range, 23-37 years). The patients suffered from unilateral weakness (n = 2), seizure (n = 1), and hypersomnia, anorexia and confusion (n = 1). The vital signs and the results of laboratory tests were within normal limits in all the four cases except erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). ESR was elevated in one patient and CRP was elevated in two patients. The magnetic resonance imaging (MRI) scans revealed single (n = 2) or multiple (n = 2) irregularly enhancing lesions. Radiological studies initially indicated tumors such as glioma (n = 2) or lymphoma (n = 1), except in one case, in which the radiological analysis indicated vasculitis or demyelinating disease. All the cases involved both medium-sized (50-250 microm in diameter) and small-sized vessels (20-49 microm in diameter). The vascular, perivascular and parenchymal lymphocytes were polymorphous; however, CD20-positive B-cells were predominated in blood vessels while the CD8-positive T-cells infiltrated predominantly in brain parenchyma. Therefore, our patients revealed B-cell dominant lymphocytic vasculitis. Two patients who underwent active treatment (corticosteroid alone or with cyclophosphamide) showed remarkable clinical and radiological improvement but two patients still have initial neurological symptoms, namely, confusion and newly developed seizures, respectively, during the 19-101-month follow-up periods; this effect can be attributed to irreversible brain damage. Therefore, although early brain biopsy may be associated with histopathologic diagnostic pitfalls, it is a mandatory procedure for obtaining a confirmative diagnosis as well initiating early therapy, thereby reducing brain damage.
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PMID:B-cell dominant lymphocytic primary angiitis of the central nervous system: four biopsy-proven cases. 1973 59

Herein, we report the case of a 72-year-old male with an exceedingly rare manifestation of a low-grade lymphoma in the brain associated with light chain deposition disease (LCDD). The patient presented with epileptic seizures. Magnetic resonance imaging (MRI) of the brain revealed multiple hyperintense lesions in the right parietal lobe that were suspicious of vasculitis, low-grade glioma, or neurosarcoidosis. In the cerebrospinal fluid (CSF), but not in the serum, highly elevated IgG was found. A stereotactic biopsy of one cerebral lesion was performed. Histopathology revealed a low grade lymphoplasmacytic B-cell lymphoma with light chain deposition disease (LCDD). Bone marrow biopsy and laboratory workup did not show any systemic involvement. LCDD exclusively affecting the brain is an exceedingly rare finding. It can be associated with low-grade B-cell lymphoma. This is the first report of LCDD exclusively affecting the brain in an elderly patient. Compared with the two younger patients previously reported, the course of the disease was of a slow-evolving nature. In constellations of highly elevated IgG in CSF and multiple white matter lesions, LCDD should be considered as underlying pathology.
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PMID:Cerebral low-grade lymphoma and light chain deposition disease: exceedingly high IgG levels in the cerebrospinal fluid as a diagnostic clue. 2107 42


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