1/10. Cutaneous collagenous vasculopathy with generalized telangiectasia: an immunohistochemical and ultrastructural study.We report a 54-year-old male, with a 5-year history of spreading asymptomatic generalized cutaneous telangiectases. The patient had no mucosal or nail involvement, no positive family history and no clinical evidence of systemic disease or bleeding diathesis. Histologically, the superficial small dermal blood vessels were dilated and showed thickened walls with hyaline perivascular material, staining as collagen. The vessel walls were PAS and colloidal iron stain positive, and immuno-histochemically lacked actin staining. collagen IV, fibronectin and laminin antibodies showed the material deposited around the basement membrane zone. Ultrastructurally, the vessels were post-capillary venules (PCV) and showed marked collagen deposition around the basal lamina. There were many abnormally banded widely spaced fibres with 100-150 nm periodicity (Luse bodies), in addition to regular banded collagen. pericytes were sparse and lacked intracytoplasmic filaments, and few veil or fibroblastic cells were seen embedded within the collagen. We believe this is a form of cutaneous microangiopathy not previously described, with distinct morphology and unique ultrastructural features. It may be due to a genetic defect with erroneous production of disorganized collagen in the cutaneous microvasculature. Dermatologists and Dermatopathologists should be aware of this unusual cutaneous vasculopathy.- - - - - - - - - - ranking = 1keywords = fibre (Clic here for more details about this article) |
2/10. Complete segmental resection of the spine, including the spinal cord, for telangiectatic osteosarcoma: a report of 2 cases.STUDY DESIGN: Two case reports of telangiectatic osteosarcoma treated with complete segmental resection of the spine, including the spinal cord. OBJECTIVES: To report the en bloc tumor excision, including the spinal cord, for telangiectatic osteosarcoma, and discuss the indication of cord transection and influence after cutting the spinal cord. SUMMARY OF BACKGROUND DATA: To our knowledge, there are no previous reports describing telangiectatic osteosarcoma of the spine and the subsequent en bloc excision of the spine, including the spinal cord. methods: The clinical and radiographic presentations of 2 cases with telangiectatic osteosarcoma are presented. Because these 2 cases already had complete paralysis for at least 1 month, it was suspected that there was no possibility of recovering spinal cord function. Complete segmental spinal resection (total en bloc spondylectomy) was performed. At that level, the spinal cord was also cut and resected. RESULTS: En bloc excision of the tumor with a wide margin was achieved in both cases. In the resected specimen, the nerve cells in the spinal cord had lapsed into degenerative necrosis. The pathologic findings showed that there was no hope for recovery of spinal cord function. CONCLUSION: En bloc spinal resection, including the spinal cord, is an operation allowed when there is no hope for recovery of spinal cord function. This surgery should be accepted as an option in spine tumor surgeries.- - - - - - - - - - ranking = 0.054611387832715keywords = nerve (Clic here for more details about this article) |
3/10. Peripheral nerve involvement in ataxia telangiectasia: histological and ultrastructural studies of peroneal nerve biopsy in two cases.The authors report a histological and electron-microscopic study of the peripheral nerve in two cases of ataxia-telangiectasia showing fiber loss, storage material in schwann cells and nuclear changes. Nuclear changes are the most typical finding and are correlated with the primary metabolic disorder of dna repair.- - - - - - - - - - ranking = 0.49150249049443keywords = nerve (Clic here for more details about this article) |
4/10. Non-hereditary multiple telangiectasias of the central nervous system. Report of two clinicopathological cases.We describe 2 clinicopathological cases of non-hereditary multiple telangiectasias of the nervous system. In the first case, the general course of the disease was characterized by spells over a period of 7 years and the major lesions successively involved cranial nerves, spinal cord and brain. Neuropathological examination showed both ischemic and hemorrhagic changes. Systematized degenerative changes were found and were similar to those observed in spinocerebellar heredodegenerations (Friedreich's ataxia). In the second case, the 3 years of evolution were characterized by spells of the encephalitic type only. A review of the literature indicates the rarity of this kind of disease, the usual localization of capillary malformations and the clinical polymorphism (epilepsy, strokes, multifocal syndromes masquerading as multiple sclerosis). The pathogenesis of pathological changes is discussed.- - - - - - - - - - ranking = 0.054611387832715keywords = nerve (Clic here for more details about this article) |
5/10. Atypical retinitis proliferans, retinal telangiectasis, and vitreous hemorrhage in a patient with tuberous sclerosis.This report describes an unusual case of recurrent vitreous hemorrhage and atypical retinal neovascularization in a patient with tuberous sclerosis. During three years of observation, the patient also developed retinal telangiectasis with macular edema and lipid exudation. Although the patient did not have an obvious astrocytic hamartoma, a diffuse, flat retinal hamartoma within the nerve fiber layer was suspected.- - - - - - - - - - ranking = 0.054611387832715keywords = nerve (Clic here for more details about this article) |
6/10. radiation retinopathy after irradiation of intracranial lesions.Six patients developed a mild retinopathy after radiation therapy for intracranial lesions. The retinopathy, consisting of capillary microaneurysms, cotton-wool patches, and telangiectasis, developed after a latent period that varied between nine months and three years. The visual acuity remained within normal limits. The severity of the retinopathy is related to the retinal or optic nerve dose of radiation.- - - - - - - - - - ranking = 0.054611387832715keywords = nerve (Clic here for more details about this article) |
7/10. Acute disc swelling in juvenile diabetes. Clinical profile and natural history of 12 cases.Acute disc swelling was documented in 21 eyes of 12 patients with long-standing juvenile diabetes. All but one patient were in the second or third decade of life, with a 13-year average duration of diabetes. Seventeen eyes had initial acuity of 20/50 or better, including nine eyes with 20/25 or better; disc swelling was asymptomatic in six eyes. Simultaneous bilateral disc swelling occurred in seven patients. With no specific therapy, vision generally recovered to normal levels within a few weeks, but a few patients retained arcuate, nerve fiber bundle, field defects and optic atrophy. There was no positive correlation with the degree of diabetic retinopathy, and disc swelling did not seem to be a harbinger of progressive retinopathy or proliferation at the nerve head. Disc swelling in juvenile diabetics represents a distinct clinical entity that must be distinguished from other causes of acquired nerve head elevation, especially papilledema of increased intracranial pressure.- - - - - - - - - - ranking = 0.16383416349814keywords = nerve (Clic here for more details about this article) |
8/10. Myelinated nerve fibers and retinal vascular abnormalities.BACKGROUND/PURPOSE. Abnormal retinal vessels may develop in a region of myelinated nerve fibers, and these vessels may cause vitreous hemorrhages. methods. The clinical histories of seven patients with retinovascular abnormalities in a patch of myelinated nerve fibers are presented. None of the reported patients had other evidence of systemic disease. The cases were traced by a multicentric retrospective study. RESULTS. Retinal vascular abnormalities ranged from mild telangiectasis to frank neovascularization, with or without obstruction of the capillary network and signs of branch artery and vein occlusion. Age at diagnosis ranged from 15 to 68 years. Vitreous hemorrhages occurred in the four youngest patients and occurred at 15, 27, 27, and 43 years of age. Laser photocoagulation was applied in three patients and vitrectomy was performed in one. CONCLUSION. The authors' findings suggest that the abnormal structure of the myelinated nerve fibers and the thickened nerve fiber layer of the affected portions of retina may play a role in the onset of retinal vascular abnormalities and eventually cause telangiectasis, branch artery and vein occlusion, neovascularization, and vitreous hemorrhages. This suggestion is based on the absence of other causes of neovascularization or vitreous hemorrhage in all seven patients, and on the relatively young age of four of the patients with this association.- - - - - - - - - - ranking = 0.43689110266172keywords = nerve (Clic here for more details about this article) |
9/10. Peripapillary fluorescein leakage in 11778 Leber's optic neuropathy.A 17-year-old young man presented with a 2-month sequential visual loss in both eyes. Vision was 20/200 in the right eye and 20/400 in the left eye. Both discs were hyperemic with circumpapillary telangiectasia and nerve fiber swelling. fluorescein angiography revealed a fuzzy hyperfluorescence due to leakage from dilated capillaries on both discs in the late transit. Molecular mitochondrial DNA study confirmed a homoplasmic 11778 Leber's optic neuropathy.- - - - - - - - - - ranking = 0.054611387832715keywords = nerve (Clic here for more details about this article) |
10/10. MRI findings in macrocephaly-cutis marmorata telangiectatica congenita.We describe a child with macrocephaly-cutis marmorata telangiectatica congenita (M-CMTC), cherry red macules, megalencephaly with hemifacial and segmental overgrowth, macrosomia, and cutis marmorata telangiectasia congenita of the trunk, and visceral and subcutaneous cavernous hemangiomas. The megalencephaly is accompanied by MRI findings of CNS dysgenesis with protrusion of the cerebellar tonsils through the foramen magnum (Chiari I), lumbar syrinx, and hydrops of the optic nerves. The report of this additional patient further confirms the newly described macrocephaly-cutis marmorata telangiectatica congenita as a distinct clinical phenotype.- - - - - - - - - - ranking = 0.054611387832715keywords = nerve (Clic here for more details about this article) |
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