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1.
The objective of this 12-month study was to describe the clinical features of late-onset Pompe disease and identify appropriate outcome measures for use in clinical trials. Assessments included quantitative muscle testing (QMT), functional activities (FAA), 6-min walk test (6MWT), and pulmonary function testing (PFT). Percent predicted values indicated quantifiable upper and lower extremity weakness, impaired walking ability, and respiratory muscle weakness. Significant declines in arm and leg strength and pulmonary function were observed during the study period. The outcome measures were demonstrated to be safe and reliable. Symptom duration was identified as the best predictor of the extent of skeletal and respiratory muscle weakness.  相似文献   

2.
Intrastriatal transplantation of striatal neuroblasts from human fetuses is a promising approach for treatment of Huntington's disease, on the basis of many experimental animal studies and, most recently, pilot clinical trials. Technically, several issues remain to be resolved (e.g., the precise site of dissection of the fetal tissue; the number and location of the fetal striatal implants; or the use of immunosuppressive therapy), and await larger-scale trials and purposely designed protocols. Further clinical data must also be obtained, and preliminary promising results must be replicated in a patient group large enough to provide conclusive results. It is important to establish (1) the amount of clinical benefit provided to the patient by the grafted cells; (2) the anticipated duration of clinical benefits; and (3) the secondary rate of decline after the benefit of the graft has been overbalanced. Evaluation of these parameters will require very long-term follow-up of the patients involved, over several years after grafting, before the technique can eventually be proposed widely to patients.  相似文献   

3.
Objective: To review data examining the relationships between depression, antidepressants and cardiovascular disease. Method: Structured searches of PubMed, Medline and Embase conducted in March 2008. Results: Depression and cardiovascular disease are closely associated clinical entities. Depression appears both to cause and worsen cardiovascular disease. Cardiovascular disease is in turn associated with a high incidence of depression. Depression is associated with increased mortality in cardiovascular disease, and after myocardial infarction (MI) and stroke. Many antidepressants have cardiotoxic properties. Tricyclic drugs are highly cardiotoxic in overdose and may induce cardiovascular disease and worsen outcome in established cardiovascular disease. Reboxetine, duloxetine and venlafaxine are known to increase blood pressure. Other antidepressants have neutral or beneficial effects in various cardiovascular disorders. Conclusion: Sertraline, fluoxetine, citalopram, bupropion and mirtazapine appear to be safe to use after MI; the use of sertraline, and response to citalopram and mirtazapine may improve mortality. Paroxetine and citalopram appear to be safe to use in patients with established coronary artery disease. Limited data suggest that a variety of antidepressants are effective and safe to use after stroke.  相似文献   

4.
Memory rehabilitation in Alzheimer's disease: a review of progress   总被引:6,自引:0,他引:6  
BACKGROUND: Memory rehabilitation is a sadly misrepresented area of applied research in Alzheimer's disease. OBJECTIVES: To gather and evaluate recent evidence for the clinical effectiveness or ecologically validity of memory rehabilitation for mild to moderate Alzheimer's patients. METHODS: Computerised searches and some handsearching were conducted spanning the last five years, from 1995 to 2000, inclusively. Criteria for inclusion in this overview involved the use of a precise memory rehabilitation technique within an experimental study design applied to Alzheimer's patients with pre- and post-treatment evaluation. FINDINGS: Three potential levels of memory rehabilitation procedures with proven clinical or pragmatic efficacy were identified. The first level bears on the facilitation of residual explicit memory with structured support both at encoding and at subsequent recall; the second level of memory rehabilitation exploits the relatively intact implicit memory system (priming and procedural memory); the last deals with finding ways of coping with the patient's limited explicit memory capacities through the use of external memory aids. A proposal of suggestions for good practice and future research in memory rehabilitation is also offered with the hope to spur further development in this rapidly expanding area of applied research. CONCLUSION: The available evidence shows that alternative and innovative ways of memory rehabilitation for Alzheimer's patients can indeed be clinically effective or pragmatically useful with a great potential for use within the new culture of a more graded and proactive type of Alzheimer's disease care.  相似文献   

5.
Apomorphine (APO) is a potent dopamine agonist that is partially metabolized by catachol-O-methyl transferase (COMT). Tolcapone was the first COMT inhibitor available for use as adjunctive therapy to levodopa in Parkinson's disease (PD). In order to determine whether this compound might increase the serum area under the curve (AUC) of APO and whether this results in any clinical benefit, we administered 200 mg doses of tolcapone to five fluctuating PD patients taking an investigational sublingual APO preparation. Serial tapping speed and gait speed were assessed at 15 min intervals over four hours, in conjunction with APO serum levels, following a single dose of APO, both before and five days after starting tolcapone (600 mg/day). Serum APO levels tended to be higher (12.6%), and clinical measures suggested improvement during the APO “on” period after the addition of tolcapone (22.5% improvement in gait speed, and 7.6% improvement in tapping speed), but neither reached statistical significance. Further trials, involving larger samples are needed to clearly establish the pharmacokinetic and clinical effect of tolcapone in PD patients taking APO.  相似文献   

6.
The pull test (PT) is used as a measure of postural instability in Parkinson's disease (PD) and other movement disorders. In 1987, it was incorporated into the Unified Parkinson's Disease Rating Scale (UPDRS), a scale used to measure the severity and treatment response in PD both in research studies and in clinical practice. However, the origins of the observation of postural instability in movement disorders and the attempt to quantify it are much older. Here, we trace the history of postural instability first described as a feature of PD by Romberg in 1853. Attempts to evaluate postural instability began with the first measurement by Charcot in the 1880s by pulling the clothes of patients and progressed to the push on the sternum by Hoehn and Yahr in the 1960s. Eventually, this evolved into the formal PT proposed by Fahn in the 1980s. Despite the widespread use of the PT as part of the UPDRS, variability exists in its execution. Recommendations have been made for training of examiners in clinical trials to improve its accuracy in assessing postural instability. We agree with improving PT technique for clinical trials and advocate for its routine use in clinical practice when diagnosing and treating movement disorders. Further, we propose the name "Fahn pull test" for the maneuver based on his significant contribution to its development.  相似文献   

7.
In 2011, the National Institute on Aging and Alzheimer's Association created separate diagnostic recommendations for the preclinical, mild cognitive impairment, and dementia stages of Alzheimer's disease. Scientific progress in the interim led to an initiative by the National Institute on Aging and Alzheimer's Association to update and unify the 2011 guidelines. This unifying update is labeled a “research framework” because its intended use is for observational and interventional research, not routine clinical care. In the National Institute on Aging and Alzheimer's Association Research Framework, Alzheimer's disease (AD) is defined by its underlying pathologic processes that can be documented by postmortem examination or in vivo by biomarkers. The diagnosis is not based on the clinical consequences of the disease (i.e., symptoms/signs) in this research framework, which shifts the definition of AD in living people from a syndromal to a biological construct. The research framework focuses on the diagnosis of AD with biomarkers in living persons. Biomarkers are grouped into those of β amyloid deposition, pathologic tau, and neurodegeneration [AT(N)]. This ATN classification system groups different biomarkers (imaging and biofluids) by the pathologic process each measures. The AT(N) system is flexible in that new biomarkers can be added to the three existing AT(N) groups, and new biomarker groups beyond AT(N) can be added when they become available. We focus on AD as a continuum, and cognitive staging may be accomplished using continuous measures. However, we also outline two different categorical cognitive schemes for staging the severity of cognitive impairment: a scheme using three traditional syndromal categories and a six-stage numeric scheme. It is important to stress that this framework seeks to create a common language with which investigators can generate and test hypotheses about the interactions among different pathologic processes (denoted by biomarkers) and cognitive symptoms. We appreciate the concern that this biomarker-based research framework has the potential to be misused. Therefore, we emphasize, first, it is premature and inappropriate to use this research framework in general medical practice. Second, this research framework should not be used to restrict alternative approaches to hypothesis testing that do not use biomarkers. There will be situations where biomarkers are not available or requiring them would be counterproductive to the specific research goals (discussed in more detail later in the document). Thus, biomarker-based research should not be considered a template for all research into age-related cognitive impairment and dementia; rather, it should be applied when it is fit for the purpose of the specific research goals of a study. Importantly, this framework should be examined in diverse populations. Although it is possible that β-amyloid plaques and neurofibrillary tau deposits are not causal in AD pathogenesis, it is these abnormal protein deposits that define AD as a unique neurodegenerative disease among different disorders that can lead to dementia. We envision that defining AD as a biological construct will enable a more accurate characterization and understanding of the sequence of events that lead to cognitive impairment that is associated with AD, as well as the multifactorial etiology of dementia. This approach also will enable a more precise approach to interventional trials where specific pathways can be targeted in the disease process and in the appropriate people.  相似文献   

8.
Three Alzheimer's disease patients with Pisa syndrome were presented. These patients were treated with cholinesterase inhibitors within a post-marketing surveillance study. The clinical and pharmacological histories of these patients suggest a relation between cholinesterase inhibitors and the pleurothotonus. The diffuse use of cholinesterase inhibitors in the treatment of dementia may cause an increasing number of Pisa syndrome cases.  相似文献   

9.
《Revue neurologique》2022,178(5):450-459
Whilst the development of new drugs designed for the treatment of Alzheimer's disease (AD) has been widely publicised, we do not yet have treatments that are proven to slow the progression of AD. The decision taken by the US Food and Drug Administration (FDA) to grant a licence for the use of aducanumab, based on the premise that β-amyloid removal would result in downstream benefits rather than demonstration of cognitive efficacy per se contrasts with that made by the European Medicines Agency (EMA), who declined to grant a licence, citing lack of evidence of clinical improvement, and a failure to demonstrate that the treatment was sufficiently safe. Multiple factors have complicated the search for new and effective treatments for the management of AD. It is a complex neurodegenerative condition in which multiple comorbidities are common in the affected population. However, such conditions are commonly exclusion criteria in clinical trials for new treatments. Here we discuss how some of these comorbidities impact the development of clinically efficient treatments for AD. Firstly, we will examine what is meant by AD, and how definitions of this condition have changed and continue to evolve. Secondly, we describe some of the most important comorbid conditions accompanying and in some cases mimicking AD. Finally, we will examine how the inclusion, or exclusion, of these conditions from AD research may have had an effect on treatment trials, the implications of co-morbidities on “real-life” use of novel therapeutics especially when these have been trialled in patients with relatively pure disease, and how clinical trials may need to adapt to account for comorbidities in the future.  相似文献   

10.
We have completed a survey of European neurological practice concerning cerebral vasculitis. Twenty-nine respondents from 15 countries provided information concerning the diagnosis and management. The results confirmed the anticipated low frequency of the disease, but also illustrated the power of any putative collaborative effort. Interestingly, there was a wide variation in clinical practice, in particular concerning the perceived importance of cerebral angiography as a diagnostic test and the very common use of steroids as first-line treatment, rather than more potent immunosuppressive agents. This variation is probably to be explained at least, in part, by the absence of any firm evidence base to inform clinical practice. A European collaborative effort--in which there has emerged considerable interest--offers a realistic opportunity to generate sound clinical evidence and thence scientifically robust practical guidelines.  相似文献   

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