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At least 145 records · Page 8Linked to original sources
PubMed1963

PROGRESSIVE MULTIFOCAL LEUCOENCEPHALOPATHY AND PRIMARY HYPERSPLENISM. WITH A NOTE ON THE ASSOCIATION BETWEEN DISEASE OF THE RETICULOENDOTHELIAL SYSTEM AND PROGRESSIVE MULTIFOCAL LEUCOENCEPHALOPATHY.

A patient with progressive multifocal leucoencephalopathy was found to have primary hypersplenism, a benign disorder of the reticuloendothelial system. He failed to respond to conventional doses of corticosteroids. The clinical and pathological manifestations of his illness are described, and the development of the histopathological changes in the nervous system is discussed. Consideration of available data on progressive multifocal leucoencephalopathy reveals a striking association with disease of the reticuloendothelial system, the significance of which is discussed in relation to aetiology and treatment.

Brain Diseases↗
PubMed1992

Progressive subcortical gliosis and progressive supranuclear palsy can have similar clinical and PET abnormalities.

In studies of cerebral glucose metabolism utilising positron emission tomography (PET) with 18F-fluoro-2-deoxy-D-glucose, patients with the clinical picture of progressive supranuclear palsy (PSP) have shown predominantly frontal glucose hypometabolism. This is presumed to represent deafferentation of cerebral cortical from subcortical structures. In these studies, however, the diagnosis of PSP has not been verified by pathological examination. Necropsy examinations were performed in three patients with the clinical features of PSP in whom PET had demonstrated predominantly frontal hypometabolism. In two of these patients the diagnosis of PSP was confirmed pathologically, and no morphological abnormalities were found in the cerebral cortex. The third patient had extensive cortical and subcortical neuronal loss and gliosis without neurofibrillary tangles, consistent with the diagnosis of progressive subcortical gliosis (PSG). Even in retrospect no unique clinical neurological abnormality or finding on laboratory investigation could be identified that distinguished this latter patient from those with pathologically confirmed PSP. We conclude that PSG and PSP may be indistinguishable during life, and necropsy confirmation is needed for definite diagnosis.

Blood Glucose↗
PubMed2004

Overview of primary progressive multiple sclerosis (PPMS): similarities and differences from other forms of MS, diagnostic criteria, pros and cons of progressive diagnosis.

Patients with the primary progressive form of multiple sclerosis (PPMS) have a unique clinical course and demonstrate additional demographic and imaging features, which separate them from the relapsing/remitting form of the condition. Whether these features indicate a fundamental difference in the underlying pathogenesis of the condition or simply reflect opposite ends of a clinical spectrum is unclear. What is clear, however, is that this form of MS provides a valuable model of progression, which has the potential to explain this most disabling component of the disease process. The lack of the hallmark relapses and remissions in PPMS poses diagnostic difficulties, some of which have been addressed by recently published diagnostic criteria. Following diagnosis, the need for information, specific to this form of MS, must be recognized and addressed.

Diagnosis, Differential↗
PubMed2003

[A follow-up study on brainstem atrophy in progressive supranuclear palsy--when does brain MRI contribute to the differential diagnosis between progressive supranuclear palsy and Parkinson disease?].

OBJECTIVE: To investigate when the MRI can discriminate progressive supranuclear palsy (PSP) from Parkinson disease (PD). METHODS: We obtained the following parameters using T1-weighted axial images of the midbrain and the middle pons from 40 studies of 17 PSP patients and 26 studies of 26 PD patients; 1. anteroposterior diameter of the pons (Pons AP), 2. anteroposterior length of the pontine tegmentum (Pons T), 3. anteroposterior diameter of the midbrain (Midbrain AP), 4. ratio of anteroposterior diameter of the pontine base to that of the pontine tegmentum (Pons B/T ratio), 5. ratio of the sum of the bilateral crus cerebri widths to the midbrain AP (Midbrain [Cr + Cl]/AP ratio). All the PSP patients were studied more than twice and each study was classified into four groups according to the duration of the illness; (1) less than 24 months (7 studies), (2) from 24 to 47 months (10 studies), (3) from 48 to 71 months (14 studies) and (4) 72 months or longer (9 studies). RESULT: The first MRI studies (duration of disease 39.9 +/- 22.1 months) were compared with the second studies (duration of disease 67.6 +/- 31.6 months) in 17 PSP patients. Pons AP, Pons T, and Midbrain AP were significantly smaller in the second studies, indicating progressive pontine and midbrain atrophy. Compared with the PD group, the PSP group showed significant atrophy four years after onset of the disease in Pons AP and two years in Pons T. Pons B/T ratio, and Midbrain [Cr + Cl]/AP ratio were significantly smaller in the PSP group two years after onset, suggesting midbrain and pontine tegmentum atrophy. The diagnostic MRI criteria of Pons B/T ratio more than four and Midbrain [Cr + Cl]/AP ratio more than two each showed accuracy in PSP of more than 70% two years after onset. CONCLUSIONS: Discrimination of PSP from PD was difficult during the first two years after onset. The atrophic process in the midbrain and the pontine tegmentum may precede that of the pontine base. Pons B/T ratio and Midbrain [Cr + Cl]/AP ratio presented here are potentially good indexes for the discrimination of these two diseases.

Aged↗
PubMed1997

Slowing the progression of Alzheimer disease: monitoring progression.

The search for biologic markers of Alzheimer disease (AD) progression has thus far had limited success. A technique is described, involving magnetic resonance imaging, that can be used to follow the progress of brain atrophy and hence the loss of neuronal tissue. It involves acquisition of three-dimensional images of the brain on two consecutive occasions and their precise co-registration so as to assess the loss of tissue volume over time. Median loss of brain volume in a group of individuals with sporadic and familial AD ranged from about 5-20 ml/year compared to less than 2 ml/year for controls. Loss of cerebral tissue volume was also seen in two individuals from a pedigree with familial AD at a time when symptoms were only just apparent.

Alzheimer Disease↗
PubMed1997

Long-term progression of chronic renal insufficiency in the AIPRI Extension Study. The Angiotensin-Converting-Enzyme Inhibition in Progressive Renal Insufficiency Study Group.

The Angiotensin-converting-enzyme Inhibition on Progressive Renal Insufficiency (AIPRI) Study showed that the ACE inhibitor benazepril provides protection against loss of renal function in patients with chronic renal insufficiency (CRI) caused by various renal diseases. As a result of unexpectedly low mortality in the placebo group, there was a substantial imbalance in mortality during the course of this study (8 patients on benazepril vs. 1 on placebo). The aim of the extension study was to follow-up the patients from the AIPRI core study until autumn 1996, focusing on CRI progression and mortality. Data collection was post hoc. Patients were treated according to investigators' usual practices, without knowledge of the core study trial medication or (initially) the core trial results. A new primary efficacy parameter was defined as the time from the start of core study treatment to the occurrence of the first event in the combined composite end-point of dialysis, renal transplantation or death related to renal disease. Serial serum creatinine levels and all-cause mortality were also recorded. The median total follow-up for core + extension periods was 6.6 years. Many patients from both treatment groups (64% on benazepril and 61% on placebo) received ACE inhibitors during follow-up. In the intention-to-treat analysis of the core + extension data, only 79 of 300 patients from the benazepril group, compared to 102 of the 283 patients from the placebo group needed dialysis or renal transplantation, or died related to renal disease (P < 0.013, log-rank test). The mortality imbalance seen in the core trial was not evident with the longer follow-up (25 deaths in the benazepril and 23 in the placebo group, before dialysis). These data clearly demonstrate a long-term beneficial effect in patients randomized to take benazepril during the core study, but because treatment during the extension period was not randomized, the results of this intention-to-treat analysis need to be interpreted with care.

Angiotensin-Converting Enzyme Inhibitors↗
PubMed1997

Influence of HLA alleles on the rate of progression of vertically transmitted HIV infection in children: association of several HLA-DR13 alleles with long-term survivorship and the potential association of HLA-A*2301 with rapid progression to AIDS. Long-Term Survivor Study.

The influence of host immunogenetics on the outcome of vertically transmitted HIV infection in children was examined in a multicenter cross sectional study of long term survivors and rapid progressors. Sequence-based typing was performed for the DRB1, DQB1 and HLA-A loci. 36.7% of 30 children surviving more than 8 years had one or more of the HLA-DR13 alleles, versus none of 14 rapidly progressing children who died within 2 years of age, p = 0.009, Haldane RR = 17.1. The alleles variably associated with this beneficial response to HIV were: DRB1*1301, DRB1*1302, DRB1*1303 and DRB1*1310, suggesting that the DR13 effect acted as a dominant trait. An additional 6 children were typed only by the SSOP method resulting in 44.4% of 36 long term surviving children with a DR13 allele and none of 14 rapid progressors, p = 0.002, Haldane RR = 23.3. No single DQB1 allele accounted for the HLA-DR13 allele association. In contrast, the presence of HLA A*2301 was associated with rapid progression to AIDS, 4% of long term survivors vs. 57.1% of 7 rapid progressors, p = 0.0006, RR = 0.031. Although the sample size is small, the marked differences in allele frequency along with differences between the peptide binding pockets of the HLA-A9 group of alleles including HLA A*2301 and the remainder of the HLA-A alleles suggest a structural basis for the dominant disadvantageous immune response to HIV conferred by A*2301.

Acquired Immunodeficiency Syndrome↗
PubMed1981

Method for detecting a progressive renal disorder superimposed on a preexisting progressive renal disorder.

We describe a patient with declining renal function due to polycystic kidney disease, who then insidiously develops bilateral renal artery stenosis. The major clue which led to the discovery of the bilateral renal artery stenosis was the finding of an unexpected increase in the rate of loss of renal function. This assessment was based on analysis of the slope of a plot of the reciprocal of the patient's serum creatinine concentration vs. time (reciprocal creatinine plot). The rationale for the use of the reciprocal creatinine plot to detect a progressive renal disorder superimposed upon a preexisting progressive renal disorder, is discussed.

Creatinine↗
PubMed1999

Progress's Pilgrim: a critical narrative of research in progress.

There is increasing interest in the use of stories to develop nursing and health care practice. This paper reports on how we used story to understand and develop research on nursing practice. Story (or narrative) and science can be seen as distinct but complementary paradigms. We have found that a story framework can help researchers to reflect on a process of social scientific investigation, and to consider how to 'go on' in that process. In a study on 'Community psychiatric nurses' empowerment of people with enduring mental disorders in the community: involving users to develop services' we have encountered a number of interesting and challenging issues related to design and use of methods. We present these issues within a framework of story analysis, focusing on issues related to empowerment. This analysis draws on Burke's 'pentad' of story elements as a framework for narrative analysis. We present the elements of the 'story of the study-as-funded' and as it was carried out through the pilot stage, and outline the story of developments in the main study. 'Trouble' in a story centres on a problematic 'ratio' of story elements. The 'trouble' at this stage in the progress of our study relates to lack of fit between some parts of the instruments (the methods) and the goal (empowerment), and to the status of the CPNs as actors or agents. Narrative analysis sensitizes us to these issues of 'trouble' and provides a means of addressing them. Like John Bunyan's Pilgrim, we have learned through our progress; unlike Pilgrim, we know not our end.

Community Mental Health Services↗
PubMed1999

Cognitive function in primary progressive and transitional progressive multiple sclerosis: a controlled study with MRI correlates.

The relative rarity of primary progressive (PP) and transitional progressive (TP) multiple sclerosis has meant that little documentation of cognitive function in such patients is currently available. The aim of this study was to investigate the cognitive skills of patients with PP and TP multiple sclerosis relative to matched healthy controls, and to examine the relationship of this impairment to MRI parameters. Sixty-three patients (43 PP, 20 TP) were individually matched with healthy controls, who undertook the same cognitive tasks as the patient group. The neuropsychological assessment comprised Rao's brief repeatable battery, a reasoning test, and a measure of depression. Patients also underwent T1- and T2-weighted brain MRI. These patients were taken from a larger cohort (158 PP, 33 TP) in whom it had been demonstrated that the re were no significant differences between the mean scores of the PP and TP groups on any of the cognitive variables. The 63 patients were therefore taken as one group for comparison with the healthy controls. These patients performed significantly worse than the controls in tests of verbal memory, attention, verbal fluency and spatial reasoning. An impairment index was constructed and applied to the patient data. This correlated modestly with T2-lesion load (r = 0.45, P = 0.01), T1-hypointensity load (r = 0.45, P = 0.01) and cerebral volume (r = -0.35, P = 0.01). Thus, PP and TP multiple sclerosis patients demonstrate significant cognitive dysfunction when compared with matched healthy controls. The relationship between this impairment and MRI parameters is moderate, suggesting that cognitive dysfunction in PP and TP multiple sclerosis has a complex and multifactorial aetiology, which is not adequately explained by pathology as demonstrated on conventional MRI.

Adult↗
PubMed2000

Music assisted progressive muscle relaxation, progressive muscle relaxation, music listening, and silence: a comparison of relaxation techniques.

The purpose of this study was to compare the effects of music assisted progressive muscle relaxation (M + PMR), progressive muscle relaxation (PMR), music listening, and silence/suggestion on measures of anxiety and perceived relaxation. The study also examined participant responses to a posttreatment questionnaire to identify relationships between musical and nonmusical elements in relaxation techniques. Sixty university students participated in the study. Fifteen participants were randomly assigned to each treatment condition. Subjects were tested individually using the same relaxation script for M + PMR and PMR conditions. One-way analyses of covariance were computed to compare pre and posttest differences among groups. Results of the ANCOVA revealed no differences among groups for the State Trait Anxiety Inventory (STAI) or the Visual Analog Scale (VAS). Analysis of variance, however, revealed each treatment condition to be equally effective in producing significant changes in anxiety and perceived relaxation from the pre to posttest period. Additionally, mean score differences revealed decreases for all conditions with M + PMR eliciting the greatest amount of change. A content analysis of posttreatment questionnaire items revealed detailed information about each participant's relaxation experience, state of mind, and use of self-generated relaxation techniques.

Adult↗
PubMed1996

Progressive intracranial aneurysmal disease in a child with progressive hemifacial atrophy (Parry-Romberg disease): case report.

Intracranial aneurysms are uncommon in children, and their presence often leads to suspicion of a systemic connective tissue disorder. We describe the case of a young male patient with progressive hemifacial atrophy (Parry-Romberg disease) and multiple intracranial aneurysms, a previously undescribed association, and propose that a neural crest defect may be the underlying abnormality in this patient. At age 5 years, the patient was treated for a giant aneurysm of the left cavernous carotid artery with carotid ligation in the neck and a superficial temporal artery-middle cerebral artery bypass. At age 12 years, the patient was similarly treated for a giant aneurysm of the right cavernous carotid artery, which had progressed from a previously noted minute dilatation at age 5 years, with carotid ligation and a superficial temporal artery-middle cerebral artery bypass. At age 21 years, the patient was endovascularly treated for a de novo saccular aneurysm of the left posterior cerebral artery at the P1-P2 junction and a fusiform aneurysm of the distal left posterior cerebral artery. Various studies have suggested that the facial dermis, the subcutaneous tissues, and the skeleton, as well as the tunica media of the cervicocephalic arteries, all arise from neural crest cells, and a disorder of neural crest migration might explain the constellation of findings in this patient.

Adolescent↗
PubMed1992

Progressive systemic sclerosis causing rapidly progressive myocardial disease and death.

We have presented an unusually swift progression of progressive systemic sclerosis (PSS), with death from cardiogenic shock in a 22-year-old woman who had severe hypertension and acute renal insufficiency. She arrived at our hospital with pericardial tamponade and shock. Despite initial improvement after pericardiocentesis, the patient's condition soon deteriorated and she died of cardiogenic shock. PSS was diagnosed at autopsy. Although the course of PSS is frequently indolent, it may also be fulminant, leading to death before diagnosis is determined.

Acute Kidney Injury↗
PubMed1991

The progressive systemic sclerosis/systemic lupus overlap: an unusual clinical progression.

Three patients with the unusual combinations of discoid lupus, systemic lupus erythematosus (SLE), and progressive systemic sclerosis (PSS) are reported. The first patient developed PSS eight years after a diagnosis of discoid lupus had been made and this was complicated by myositis six years later. The second patient developed PSS more than 20 years after being diagnosed as having SLE. The third patient developed SLE with predominant features of urticarial vasculitis six years after PSS. Mild myositis also ensued. There were no antibodies to U1RNP demonstrable in any of these patients. The clinical progression of SLE to PSS or vice versa in the absence of features of mixed connective tissue disease is distinctly uncommon.

Adolescent↗
PubMed2006

Effect of organic solvent exposure on chronic kidney disease progression: the GN-PROGRESS cohort study.

It has been suggested that solvent exposure may have a role in the progression of glomerulonephritis (GN) to ESRD, but this has never been tested with an appropriate cohort study design. A total of 338 non-ESRD patients with a first biopsy for primary GN between 1994 and 2001 were included: 194 IgA nephropathies (IgAN), 75 membranous nephropathies (MN), and 69 FSGS. ESRD, defined as an estimated GFR <15 ml/min per 1.73 m2 or dialysis, was registered during a mean follow-up period of 5 yr. Patients' lifelong solvent exposures before and after diagnosis were recorded by interview and assessed by industrial hygienist experts. Cox models were used to estimate adjusted hazard ratios (HR) of ESRD related to exposures. Overall, 15 and 14% of the patients had been exposed at a low and a high level before diagnosis, respectively. Forty-two with IgAN, 12 with MN, and 22 with FSGS reached ESRD. A graded relationship was observed for MN (age- and gender-adjusted HR [95% confidence interval] for low exposure versus none was 3.1 [0.5 to 18.2] and for high exposure versus none was 8.2 [1.9 to 34.7]) and for IgAN (1.6 [0.7 to 3.9] and 2.2 [1.0 to 4.8]) but not for FSGS. Solvent risk was mediated only partly by baseline proteinuria: Adjusted HR for high exposure versus none was 5.5 (1.3 to 23.9) for MN and 1.8 (0.8 to 3.9) for IgAN. In patients with IgAN, there was a trend in increasing HR with exposure duration before and its persistence after diagnosis. These findings support the hypothesized association of solvent exposure with the progression of GN to ESRD. They should prompt clinicians to give greater attention to patients' occupational exposures and possibly to consider professional reclassification.

Adult↗
PubMed1989

The progress of oesophageal involvement in progressive systemic sclerosis during D-penicillamine treatment.

In 21 patients with initial signs of progressive systemic sclerosis, oesophageal motility was monitored manometrically from the start of D-penicillamine treatment and over a period of up to 5 years. Urinary excretion of the collagen-specific amino acids hydroxyproline and hydroxylysine, and of proline was used as a guideline for monitoring the bioavailability of D-penicillamine. D-penicillamine therapy was found to be unable to arrest the progress of oesophageal involvement. A downward trend over time--statistically significant at p = 0.03, p = 0.02, and p less than 0.005--was found for lower oesophageal sphincter pressure, peristaltic wave pressure in the distal third, and peristaltic wave pressure in the middle third of the oesophagus, respectively.

Esophagitis↗
PubMed1975

Meningomyelocele and progressive hydromyelia. Progressive paresis in myelodysplasia.

Five cases of myelodysplasia with progressive paraparesis are presented. Three of the five patients developed spasticity, but dissociated sensory loss and loss of sphincter control was not a prominent feature. All were found to have compensated hydrocephalus and extensive communicating hydromyelia. The use of myelography and ventriculography in the diagnosis of hydromyelia is discussed. Ventricular drainage led to clinical improvement in two cases and radiological improvement in one. The relationship of compensated hydrocephalus, meningomyelocele, and progressive hydromyelia postnatally may support the hydrodynamic hypothesis of myelodysplasia.

Adult↗
PubMed1983

Dietary protein intake and progressive glomerular sclerosis: the role of capillary hypertension and hyperperfusion in the progression of renal disease.

Unrestricted intake of protein-rich foods is accompanied by sustained increases in glomerular capillary pressures and flows. Intrarenal hypertension and hyperperfusion associated with protein intake may eventually cause glomerular sclerosis and account for decreased renal function seen with aging. Further elevation of glomerular capillary pressures and flows contributes to progressive glomerular destruction and eventual loss of renal function when nephron number has been reduced by renal disease. Progressive loss of renal function may be retarded by restriction of protein intake. Protein restriction appears to preserve renal function by limiting intrarenal capillary hypertension and hyperperfusion.

Aging↗