Cases reported "Disease Progression"

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1/134. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.

    The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.
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ranking = 1
keywords = multiple sclerosis, sclerosis
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2/134. University of Miami Division of Clinical pharmacology Therapeutic Rounds: ischemic renal disease.

    Ischemic renal disease (IRD) is defined as a significant reduction in glomerular filtration rate and/or loss of renal parenchyma caused by hemodynamically significant renal artery stenosis. IRD is a common and often overlooked clinical entity that presents in the setting of extrarenal arteriosclerotic vascular disease in older individuals with azotemia. IRD is an important cause of chronic renal failure and end-stage renal disease (ESRD), and many patients with a presumed diagnosis of hypertensive nephrosclerosis may actually have undiagnosed ischemic nephropathy as the cause of their ESRD. The primary reason for establishing the diagnosis of IRD is the hope that correction of a renal artery stenosis will lead to improvement of renal function or a delay in progression to ESRD. There are six typical clinical settings in which the clinician could suspect IRD: acute renal failure caused by the treatment of hypertension, especially with angiotensin-converting enzyme inhibitors; progressive azotemia in a patient with known renovascular hypertension; acute pulmonary edema superimposed on poorly controlled hypertension and renal failure; progressive azotemia in an elderly patient with refractory or severe hypertension; progressive azotemia in an elderly patient with evidence of atherosclerotic disease; and unexplained progressive azotemia in an elderly patient. It is important for the clinician to identify IRD, because IRD represents a potentially reversible cause of chronic renal failure in a hypertensive patient.
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ranking = 0.12686278788338
keywords = sclerosis
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3/134. Lipoprotein (a) immunapheresis in the treatment of familial lipoprotein (a) hyperlipoproteinemia in a patient with coronary heart disease.

    This paper reports 2 years' experience with lipoprotein (a) (Lp[a]) immunapheresis which was successfully handled on a now 40-year-old patient with familial Lp(a) hyperlipoproteinemia inducing severe coronary heart disease with 2 myocardial infarctions and diffuse coronary sclerosis. Continued treatment by Lp(a) immunabsorption with specific sheep antibodies reduced stenosis in coronary vessels more than 50% and stopped the progression of coronary heart disease. A special apheresis technique and the results of continued absorption effects are described.
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ranking = 0.12686278788338
keywords = sclerosis
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4/134. Discordant evolution of asymptomatic proteinuria in identical twins.

    We describe a pair of 17-year-old identical twin brothers with asymptomatic proteinuria, one of whom showed focal segmental glomerulosclerosis (FSGS) while the other showed immunoglobulin m (IgM) nephropathy. For each twin, audiological examination was normal. There was no family history of renal failure, deafness, or hematuria. HLA typing revealed an identical phenotype consisting of A25, A33, B44, B54, Cw1, Cw7, DR7 and DRB1. There is still controversy about whether minimal change disease, IgM nephropathy, and FSGS are discrete entities or different aspects of the same disease. The coexistence of IgM nephropathy and FSGS in identical twins suggests that the same genetic factors may be involved in the development of both diseases. However, although the brothers are identical twins, they had different eating habits and body weight. The twin who preferred to eat a protein-rich diet and who was heavier developed early proteinuria and manifested FSGS on renal biopsy. The discordant evolution of asymptomatic proteinuria in identical twins may provide a clue for the existence of environmental factors on the progression from IgM nephropathy to FSGS. Therefore, this report provides indirect support for the hypothesis that IgM nephropathy and FSGS represent different aspects in the spectrum of a single disease.
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ranking = 0.12686278788338
keywords = sclerosis
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5/134. multiple sclerosis presenting as a single mass lesion.

    A 6-year-old male presented with a subacute onset of hemiplegia and a mass lesion on computed tomography scan. pathology from a surgical biopsy revealed demyelination only. Ten months later, he had a recurrence on the opposite side. A magnetic resonance imaging scan revealed a corresponding mass lesion in the other cerebral hemisphere, allowing the diagnosis of multiple sclerosis. Consideration should be given to diagnoses other than a tumor when the clinical picture and radiologic features are atypical.
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ranking = 1.5074511515335
keywords = multiple sclerosis, sclerosis
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6/134. Effect of open label pulse cyclophosphamide therapy on MRI measures of disease activity in five patients with refractory relapsing-remitting multiple sclerosis.

    OBJECTIVE: To evaluate the response to cyclophosphamide (CTX) of five patients who failed an average three treatments with multiple other therapeutic agents, using serial monthly MRI measures. methods: Five patients with relapsing-remitting multiple sclerosis (MS) and documented MRI disease activity were started on monthly pulse intravenous CTX at a dose of 1 g/m2. CTX was administered without an induction phase according to the protocol similar to the treatment of lupus nephritis. The five patients were followed with monthly MRI and clinical evaluation for a mean of 28 months. RESULTS: All the patients showed a rapid reduction in the contrast-enhancing lesion frequency and in three patients there was a decrease in the T2 lesion load within the first 5 months after starting CTX treatment. The administration of CTX during overnight hospitalization was safe and well tolerated. CONCLUSIONS: These findings suggest that aggressive immunosuppressive therapy may be useful in some rapidly deteriorating refractory patients and further controlled study should be considered in order to full evaluate this type of treatment as a potential therapy in MS.
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ranking = 177.43173444479
keywords = relapsing-remitting, relapsing-remitting multiple sclerosis, relapsing-remitting multiple, multiple sclerosis, sclerosis
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7/134. cladribine treatment of a patient with hairy cell leukemia and concomitant multiple sclerosis.

    This is the first report on a patient suffering from both multiple sclerosis (MS) and hairy cell leukemia. The patient was first treated with interferon-alpha. Due to disease progression two courses of cladribine were given resulting in an improvement of the clinical course of both diseases. Interestingly, it was possible to arrest and even ameliorate the progression of MS by administering as little as 30% of the dosage recently recommended for the treatment of this disease.
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ranking = 5
keywords = multiple sclerosis, sclerosis
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8/134. Bilateral caudate infarct--a case report.

    Caudate strokes comprise only a small proportion of all subtypes of strokes. Bilateral caudate infarcts are even rarer and only a few cases have been reported in the literature. We report an 86-year-old woman with bilateral caudate infarcts. She had no past medical history of note. She presented with headache for several days and drowsiness on day of admission. Clinical examination revealed abulia, inability to comprehend or verbalize (acute mutism), right-sided neglect and right-sided hemiparesis. Computed tomographic (CT) scan brain revealed decreased attenuation in both heads of both caudate nuclei with extension across the anterior limb of both internal capsules to involve the lentiform nuclei. echocardiography showed aortic valve sclerosis, mild mitral and aortic regurgitation and normal left ventricular function. Carotid ultrasound revealed mild stenosis of proximal right internal carotid and left distal common carotid and adjacent proximal internal carotid arteries. She showed initial improvement in the first week, but subsequently had a progressive downward course despite rehabilitation and died 44 days after her stroke. A patient with bilateral caudate infarcts is likely to have poor prognosis for rehabilitation and survival.
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ranking = 0.12686278788338
keywords = sclerosis
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9/134. pemphigus vulgaris induced by D-penicillamine therapy in a patient with systemic sclerosis.

    D: -penicillamine-induced pemphigus occurs infrequently, typically in patients with rheumatoid arthritis. We describe a patient with systemic sclerosis who experienced this complication 3 months after starting D -penicillamine therapy. Nikolsky's sign, histopathologic findings, and direct immunofluorescence all confirmed the diagnosis. Termination of disease progression required intravenous pulse glucocorticoids, azathioprine, and 3 courses of plasmapheresis. The presentation, treatment, and etiology of D -penicillamine-induced pemphigus are reviewed, and the incidence of this complication in scleroderma patients is examined.
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ranking = 0.6343139394169
keywords = sclerosis
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10/134. Rapidly progressive aphasia and motor neuron disease: a clinical, radiological, and pathological study of an autopsy case with circumscribed lobar atrophy.

    This report concerns an autopsy case of rapidly progressive aphasia and motor neuron disease. The patient was a Japanese woman who was 75 years old at the time of death. The family history did not reveal hereditary burden. She developed language disturbances and difficulty in swallowing at age 74. Neurological examination 1 month after the disease onset revealed motor aphasia without dementia and bulbar sign, followed by muscle weakness of the four extremities. Neuroradiological examination revealed progressive atrophy of the anterior part of the left temporal lobe. She died of respiratory difficulty 10 months after the disease onset. Macroscopically, neuropathological examination showed circumscribed atrophy of the left perisylvian region and, histologically, neuronal loss in the cerebral cortex, including the primary motor area, substantia nigra, brain stem motor nuclei, and anterior horns of the spinal cord, in addition to obvious degeneration of the pyramidal tracts and presence of Bunina bodies. ubiquitin-immunoreactive neuronal inclusions were present in the hippocampal dentate granular cells and frontotemporal cortical layer II neurons. Based on these clinicopathological findings and a review of the literature, we concluded that our case is the first reported case of amyotrophic lateral sclerosis with dementia that clinically showed rapidly progressive aphasia.
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ranking = 0.12686278788338
keywords = sclerosis
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