1/12. Vasculopathic changes of cadasil can be focal in skin biopsies.Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (cadasil) is a newly described cause of vascular dementia. Pathologic examination shows multiple small infarcts in the deep cerebral white matter together with a nonatherosclerotic, nonamyloid angiopathy involving the media of small cerebral arteries. Ultrastructurally, characteristic granular material is present in the basal lamina of vascular smooth muscle cells in cerebral and extracerebral blood vessels. The ultrastructural changes have also been demonstrated in skin biopsies of affected patients; consequently, some investigators have recently recommended skin biopsies for the diagnosis of cadasil. This study describes a 54-year-old male with a family history for strokes who had clinical and radiological features suggestive of cadasil. A skin biopsy was performed to confirm the diagnosis. Initially, the characteristic vasculopathic changes of cadasil were not identified within small blood vessel walls. However, multiple deeper sections in other areas showed electron-dense material associated with vascular smooth muscle cells, characteristic of cadasil. Subsequent genetic testing demonstrated a single nucleotide substitution at position 659 on chromosome 19p13.1 causing an amino-acid change (Cys --> Phe), a finding indicative of cadasil. The involvement of blood vessels within the dermis makes skin biopsy a useful adjunct in the diagnosis of cadasil. However, as illustrated by this case, the findings may be focal, requiring a thorough evaluation of the entire biopsy specimen.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/12. Late-onset neuroaxonal leucoencephalopathy with spheroids and vascular amyloid.A 54-year-old man with a 7-year history of early-onset, slowly progressive dementia and motor impairment characterised by diffuse, non-enhancing white matter signal change. Neuropathologic examination demonstrated subcortical pigmentation with neuroaxonal spheroid formation, profound axonal loss with secondary myelin degeneration and widespread betaA4 immunopositivity involving meningeal and subcortical vessels. There was relative sparing of brain stem and cerebellar white matter. The neocortex was normal. The appearances expand the spectrum of adult-onset neuroaxonal leucoencephalopathy with spheroids (NALS) and demonstrate that vascular betaA4 amyloid plays a key role in the white matter pathology of NALS.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
3/12. Neoplastic angioendotheliomatosis (NAE) of the CNS in a patient with AIDS subacute encephalitis, diffuse leukoencephalopathy and meningo-cerebral cryptococcosis.A 12-year-old, hemophilic boy died with acquired immune deficiency syndrome (AIDS) after a clinical course characterized by progressive psycho-organic syndrome and opportunistic infections. Postmortem neuropathological examination revealed a cerebral form of neoplastic angioendotheliomatosis (NAE), leukoencephalopathy, giant cell encephalitis and meningo-cerebral cryptococcosis. The most unusual finding was the presence of proliferated neoplastic cells within lumina of some blood vessels throughout the central nervous system (CNS). These cells displayed cytologic features of malignancy and stained positively for common leukocyte antigen. Coronal sections showed diffuse cerebral and cerebellar leukoencephalopathy with most pronounced loss of myelin and axons in deep white matter, while the subcortical arcuate fibers and the corpus callosum were partially spared. In these areas numerous small foci of severe myelin loss were present. Microglial nodules and distinctive multinucleated giant cells (MGC) were numerous. Intracytoplasmic and intranuclear acidophilic inclusions were found in a few multinuclear and mononuclear cells. Close contact between mononuclear and multinuclear cells suggesting their fusion was also observed. As far as we know this is the first case of NAE encountered in AIDS, one of the rare primary cerebral forms and the youngest reported case of NAE up to now. This case could be considered as one proof more that NAE is a special form of malignant lymphoma.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
4/12. Leukoencephalopathy in childhood leukemia.A 14-year-old boy survived for 7 years after the initial diagnosis and treatment of acute lymphocytic leukemia. Neurologic deterioration occured repeatedly throughout his complicated clinical course but it was most severe and only partially reversible following orally administered pyrimethamine. The neuropathologic lesions were distinctive and included a diffuse reactive astrocytosis, axonal degeneration, status spongiosis, circumscribed foci of demyelination and coagulative necrosis, and mural thickening with luminal narrowing of microcirculatory vessels. This collection of findings represents the leukoencephalopathy of childhood leukemia that we and others believe results in large part from the combined effects of cranial irradiation and chemotherapy. The role of folic acid antagonists, namely methotrexate and pyrimethamine, are particularly noteworthy in this regard.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
5/12. Hereditary retinal vasculopathy with cerebral white matter lesions.We report on a syndrome of progressive visual loss and leukoencephalopathy affecting several relatives. Affected individuals had evidence of retinal vasculopathy on fluorescein angiogram and periventricular white matter lesions on brain magnetic resonance imaging. The clinical manifestations in this family suggest transmission of an autosomal dominant vasculopathy affecting both retinal and intracranial vessels.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
6/12. An autopsy case of multiple leucoencephalitis showing hemiatrophy of the brain.A 39-year-old female died 25 years after the onset of epileptic seizure. In the clinical course, she suffered epileptic seizures, left hemiparesis and muscular atrophy of the left upper extremity, psychological character change, dementia, forced laughter and hallucinatory and delusional states. Pathologically, severe changes were found in the right hemisphere consisting of lesions of white matter with myelin loss, especially in the frontal, temporal and occipital lobes. gliosis was diffusely observed in the white matter excepting areas with severe changes. In some parts of the cortex, the cytoarchitecture was destroyed and in this area, blood vessels numerously increased. In the left occipital white matter, perivascular infiltration mainly consisting of lymphocytes was observed with numerous fat granule cells and gemystocytic astrocytes around them. The present case may belong to the category of multiple leucoencephalitis, however, it had many characteristic features, such as a long clinical course (25 years) and pathologically revealed hemiatrophy of the brain.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
7/12. Leukoencephalopathy in Waldenstrom's macroglobulinemia. Immunohistochemical and electron microscopic observations.The clinical and pathologic features of a case of Waldenstrom's macroglobulinemia with leukoencephalopathy are reported. Multiple cerebral foci of demyelination, accompanied to a lesser extent by axonal degeneration, were associated with perivascular infiltrates of plasmacytoid lymphocytes and with permeation of the white matter by macroglobulins. Immunohistochemical studies demonstrated a predominance of IgM kappa within the blood plasma, in cerebral blood vessel walls, in the foci of demyelination, and within perivascular histiocytes. Electron microscopy disclosed the presence, in macrophages and pericytes, of membrane-bound cytoplasmic inclusions consisting of tubular arrays, suggestive of cryoglobulin deposits. We hypothesize that the high serum levels of macroglobulins accompanied by lymphoplasmocytic infiltrates may, either by way of viscosity-related ischemia, or by a direct toxic effect, have caused abnormal vascular permeability, infiltration of the cerebral parenchyma by paraproteins, and, ultimately, focal degeneration of the white matter.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
8/12. Vascular changes of methotrexate-related disseminated necrotizing leukoencephalopathy.We investigated cerebral lesions of methotrexate (MTX)-related disseminated necrotizing leukoencephalopathy (DNL) in two autopsy cases of leukemia by the reconstruction technique of the serial sections and thick sections (700-800 microns) stained with silver impregnation to detect the topographic relation between the vascular changes and parenchymal lesions. We revealed the vascular changes, such as fibrinoid degeneration, hyalinized thickening of the vascular wall, dilatation of lumen and stenosis due to swelling of the endothelial cells and exsudation in the wall, particularly prominent in venules and capillaries of venous side in the territory of the superficial medullary veins. There were no remarkable changes in the arteries, except for the moderate endothelial swelling of arteriolar capillaries. The parenchymal lesions were topographically associated with these vascular changes, and the small necrotic foci confluented each other and formed large irregular necrotic foci. We considered that the DNL may be ascribed to dyshoric damage of the veins and capillaries of the venous side and that the mechanism of vascular injury was probably due to the prolonged direct action of intrathecal MTX on the vessels.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
9/12. Progressive multifocal leukoencephalopathy in a patient with rheumatoid arthritis and polymyositis.We describe a young woman who developed postpartum rheumatoid arthritis, polymyositis and small vessel cerebrovascular disease. Subsequently she developed biopsy proven progressive multifocal leukoencephalopathy (due to jc virus infection) which may have been a complication of her immunosuppression. This rare complication of autoimmune rheumatic diseases is discussed.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
10/12. pneumocystis carinii thyroiditis diagnosis by fine needle aspiration cytology: a case report.BACKGROUND: Extrapulmonary infection or dissemination of pneumocystis carinii (PC) is rare, but under certain conditions the parasite can spread via the bloodstream or lymphatic vessels. Systemic pneumocystosis most often involves the lymph nodes, stomach, spleen, liver, skin, pancreas, choroid and eye. Isolated lesions containing PC have also been identified in the thyroid. CASE: A 41-year-old homosexual male infected with the human immunodeficiency virus (hiv) developed a PC infection in the thyroid gland. The patient had had thrush and anal herpes since being diagnosed as hiv positive in 1984. In 1992 the patient developed a mass in the area of the right lobe of the thyroid gland. Smears from fine needle aspiration cytology of the thyroid mass revealed epithelioid cells. However, a cell block revealed numerous PC organisms on Gomori methenamine-silver stain; that finding was confirmed by an excisional biopsy. The patient had not previously been diagnosed with PC pneumonia. CONCLUSION: Our case of thyroid involvement with PC expands the clinical spectrum of extrapulmonary pneumocystosis in patients with the acquired immunodeficiency syndrome. We believe that in these patients the incidence of Pneumocystis thyroiditis will continue to rise and be reported.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
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