11/247. Primary chronic osteomyelitis associated with synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO syndrome).Diffuse sclerosing osteomyelitis may indicate the mandibular localisation of the SAPHO syndrome. Twelve patients with diffuse sclerosis of the mandible were examined for symptoms of the SAPHO syndrome. Nine patients were found to have primary chronic osteomyelitis and eight of these represented a SAPHO syndrome. Results in this series support the hypothesis of an association between primary chronic osteomyelitis and the SAPHO syndrome.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
12/247. White-matter change in mesial temporal sclerosis: correlation of MRI with PET, pathology, and clinical features.PURPOSE: To assess the magnetic resonance imaging (MRI), positron emission tomography (PET), pathology, and clinical findings of patients with the MRI feature of white-matter change (WMC) in the anterior temporal lobe. methods: Fifty-six patients with pathologically proven mesial temporal sclerosis were included in this study. MRI and 18F-2-deoxyglucose-(FDG) PET images were obtained before surgery in all patients. The patients were divided into two groups according to the presence of WMC on their MRI. WMC consists of an indistinct gray-white matter demarcation and an increased signal intensity of the anterior temporal lobe on T2-weighted images. The two groups were then compared in terms of MRI, PET, pathology, and clinical features. RESULTS: The MRI feature of WMC was observed in 18 (32%) of the 56 patients. PET images of those patients revealed more severe hypometabolism of the ipsilateral temporal lobes (p< 0.05). In terms of histologic findings, larger numbers of heterotopic neurons were observed in the anterior temporal lobe white matter of these patients who also shared the following clinical features: earlier seizure onset, frequent history of febrile convulsions, and favorable surgical outcomes. CONCLUSIONS: The MRI feature of WMC is an additive sign for correct seizure lateralization and may be related to a favorable surgical outcome in patients with temporal lobe epilepsy.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
13/247. Ictal vomiting in partial seizures of temporal lobe origin.We report 3 cases presenting ictal vomiting during partial seizures of temporal lobe origin. Two patients had complex partial seizures accompanying vomiting characteristics. Ictal vomiting occurred early in the course of the seizure when rhythmic discharges involved predominantly the left hemisphere, the language dominance hemisphere. The other patient had ictal vomiting in simple partial seizures which originated from the right temporal lobe or the language nondominant side. All 3 patients underwent anterior temporal lobectomy with promising outcomes. Pathologic diagnosis included hippocampal sclerosis in 2 patients and astrocytoma in 1 patient. In our patients, ictal vomiting does not lateralize temporal lobe epilepsy and is not specific to pathology.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
14/247. Hippocampal sclerosis: development in adult life.Hippocampal sclerosis (HS) is the most common pathological lesion underlying intractable temporal lobe epilepsy. It is not known whether HS exists before the onset of epilepsy or whether it is caused by seizures. Its has been proposed that childhood seizures cause HS. Optimized magnetic resonance imaging (MRI), hippocampal volumes and T(2) signal quantitation were performed 2 weeks and 8 months following at tonic-clonic seizure in a 23-year-old man. MRI 14 days after the seizure showed symmetrical hippocampal volumes (ratio R/L = 1.03) with intact internal architecture bilaterally but marked signal change in the right hippocampus (T(2) right = 121, T(2) left = 103, normal < or = 108 ms). Eight months later this hippocampus showed severe atrophy with a volume ratio of 0.65 and T(2) values of 117 (right) and 109 ms (left). High-resolution imaging showed that volume loss occurred mainly in the CA1 region which showed high signal in the initial study. Characteristic MRI features of HS can develop in adults and HS cannot always be assumed to have its origins in childhood. Hypoxia in the context of seizures may be an important component in hippocampal damage. HS may be a preventable lesion and MRI signal change seen in the neuronal layers of the hippocampus may be an indication for neuroprotection. copyright copyright 1999 S. Karger AG, Basel- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
15/247. [11C](R)-PK11195 positron emission tomography imaging of activated microglia in vivo in Rasmussen's encephalitis.This study was designed to explore the feasibility of PET using [11C](R)-PK11195 as an in vivo marker of activated microglia/brain macrophages for the assessment of neuroinflammation in Rasmussen's encephalitis (RE). [11C](R)-PK11195 PET was carried out in four normal subjects, two patients with histologically confirmed RE, and three patients with clinically stable hippocampal sclerosis and low seizure frequency. Binding potential maps showing specific binding of [11C](R)-PK11195 were generated for each subject. Regional binding potential values were calculated for anatomically defined regions of interest after coregistration to and spatial transformation into the subjects' own MRI. In one patient with RE who underwent hemispherectomy, the resected, paraffin-embedded brain tissue was stained with an antibody (CR3/43) that labels activated human microglia. Whereas specific binding of [11C](R)-PK11195 in clinically stable hippocampal sclerosis was similar to that in normal brain, patients with RE showed a focal and diffuse increase in binding throughout the affected hemisphere. In RE, [11C](R)-PK11195 PET can reveal in vivo the characteristic, unilateral pattern known from postmortem neuropathologic study. PET imaging of activated microglia/brain macrophages offers a tool for investigation of a range of brain diseases where neuroinflammation is a component and in which conventional MRI does not unequivocally indicate an inflammatory tissue reaction. [11C](R)-PK11195 PET may help in the choice of appropriate biopsy sites and, further, may allow assessment of the efficacy of antiinflammatory disease-modifying treatment.- - - - - - - - - - ranking = 2keywords = sclerosis (Clic here for more details about this article) |
16/247. nephrotic syndrome and end-stage renal disease with WT1 mutation detected at 3 years.We report a boy who presented at 3 years with nephrotic syndrome and end-stage renal failure. Although histopathological findings showed end-stage kidney, isolated diffuse mesangial sclerosis (IDMS) was suspected because of his clinical course, and was confirmed by the presence of WT1 (wilms tumor suppressor gene) mutation. He did not have ambiguous genitalia or wilms tumor. The karyotype was 46:XY. A constitutional mutation in exon 7 (953G-->A, 312Arg-->Gin) was detected. A few cases of male IDMS, associated with WT1 mutations, have been reported. We believe that investigation for the WT1 mutation should be performed not only in denys-drash syndrome and IDMS, but also in end-stage renal disease with unexplained nephrotic syndrome of early onset. WT1 mutation-associated nephrotic syndrome has an increased risk of wilms tumor. Careful ultrasound evaluations or bilateral nephrectomies are indicated.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
17/247. Topical treatment of cutaneous chronic graft versus host disease with halofuginone: a novel inhibitor of collagen type i synthesis.BACKGROUND: In chronic graft-versus-host disease (cGvHD), skin fibrosis, contractures, and an increase in collagen content form the hallmark. We report a successful treatment of a cGvHD patient by topical application of halofuginone, an inhibitor of collagen alpha1(I) gene expression. methods: Halofuginone-containing ointment was applied daily on the left side of the neck and shoulder of a cGvHD patient. Collagen alpha1(I) gene expression and collagen content in skin biopsy specimens were evaluated by in situ hybridization and sirius red staining, respectively. RESULTS: After 3 and 6 months, a marked reduction in skin collagen synthesis was observed, accompanied with increase neck rotation on the treated side. After cessation of treatment, the sclerosis, skin tightness, and collagen alpha1(I) gene expression returned to baseline level. No adverse effects were observed, and no plasma levels of halofuginone could be detected. CONCLUSIONS: Halofuginone may provide a promising novel and safe therapy for cGvHD patients.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
18/247. Giant cell angiofibroma of the buccal mucosa.We report a case of a patient with a recently described entity (giant cell angiofibroma) occurring in the oral cavity. A submucosal solid tumor arising in the right buccal mucosa of a 46-year-old woman was histologically characterized by a patternless spindle-cell proliferation within a generally myxoid stroma containing areas of perivascular sclerosis. Numerous multinucleated giant cells of the floret type were present. Immunohistochemically, the tumor cells and multinucleated giant cells exhibited positivity for CD34. To the best of our knowledge, this is the first report of a giant cell angiofibroma arising in the oral cavity.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
19/247. Histopathological findings of X-linked retinoschisis with neovascular glaucoma.BACKGROUND: X-linked retinoschisis (XLRS) is rarely complicated by neovascular glaucoma. Only a few reports of XLRS histopathological findings with neovascular glaucoma have been published. methods: A 41-year-old man with XLRS complicated by neovascular glaucoma in his left eye was examined with electroretinography, B-scan, ultrasound biomicroscopy and computed tomography. He was examined by ophthalmoscopy and fluorescein angiography in the other eye. An enucleation was performed in his left eye due to uncontrollable high intraocular pressure and persistent ocular pain. We examined the enucleated eye histopathologically. RESULTS: Examination of the enucleated eye showed nuclear sclerosis of the lens, pigmented retrolental membrane and retinoschisis which separated the inner layer of the retina and made a large space in the vitreous cavity without any apparent detachment of the outer layers of the retina. Sclerotic vessels were present histopathologically in both the inner and outer layers of the retina. There was a peripheral anterior synechia, ectropion uveae and a fibrovascular membrane, which contained many lumina of neovascularization, indicating marked rubeosis iridis. Small cystic spaces were observed in both the schitic retina in the peripheral region and the foveal schisis at the outer layer of the retina. The photoreceptor cells had become markedly atrophied and multiple regions of calcification were observed. The optic nerve showed severe atrophy with gliosis, but the central retinal artery and vein were still open within the nerve. CONCLUSIONS: These histopathological findings suggest that rubeosis iridis may have developed secondarily to retinal ischemia due to occlusion of the retinal blood vessels.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
20/247. Idiopathic sclerotic inflammation of the orbit with left optic nerve compression in a patient with multifocal fibrosclerosis.We present the MR imaging findings in a 43-year-old male patient with bilateral idiopathic sclerosing inflammation of the orbit. Bilateral enhancing retrobulbar masses, with concentric compression of the retrobulbar segment of the left optic nerve, were seen. MR imaging proved to be the only means to distinguish between the different intraorbital structures and to determine the exact site of optic nerve compression. To our knowledge, this is the first documented case of MR imaging findings of this entity.- - - - - - - - - - ranking = 4keywords = sclerosis (Clic here for more details about this article) |
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