Showing papers in "Neurodegenerative disease management in 2012"
TL;DR: Scales of more complex ADL are needed to better capture individuals with preclinical AD before they start to progress to MCI.
Abstract: SUMMARY Impairment in activities of daily living (ADL) accompanies cognitive and behavioral symptoms in Alzheimer’s disease (AD). Conventionally, ADL impairment has been relegated to the stage of dementia, but instrumental ADL impairment has been shown to occur earlier at the stage of mild cognitive impairment (MCI). There are many subjective and performance-based instrumental ADL scales, some of which are useful in distinguishing between MCI and AD dementia, and even between MCI and clinically normal elderly individuals. These scales have been associated with amyloid and neurodegeneration biomarkers of AD. Clinically normal elderly individuals who have a positive AD biomarker are thought to be at the preclinical stage of AD. Scales of more complex ADL are needed to better capture individuals with preclinical AD before they start to progress to MCI.
114 citations
TL;DR: It is concluded that ethical nonstigmatizing care focuses on personhood and relational aspects of care, and that work to reduce stigma is needed in four main areas: policy, research, information/education and service design/delivery.
Abstract: SUMMARY In this article, we reflect on stigma, and the evidence about its effect on the care of individuals and families living with a dementia. We argue that it distorts services at all levels, from individuals making the decision about seeking help, through those commissioning, designing and providing services, to politicians deciding about priorities and resources. We conclude that ethical nonstigmatizing care focuses on personhood and relational aspects of care, and that work to reduce stigma is needed in four main areas: policy, research, information/education and service design/delivery. Dementia is not something that affects other people – it will impact on many of us as we grow older. People living with a dementia must be heard and respected.
64 citations
TL;DR: Neither the lower brainstem LB inclusions nor the cerebellar PC loss represent the neurodegenerative basis of ET, and there is no evidence for reduced tyrosine hydroxylase levels in ET patients.
Abstract: The pathophysiology of essential tremor (ET) remains unknown. Standard neuropathological studies have reported no consistent changes but a detailed study found neurodegeneration in all ET cases - 24% demonstrated lower brainstem Lewy body (LB) inclusions and 76% experienced a loss of cerebellar Purkinje cells (PCs) and its sequelae. We review the evidence on neurodegeneration in ET. The prevalence of LB inclusions in ET brains is similar to that in the asymptomatic general population. These incidental LB disease cases have evidence for reduced striatal tyrosine hydroxylase levels, as found in Parkinson's disease, but there is no evidence for reduced tyrosine hydroxylase levels in ET patients. Reduced mean PC counts in ET cases compared with the controls reported by some studies could not be replicated by others. Most ET cases have the same number of PCs as controls of a comparable age. Neither the lower brainstem LB inclusions nor the cerebellar PC loss represent the neurodegenerative basis of ET. Further studies are needed to determine the pathophysiology of ET.
44 citations
TL;DR: The European Huntington’s Disease Network Standards of Care Speech and Language Therapy Working Group has brought together expert speech and language therapists from across Europe to produce guidelines to improve the management of communication disorders for individuals with HD.
Abstract: SUMMARY Speech and language therapists play an important role in the management of communication difficulties in Huntington’s disease (HD). As the disease progresses the effectiveness of communication becomes increasingly compromised by a combination of changes in motor function, diminishing cognitive linguistic abilities and neuropsychiatric changes, such as depression and apathy. The complexities and challenges presented by communication breakdown in HD require comprehensive assessment and interventions that are responsive to the changing motor, cognitive and emotional needs of the individual. The European Huntington’s Disease Network Standards of Care Speech and Language Therapy Working Group has brought together expert speech and language therapists from across Europe to produce guidelines to improve the management of communication disorders for individuals with HD. The guidelines were developed with the aim of promoting timely and appropriate assessment and focused management throughout all stages of...
33 citations
32 citations
TL;DR: Neuropsychiatric symptoms had a stronger association than demographic and cognitive factors and a similar association with functional impairment and Psychotic symptoms were consistently associated with increased caregiver burden compared with other NPS.
Abstract: SUMMARY Neuropsychiatric symptoms (NPS) are very common amongst patients with Alzheimer’s disease and contribute significantly to caregiver burden. Different NPS may contribute differently to caregiver burden. This has important implications for targeting treatment. Medline was searched using the keywords ‘caregiver burden’, ‘BPSD’, ‘behavioral problems’, ‘Alzheimer’s disease’, ‘dementia’ and ‘dementia of the Alzheimer type’. After an extensive search, 14 papers were identified. Caregiver burden and related quality-of-life measures were significantly associated with NPS in all studies. When compared with other factors, NPS had a stronger association than demographic and cognitive factors and a similar association with functional impairment. Psychotic symptoms were consistently associated with increased caregiver burden compared with other NPS. Pharmacological and nonpharmacological interventions should focus on addressing the NPS that are most associated with increased caregiver burden.
31 citations
TL;DR: The European Huntington's Disease Network Standards of Care Dietitians Group has brought together expert dietitians from across Europe to produce nutritional guidelines to improve the nutritional management of individuals with Huntington’s disease.
Abstract: SUMMARY The delivery of good nutritional care is a fundamental element of the management of individuals with Huntington’s disease and all patients with Huntington’s disease will, at some time, need dietary intervention because of the sequela of the disease; yet there are no European nutritional guidelines. The European Huntington’s Disease Network Standards of Care Dietitians Group has brought together expert dietitians from across Europe to produce nutritional guidelines to improve the nutritional management of individuals with Huntington’s disease. The guidelines were developed to promote optimal nutritional screening, assessment and management of individuals throughout all stages of the disease, with the aim of improving the standard of nutritional care delivered. Literature was systematically searched in an attempt to ensure that the recommendations are based on sound evidence and where evidence is lacking, specific guidance is based on consensus expert dietetic opinion. The provision of nutritional care varies widely between countries. Implementation of these nutritional guidelines across Europe should improve the quality of nutritional care delivered to individuals with Huntington’s disease.
28 citations
TL;DR: European guidelines to improve the management of swallowing disorders for individuals with Huntington’s disease were developed with the aim of promoting timely and appropriate assessment of the swallowing process and focused management throughout all stages of the disease.
Abstract: SUMMARY Speech and language therapy has an important role in the management of Huntington’s disease (HD). Swallowing difficulties affect most individuals with HD. Throughout the disease process these difficulties require management with timely and effective therapeutic intervention. Currently there are no European guidelines for the assessment and management of swallowing impairments in HD. The European Huntington’s Disease Network (EHDN) Standards of Care Speech and Language Therapy Working Group has brought together expert speech and language therapists from across Europe to produce guidelines to improve the management of swallowing disorders for individuals with Huntington’s disease. The guidelines were developed with the aim of promoting timely and appropriate assessment of the swallowing process and focused management throughout all stages of the disease. Literature was systematically searched in an attempt to ensure that the recommendations are based on sound evidence. Where evidence was lacking, sp...
28 citations
TL;DR: There is no internationally recognized standard of care for Huntington’s disease and there are very few peer-reviewed papers on this topic, despite clear evidence of the need for such work; the evidence for best practice is lacking.
Abstract: ISSN 1758-2024 10.2217/NMT.11.85 Neurodegen. Dis. Manage. (2012) 2(1), 1–5 Huntington’s disease (HD) is a relentlessly progressive and incurable neurodegenerative disease that affects men and women, usually presenting late in their 4th decade. Each offspring of an affected person has a 50% risk of inheriting this autosomal dominant disorder. It bears the name of George Huntington who described the disease in his talk, then paper, in 1872 [1] although others had written in earlier years about what is clearly the same disease [2]. The cardinal signs of HD include the movement disorder that is usually but not always choreiform in nature, as well as cognitive impairment, personality change and neurological features, which include swallowing disorder and speech impairment. Some of these outcomes of the disease can be managed with some success, but there is no cure. ‘This man has Huntington’s disease; there is nothing more I can do’ has been seen in patients’ hospital notes on a regular basis, but there is none the less much that can be done to improve the quality of life of the HD patient and their families. There is no internationally recognized standard of care for Huntington’s disease and there are very few peer-reviewed papers [3–6] on this topic, despite clear evidence of the need for such work [7]. The evidence for best practice is lacking. In part, this reflects the fact that in many countries there are no specialist clinics for this patient group, and statistically significant data about care from large patient groups are lacking. Throughout the world, the care provided for HD families varies widely. Some clinics are led by clinicians with an interest who have developed their service on an ad hoc basis. Some are part of general psychiatric or neurology clinics. There are differing approaches to such clinics, and differing opinions about what is necessary at such a clinic. Data presented by Simpson were collected from 28 clinicians involved in specialist care of HD in nine European countries [8]. In total, 68% of the respondents were neurologists, 54% of them shared clinical responsibility for the patients with another clinician. These other specialities included neurology, genetics, psychiatry, neuropsychiatry, neuropsychology and specialists in
28 citations
TL;DR: The role of metabotropic glutamate receptors within the basal ganglia is reviewed and the potential for modulation of metabotrop glutamate receptors as a treatment for PD is discussed.
Abstract: SUMMARY Parkinson’s disease (PD) is a progressive neurological disorder predominantly characterized by motor symptoms including bradykinesia and resting tremor. The gold standard of treatment for PD remains dopamine replacement therapy, which eventually fails due to continued progression of the disease and the development of debilitating side effects. Recent breakthroughs are providing the first major advances in the development of fundamentally new pharmacological strategies for the treatment of PD that do not rely on dopamine replacement strategies, but rather aim to reduce the overactive indirect pathway within the basal ganglia. In this article, we will review the role of metabotropic glutamate receptors within the basal ganglia and discuss the potential for modulation of metabotropic glutamate receptors as a treatment for PD.
25 citations
TL;DR: Biomarker evidence and clinical observations support the hypothesis that there is a diagnosable condition termed preclinical Alzheimer's disease (AD), and it is not clear whether subtle cognitive decline can be detected reliably on an individual basis.
Abstract: SUMMARY Biomarker evidence and clinical observations support the hypothesis that there is a diagnosable condition termed preclinical Alzheimer’s disease (AD). Recently, a workgroup convened under the auspices of the National Institute on Aging and the Alzheimer’s Association proposed a framework for defining preclinical AD. The definition was based on the presence of biomarkers that are indicative of the AD pathophysiological process. In the context of abnormal AD biomarkers, the workgroup postulated that ‘subtle cognitive changes’ occurred as well. Based on studies of genetically at-risk individuals and those destined to become demented, who were observed while still cognitively normal, low performance on learning and memory functions may be the earliest cognitive manifestations of preclinical AD, at the group level at least. It is not clear whether subtle cognitive decline can be detected reliably on an individual basis. Preclinical AD cognitive changes could be diagnosed by traditional neuropsychologic...
TL;DR: Those who were referred to the Alzheimer Society via First Link were referred sooner after the diagnosis of Alzheimer’s disease and related dementias than those who were self-referred.
Abstract: dementia, by linking them and their caregivers with support services earlier in the disease course, by facilitating referrals to the Society by health professionals rather than putting the onus on clients to self-refer. This study evaluated First Link as implemented in two provinces in Canada and involved a mixed method, prospective cohort design; data sources included the Alzheimer Society referral database, surveys of caregivers (n = 135) and health professionals (n = 82), and interviews with key stakeholders (n = 40) and individuals with dementia and their caregivers (n = 33). On average, those who were referred to the Alzheimer Society via First Link were referred sooner after the diagnosis of Alzheimer’s disease and related dementias than those who were self-referred (6–7 vs 17–18 months, respectively). Referral sources report that as a result of First Link they have a better understanding of how to manage individuals with dementia and the role of the Alzheimer Society, and are more aware of the available community resources to serve this population. Caregivers reported that as a result of First Link they were more knowledgeable about Alzheimer’s disease and related dementias and more aware of the available community support and resources. The new model of service access, as facilitated by First Link, ensures caregivers and individuals with dementia have the opportunity to be supported as soon as possible after diagnosis and represents a significant move toward increasing the capacity for dementia care.
TL;DR: A review of existing studies investigating inflammatory markers as potential biomarkers of dementia is presented, highlighting some strengths and limitations of the current research and discussing the future directions for this field.
Abstract: Dementia is a huge public health concern today owing to the exponentially increasing number of older adults it affects each year, and there has been a large number of investigators looking at potential biomarkers of dementia. Peripheral inflammatory markers have emerged as one potential class of markers that may be useful in predicting those individuals at a greater risk of developing dementia, or in expounding the underlying mechanisms or pathways of this complex disease. Although some evidence has been promising, indicating that peripheral inflammatory markers are indeed crucial in brain changes that occur in both normal aging and in dementia, results have been mixed on their usefulness for predicting dementia or cognitive decline in older adults. Here, the authors present a review of existing studies investigating inflammatory markers as potential biomarkers of dementia, highlighting some strengths and limitations of the current research and discuss the future directions for this field.
TL;DR: Clinical and imaging measures that have been assessed as potential markers of disease progression in preclinical and early symptomatic Huntington's disease are reviewed.
Abstract: SUMMARY Huntington’s disease (HD) is an autosomal dominant progressive neurodegenerative disorder that typically begins in middle adulthood. The neurodegenerative process that underlies HD, however, likely begins many years before clinical diagnosis. Since genetic testing can identify individuals that will develop HD during this preclinical period, clinical trials aiming to slow disease progression will likely focus on this phase of the illness in an effort to delay disease onset. How to best measure the efficacy of potential disease-modifying therapies in preclinical HD remains a complex challenge. This article will review the clinical and imaging measures that have been assessed as potential markers of disease progression in preclinical and early symptomatic HD.
TL;DR: The Physiotherapy Guidance for HD is a comprehensive, consensus- and evidence-based document that can be used by physiotherapists to implement a plan of care that is currently consistent with best practice for individuals at all stages of HD.
Abstract: Background: Physiotherapy may provide a means of delaying onset or progression of Huntington’s disease (HD), resulting in improved daily functioning and quality of life. Physiotherapy is being more frequently recommended for people with HD, but there have been no specific guidelines published for implementation of a structured physiotherapy program. The Physiotherapy Working Group (PWG) of the European Huntington’s Disease Network (EHDN) set out to develop a comprehensive Guidance Document for physiotherapists to provide best practice guidelines. Methods: A review of the literature was conducted using a systematic approach. There was insufficient literature in support of physiotherapy interventions and approaches to be able to conduct a complete evidenced-based review, therefore, physiotherapy expert subgroups were formed to incorporate consensus as to best practice. A draft document was distributed to the entire membership of the working group, to outside physiotherapists and other healthcare professionals within EHDN to elicit feedback and comments. Results: A Guidance Document covering eight specific areas pertaining to physiotherapy management of HD was developed. In order to facilitate the document’s practical usability among clinicians, a treatment-based classification system is proposed to categorize patients based on presenting signs and symptoms, and provide a foundation for development of a more standardized intervention approach. Discussion: The Physiotherapy Guidance for HD is a comprehensive, consensus- and evidence-based document that can be used by physiotherapists to implement a plan of care that is currently consistent with best practice for individuals at all stages of HD. As evidence becomes available, future systematic reviews will be required in order to inform further development. The use of treatment-based classifications, which aim to better categorize common signs and symptoms and link them to appropriate intervention plans, may be useful in relatively rare diseases, such as HD, to aid clinical reasoning and promote effective outcome evaluation.
TL;DR: In this article, the effects on communication of cognitive-linguistic and prosody perception and production are highlighted, even when listeners perceive no frank changes in the early stages of Parkinson's disease.
Abstract: SUMMARY This article covers recent developments in the understanding of communication changes in idiopathic Parkinson’s disease and selected issues in intervention. By contrast to earlier narrow considerations of voice and speech, the effects on communication of cognitive–linguistic and prosody perception and production are also highlighted. Decline can occur from the earliest stages, even when listeners perceive no frank changes. Communication may be relatively trouble-free in one-to-one quiet clinical situations but declines in multi-talker, dual/competing task situations. Assessment should reflect this to gain more accurate insights into daily living performance. Currently, therapies focusing on attention-to-effort in voice production and on monitoring the sense of effort and loudness production appear to be most successful. Medical and surgical treatments have little effect on intelligibility and communication and may even exacerbate the situation.
TL;DR: The everyday functional problems experienced by people with Huntington’s disease, the role of the occupational therapist, the systematic literature review which preceded the formulation of the guidelines are described and excerpts from the guideline are used to illustrate some of the issues raised.
Abstract: SUMMARY This paper introduces the ‘Guidelines for Occupational Therapists working with people with Huntington’s disease’ which is currently being developed by the European Huntington’s Disease Network (EHDN) Standards of Care Occupational Therapists working group. This article aims to describe the everyday functional problems experienced by people with Huntington’s disease, the role of the occupational therapist, the systematic literature review which preceded the formulation of the guidelines and use excerpts from the guideline to illustrate some of the issues raised.
TL;DR: Cardiovascular and metabolic risk factors for cognitive decline and dementia, such as hypertension, diabetes, metabolic syndrome and obesity, may be modified by diet and healthy dietary habits at midlife could contribute to decrease the risk of late-life dementia.
Abstract: SUMMARY Cardiovascular and metabolic risk factors for cognitive decline and dementia, such as hypertension, diabetes, metabolic syndrome and obesity, may be modified by diet. Moreover, several classes of nutrients, including long-chain ω-3 polyunsaturated fatty acids, antioxidants (i.e., vitamins C and E, carotenoids and polyphenols) and vitamins B and D can lower inflammation and oxidative stress associated with neurodegeneration, in addition to specific neuroprotective effects. However, most randomized clinical trials of supplementation with high doses of single nutrients have been disappointing. Healthy dietary patterns, such as the Mediterranean diet, combine several classes of nutrients that could work in synergy to slow down cognitive decline. Healthy dietary habits at midlife could contribute to decrease the risk of late-life dementia.
TL;DR: Combination of interventions, such as non-pharmacologic treatments, pharmacotherapy, and medical foods, with complementary mechanisms of action may provide a rational approach that may result in maximum preservation of cognitive function in patients with AD.
Abstract: SUMMARY Alzheimer’s disease (AD) is characterized by progressive declines in cognitive function and the ability to carry out activities of daily living as well as the emergence and worsening of behavioral/neuropsychiatric symptoms. While there is no cure for AD, nonpharmacologic interventions and medications that modulate neurotransmission can slow symptomatic progression. Medical foods may also be useful as adjuncts to pharmacologic agents in AD. Medium-chain triglycerides aimed at improving cerebral metabolism significantly improve AD Assessment Scale-Cognitive scores when added to ongoing pharmacotherapy in patients with mild-to-moderate AD. The combination of interventions, such as nonpharmacologic treatments, pharmacotherapy and medical foods, with complementary mechanisms of action may provide a rational approach that may result in maximum preservation of cognitive function in patients with AD.
TL;DR: The current relationship between family caregivers and home care providers needs to be reconceptualized from one that is nonsupportive and potentially exploitive, to one that fosters a collaborative, empowering relationship.
Abstract: SUMMARY The current relationship between family caregivers and home care providers needs to be reconceptualized from one that is nonsupportive and potentially exploitive, to one that fosters a collaborative, empowering relationship. I present my argument in three parts. First, I focus on the problem of the current complementary model of care, one in which home care workers supplement and compensate for the care provided by family caregivers. In effect, I outline how this model of dementia home care in Canada perpetuates an inequitable distribution of care between formal and familial caregivers. Here, I underscore the negative consequences of this complementary approach on the caregiver–provider relationship. In the second part, I call for a reappraisal of ‘supportive relationships’ between home support workers and caregivers, identifying underlying values and principles (e.g., quity) that I believe should inform home care practices and policies in the future. Finally, I propose some possible strategies th...
TL;DR: In this article, an overview of MRI changes in DLB and AD, the relationship to other imaging modalities such as single-photon emission computed tomography and PET, highlighting the differences between the conditions currently applicable to the clinical setting, as well as recent developments in MRI me...
Abstract: SUMMARY Despite the characteristic clinical differences between dementia with Lewy bodies (DLB) and Alzheimer’s disease (AD), a large degree of overlap exists. In particular, the clinical diagnostic criteria for DLB lack sensitivity so that many cases of DLB will be missed during life. MRI techniques offer a noninvasive method to assess the brain structure and function in more detail. In particular, advanced MRI methods such as diffusion-tensor imaging, functional MRI, arterial spin labeling (perfusion) and magnetic resonance spectroscopy may offer more sensitive methods to detect early and, possibly, preclinical change in dementia and contribute to our understanding of the differences between AD and DLB. This paper provides an overview of MRI changes in DLB and AD, the relationship to other imaging modalities such as single-photon emission computed tomography and PET, highlighting the differences between the conditions currently applicable to the clinical setting, as well as recent developments in MRI me...
TL;DR: The findings in structural MRI studies, especially in those studies utilizing the most recent methods, and the accuracies of those new methods in differentiating AD from healthy controls and stable MCI from progressive MCI are reviewed.
Abstract: SUMMARY New research criteria for Alzheimer’s disease (AD) and mild cognitive impairment (MCI) emphasize the use of imaging biomarkers in clinical diagnosis of these disorders. The volume loss of medial temporal lobe structures, especially hippocampal atrophy, is the best validated marker of AD. Manual tracing on MRI is the present gold standard for evaluating hippocampal volume; however, it is laborious and tracer-dependent. We categorized the most recent full- or semi-automated methods by the nature of the output of the method: size and shape of subcortical structures, cortical thickness, atrophy-rate and voxel- and region-based characteristics. The features of each method are introduced. The findings in structural MRI studies, especially in those studies utilizing the most recent methods, and the accuracies of those new methods in differentiating AD from healthy controls and stable MCI from progressive MCI are reviewed.
TL;DR: Important clinical characteristics of ICDs in PD are outlined, relevant neurocognitive and neurobiological studies are reviewed, and possible ways to prevent and manage I CDs inPD are discussed.
Abstract: SUMMARY Given that impulse control disorders (ICDs) have been identified among a considerable minority of Parkinson’s disease (PD) patients, these conditions have gained increased clinical and research attention in the past decade. Dopamine-replacement therapies, taken to ameliorate PD symptoms, have been associated with ICDs in PD. Unfortunately, there are relatively sparse empirical data regarding how best to address ICDs in PD patients. Conversely, progress has been made in understanding the clinical, neurobiological and cognitive correlates of ICDs in PD. Some of these findings may inform possible courses of action for care providers working with PD patients with ICDs. The literature on ICDs in non-PD populations may also be informative in this regard. The goals of the present article are to outline important clinical characteristics of ICDs in PD, briefly review relevant neurocognitive and neurobiological studies and discuss possible ways to prevent and manage ICDs in PD.
TL;DR: It is intended that by providing and subsequently promoting these guidelines, it will focus the dental professions work on this condition and the implementation of good care for people with Huntington’s disease.
Abstract: SUMMARY These guidelines present an overall strategy for oral healthcare based on the principles of achieving a disease-free, pain-free and safe mouth. The standards of care referred to in the document seek to provide guidelines for the care and treatment that is no less a standard provided for an individual that does not have this condition. Such care and treatment will take into account the health and safety of each individual within the context of their condition. Particular emphasis is placed on dental professionals working within a multidisciplinary team, focusing on prevention of oral disease and providing treatment appropriate to the various stages of the progression of this condition. It is intended that by providing and subsequently promoting these guidelines, it will focus the dental professions work on this condition and the implementation of good care for people with Huntington’s disease. 1
TL;DR: An overview of the current knowledge of the structure and function of the corpus callosum and its role in aging and neurodegenerative diseases is provided.
Abstract: SUMMARY The corpus callosum (CC) is a major white matter bundle that connects primarily homologous areas of the cortex. The structure may be involved in interhemispheric communication and enable the lateralization of certain cerebral functions. Despite its possible role as the main conduit for interhemispheric communication, interest from researchers has, at times, been sparse. Renewed interest has led to research that has shown that the CC may play a role in both cognitive aging and neurodegenerative diseases including Alzheimer´s disease and frontotemporal dementia. Studies employing structural MRI and diffusion-weighted MRI have found distinct subregional patterns of callosal atrophy in aging, Alzheimer´s disease and frontotemporal dementia. Furthermore, imaging studies may help to elucidate the underlying pathological mechanisms of callosal atrophy. The present review aims to provide an overview of the current knowledge of the structure and function of the CC and its role in aging and neurodegenerativ...
TL;DR: A review of the literature on sleep-wake problems in patients with ALS, focusing on the implications for patient management can be found in this article, where the authors discuss the relationship between the severity of disease and the neurodegenerative process.
Abstract: SUMMARY Sleep–wake problems are frequent, although unrecognized, complications of amyotrophic lateral sclerosis (ALS). Sleep disorders such as insomnia, sleep-disordered breathing and restless legs syndrome have all been reported in patients with ALS, despite the limited number of studies and the small populations investigated so far. Sleep disturbances gradually worsen with disease progression, suggesting a relationship between the severity of disease and the neurodegenerative process. However, poor sleep can also be a consequence of several disturbances such as anxiety, depression, pain, choking, sialorrhea, fasciculations, cramps, nocturia and the inability to get comfortable and move freely in bed. Sleep disorders may have many reflections on patients with ALS, including excessive daytime somnolence, fatigue, impaired cognition, reduced quality of life and survival. This article reviews the recent literature on sleep–wake problems in patients with ALS, focusing on the implications for patient management.
TL;DR: The main findings from epidemiological cohorts on dementia pathology are compared to case–control and convenience samples, and findings on the different neuropathological features of dementia, its risk factors and clinical course are summarized.
Abstract: SUMMARY Identification of the determinants and risk factors for dementia, in addition to the underlying brain changes associated with disease, is fundamental to the development of dementia therapeutics. The gold standard to achieving this is through population-based (epidemiological) studies of the biology of aging, cognitive decline and dementia. In this paper, the main findings from epidemiological cohorts on dementia pathology are compared to case–control and convenience samples, and findings on the different neuropathological features of dementia, its risk factors and clinical course are summarized. The strengths and weakness of different research designs, the keys aspects of disease these have identified and the targets that have been subsequently developed will be discussed. It is highlighted that dementia within epidemiological frameworks is found to be a heterogeneous disease. For treatment to be effective it will therefore need to reflect not only population variation in patterns of pathology, bu...
TL;DR: The Alzheimer's Association has developed and implemented a new clinical trial-matching service designed to increase awareness of ongoing clinical trials among potential participants, improve initial matching of potential participants to enrollment criteria and thus help investigators achieve enrollment goals.
Abstract: SUMMARY Many clinical trials for Alzheimer’s disease and related disorders struggle to achieve enrollment goals, hampering the progress of research. To help address this problem, the Alzheimer’s Association® has developed and implemented a new clinical trial-matching service designed to increase awareness of ongoing clinical trials among potential participants, improve initial matching of potential participants to enrollment criteria and thus help investigators achieve enrollment goals. This service, Alzheimer’s Association TrialMatch™, uses numerous personal and disease characteristics to match users with potential clinical trials that are currently enrolling participants. It provides users with direct access to trained professional consultants who interpret and explain an individual’s eligibility for specific clinical trials and is designed for use by patients, caregivers, healthy volunteers, physicians and other healthcare providers and researchers. During the first year of operation (July 2010–June 20...
TL;DR: The assumption that analysis of speech may become a helpful tool for the monitoring of presumably nondopaminergic features and disease progression in Parkinson’s disease and for the differential diagnoses of different parkinsonian syndromes is justified.
Abstract: SUMMARY Abnormalities of voice and speech performance are common and are early features of Parkinson’s disease (PD) and other hypokinetic rigid syndromes, such as progressive supranuclear palsy. The typical pattern of dysarthria in PD is characterized by reduced loudness and pitch variability, often in combination with abnormalities of voice, articulatory insufficiencies and irregularity of speech rate and rhythm. Recent preliminary investigations on the progression of dysarthria in the course of PD, and on the comparison of dysarthria between PD and progressive supranuclear palsy speakers, justify the assumption that analysis of speech may become a helpful tool for the monitoring of presumably nondopaminergic features and disease progression in PD and for the differential diagnoses of different parkinsonian syndromes.
TL;DR: regional prevalence differences in frontotemporal disease prodrome appear insignificant and multifactorial, while being consistent with the toxicity model implic...
Abstract: SUMMARY Aims: To investigate regional and gender differences in prevalence rates and the pattern of cognitive and behavioral impairment in amyotrophic lateral sclerosis. Materials & methods: One hundred and ten subjects (55 male) from 14 amyotrophic lateral sclerosis clinics were cross-sectionally evaluated with the Penn State Brief Exam of frontal and temporal dysfunction syndromes. Results: Prevalence rates of cognitive impairment and behavioral impairment were statistically equivalent among rural, suburban and urban subgroups. Females evidenced significant strengths in fluency and limitations in configurational processing. Patterns of regional findings suggested greater frontal cortical involvement in the rural sample. Females demonstrated more bihemispheric involvement in comparison to more left hemispheric involvement for males. Conclusions: Regional prevalence differences in frontotemporal disease prodrome appear insignificant and multifactorial, while being consistent with the toxicity model implic...