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Books on the topic 'Progressive neurodegenerative diseases'
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1
Lei, Peng, Scott Ayton, and Ashley I. Bush. Metal-Protein Attenuating Compounds in Neurodegenerative Diseases. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780190233563.003.0015.
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Neurodegenerative disorders including Alzheimer’s (AD), Parkinson’s disease (PD), Huntington’s disease (HD), and amyotrophic lateral sclerosis (ALS) are progressive diseases of the aging population with currently few therapeutic options. While aggregation and deposition of disease-specific proteins link the pathologies of these diseases, targeting these aggregating proteins with therapeutics has not yet been successful in clinical trial. This chapter profiles metals (copper, zinc, and iron) as alternative drug targets for neurodegeneration. Complex changes to metals occur in these neurodegenerative diseases. Accumulating evidences have demonstrated that perturbations to metal homeostasis contribute to the progression of neuronal dysfunction and death. Importantly, several phase II trials have shown that correcting metal dyshomeostasis improves clinical outcomes; the chapter argues that it is now time to explore the therapeutic utility of metal-based drugs in larger, phase III trials.
2
Manni, Raffaele, and Michele Terzaghi. Sleep disorders in neurodegenerative diseases other than Parkinson disease and multiple system atrophy. Edited by Sudhansu Chokroverty, Luigi Ferini-Strambi, and Christopher Kennard. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199682003.003.0026.
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This chapter examines sleep–wake disturbances occurring in the most common neurodegenerative disorders. It reviews sleep alterations in Alzheimer disease and dementia with Lewy bodies. It also discusses sleep problems in progressive supranuclear palsy, corticobasal degeneration, Huntington disease, and spinocerebellar ataxias. Status dissociatus as an extreme form of sleep alteration in advanced neurodegenerative diseases is also considered. The chapter reviews the key points for the treatment of disrupted sleep in neurodegenerative disorders, with a focus on pharmacological and nonpharmacological interventions to improve sleep continuity. It also summarizes paraphysiological age-related changes in sleep patterns and discusses indications and procedures for clinical and instrumental assessment of sleep disorders in neurodegenerative disorders.
3
Safar, Jiri G. Prion Paradigm of Human Neurodegenerative Diseases Caused by Protein Misfolding. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780190233563.003.0005.
Full textAbstract:
Data accumulated from different laboratories argue that a growing number of proteins causing neurodegeneration share certain characteristics with prions. Prion-like particles were produced from synthetic amyloid beta (Aβ) peptides of Alzheimer’s disease (AD), from recombinant α-synuclein linked to Parkinson’s disease (PD), and from recombinant tau associated with frontotemporal dementias (FTD). Evidence from human prions reveals that variable disease phenotypes, rates of propagation, and targeting of different brain structures are determined by distinct conformers (strains) of pathogenic prion protein. Recent progress in the development of advanced biophysical tools identified the structural characteristics of Aβ in the brain cortex of phenotypically diverse AD patients and thus allowed an investigation of the prion paradigm of AD. The findings of distinctly structured strains of human brain Aβ, forming a unique spectrum of oligomeric particles in the cortex of rapidly progressive cases, implicates these structures in variable rates of propagation in the brain.
4
Pillai, Jagan A., and Jeffrey L. Cummings. Conclusions on Neurodegenerative Disorders. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780190233563.003.0019.
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Neurodegenerative diseases strip an individual of his or her cognitive powers, functional capacity, and autonomy while increasing dependence on others. They are an existential threat to an aging society in its ability to provide a meaningful and high quality of life to all its citizens. The classical view of neurodegenerative disorders (NDDs) emphasized the distinctness of each NDD ,with a definable clinical syndrome of neurological deficits, behavioral changes, and progressive functional decline, underpinned by inexorable neuronal loss that is pathological for the age of the subject. Neurodegenerative Disorders: Unifying Principles has covered each of these themes from multiple expert domains to basic science to clinical therapeutics. This detailed overview emphasizes the work recently accomplished to uncover shared themes across NDDs and further posits questions for the future.
5
Kreisl, William C., and Christiane Reitz. Diagnosis and Epidemiology of Dementia. Edited by Dennis S. Charney, Eric J. Nestler, Pamela Sklar, and Joseph D. Buxbaum. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780190681425.003.0050.
Full textAbstract:
Dementia is a neurodegenerative clinical syndrome encompassing a wide range of diseases characterized by progressive deterioration in cognitive ability, function and behavior. More than 24 million people worldwide are currently affected, and this prevalence is predicted to quadruple by the year 2050. The most common cause of dementia is Alzheimer’s disease, clinically characterized by a progressive impairment in cognitive function. To date, Alzheimer’s disease cannot be prevented or cured; the available treatments only slightly improve cognitive symptoms. In this chapter, we provide an overview of the most common neurodegenerative causes of dementia, beginning with Alzheimer’s disease, and summarize current information relating to the prevalence and incidence of dementia.
6
Attems, Johannes, and Kurt A. Jellinger. Neuropathology. Oxford University Press, 2013. http://dx.doi.org/10.1093/med/9780199644957.003.0006.
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This chapter describes the main neuropathological features of the most common age associated neurodegenerative diseases including Alzheimer's disease, Parkinson's disease and dementia with Lewy bodies as well as other less frequent ones such as multiple system atrophy, Pick's disease, corticobasal degeneration, progressive supranuclear palsy, argyrophilic grain disease, neurofibrillary tangle dominant dementia, frontotemporal lobar degeneration with TDP-43 pathology and Huntington's disease. Likewise cerebral amyloid angiopathy, hippocampal sclerosis, vascular dementia and prion diseases are described. A main aim of this chapter is to assist the reader in interpreting neuropathological reports, hence criteria for the neuropathological classifications of the major diseases are provided. One section covers general considerations on neurodegeneration and basic pathophysiological mechanisms of tau, amyloid-β, α-synuclein, TDP-43 and prions are briefly described in the sections on the respective diseases. Finally, one section is dedicated to cerebral multimorbidity and we give a view on currently emerging neuropathological methods.
7
Cummings, Jeffrey L., and Jagan A. Pillai. Neurodegenerative Diseases. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780190233563.003.0001.
Full textAbstract:
Neurodegenerative diseases (NDDs) are growing in frequency and represent a major threat to public health. Advances in scientific progress have made it clear that NDDs share many underlying processes, including shared intracellular mechanisms such as protein misfolding and aggregation, cell-to-cell prion-like spread, growth factor signaling abnormalities, RNA and DNA disturbances, glial cell changes, and neuronal loss. Transmitter deficits are shared across many types of disorders. Means of studying NDDs with human iPS cells and transgenic models are similar. The progression of NDDs through asymptomatic, prodromal, and manifest stages is shared across disorders. Clinical features of NDDs, including cognitive impairment, disease progression, age-related effects, terminal stages, neuropsychiatric manifestations, and functional disorders and disability, have many common elements. Clinical trials, biomarkers, brain imaging, and regulatory aspects of NDD can share information across NDDs. Disease-modifying and transmitter-based therapeutic interventions, clinical trials, and regulatory approaches to treatments for NDDs are also similar.
10
Zhou, Juan, and William W. Seeley. Brain Circuits. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780190233563.003.0007.
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Each neurodegenerative disease is defined by selectively vulnerable neurons, regions, networks, and functions, as well as genetic risk factors. In the past decade, new network-sensitive neuroimaging methods have made it possible to test the notion of network-based degeneration in living humans. This chapter focuses on two common causes of dementia, Alzheimer’s disease (AD) and frontotemporal dementia (FTD), but uses these diseases to illustrate class-wide neurodegeneration principles whenever possible. It first introduces two key concepts of neurodegenerative disease selective vulnerability: onset and progression. In parallel, it addresses two distinct but related observations about neurodegenerative disease: clinico-anatomical convergence and phenotypic heterogeneity. It then examines disease onset and models of progression in more detail, based on available neuroimaging evidence. Finally, it touches on the most important frontiers in the field of network-based neurodegeneration.
11
Cader, Sarah. Screening for neurological disease. Edited by Patrick Davey and David Sprigings. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199568741.003.0355.
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Given the nature of some neurological diseases to become progressively disabling, early detection and treatment would be very welcome. However, screening for neurological illnesses in the general population has a number of problems. There are only a few neurological conditions that exist in a detectable asymptomatic state prior to development of clinical disease, and a small number of other conditions have early symptomatic stages before the full impact of the disease manifests. In addition, in many neurological conditions, there are no definitive treatments available. There are, of course, many genetic conditions that have neurological manifestations and could be screened, and early access to neurological assessment for some progressive conditions may enable early intervention. Potential target groups include patients with neurogenetic disorders, patients with neurodegenerative disorders, patients with inflammatory disorders, and patients with metabolic disorders.
12
Zetterberg, Henrik, and Jonathan M. Schott. Fluid Biomarkers Indicative of Neurodegenerative Diseases. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780190233563.003.0008.
Full textAbstract:
A major unifying feature of neurodegenerative diseases (NDDs) is excessive neuronal loss. Depending on when and where this occurs, patients may express distinct neurological and psychiatric symptoms. Neurodegeneration is accompanied by protein aggregation, inflammation, and microglial activation that may be drivers of the disease or in some circumstances may be protective reactions to the neurodegenerative process. A key development over the past decade has been our ability to leverage these accompanying central nervous system changes to develop clinically impactful biomarkers of specific NDDs. This has been crucial in helping us develop an understanding the time line of progression of these diseases, in their early diagnosis and to help target patients appropriately in therapeutic clinical trials, This chapter gives an overview of both established and novel fluid biomarkers for neurodegeneration, protein accumulation, inflammation, and microglial activation across different neurodegenerative diseases. Common as well as disease-specific biomarker changes in cerebrospinal fluid and blood are emphasized.
13
Albin, Roger L., and Henry L. Paulson. Huntington Disease. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199937837.003.0007.
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A member of the expanded polyglutamine (polyQ) repeat family of neurodegenerative disorders, Huntington disease (HD) is a rare, autosomal, dominantly inherited neuropsychiatric disorder. Characterized by midlife onset, HD exhibits progressive motor, behavioral, and cognitive changes. There is no effective treatment and death usually ensues 15 to 20 years after diagnosis. The expanded polyglutamine repeat causes multiple cellular dysfunctions to induce neurodegeneration. Many brain regions are affected in HD though striatal degeneration is particularly prominent. Widespread availability of specific genetic testing facilitates diagnosis. Management is largely supportive care.
14
Ryskamp, Daniel A., Elena Popugaeva, and Ilya Bezprozvanny. Calcium Hypothesis of Neurodegeneration. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780190233563.003.0003.
Full textAbstract:
Aged-related changes in neuronal physiology and stressors related to specific neurodegenerative diseases collude to undermine neuronal calcium homeostasis, which is a common pathological feature in the initiation and progression of Alzheimer’s disease(AD), Huntington’s disease (HD), Parkinson’s disease, amyotrophic lateral sclerosis, spinocerebellar ataxias, glaucoma, and several other neurodegenerative disorders. Mechanisms of calcium mishandling in these diseases are discussed in this chapter by focusing on HD as an example of a monogenic disease and AD as a multifactorial disease. As aberrant Ca2+ signals are particularly toxic to synaptic elements, this chapter further focuses on issues relevant to development of therapeutics that maintain neuronal circuitry and function through the stabilization of Ca2+ regulation. Ultimately, therapies that promote calcium homeostasis will likely be most effective when used in combination with disease-specific treatment strategies such as elimination of toxic Aβ in AD or polyglutamine expanded Huntingtin protein in HD.
15
Shaw, Pamela. The motor neurone disorders. Oxford University Press, 2011. http://dx.doi.org/10.1093/med/9780198569381.003.0524.
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The motor neurone diseases are a group of disorders in which there is selective loss of function of upper and/or lower motor neurones in the motor cortex, brainstem, and spinal cord resulting in impairment in the nervous system control of voluntary movement. The term ‘motor neurone disease’, often abbreviated to ‘MND’, is used differently in different countries. In the United Kingdom it is used as an umbrella term to cover the related group of neurodegenerative disorders including amyotrophic lateral sclerosis, the commonest variant, as well as progressive muscular atrophy, primary lateral sclerosis, and progressive bulbar palsy. However, in many other countries amyotrophic lateral sclerosis, referred to as ALS, has been adopted as the umbrella term for this group of clinical variants of motor system degeneration. There is a tendency now internationally to use the ALS/MND abbreviation to cover this group of conditions. Careful diagnosis within the motor neurone diseases is essential for advising about prognosis, potential genetic implications, and for identifying those with acquired lower motor neurone syndromes who may benefit for the administration of immunomodulatory therapy.
16
Nakamura, Tomohiro, and Stuart A. Lipton. Neurodegenerative Diseases as Protein Misfolding Disorders. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780190233563.003.0002.
Full textAbstract:
Neurodegenerative diseases (NDDs) often represent disorders of protein folding. Rather than large aggregates, recent evidence suggests that soluble oligomers of misfolded proteins are the most neurotoxic species. Emerging evidence points to small, soluble oligomers of misfolded proteins as the cause of synaptic dysfunction and loss, the major pathological correlate to disease progression in many NDDs including Alzheimer’s disease. The protein quality control machinery of the cell, which includes molecular chaperones as found in the endoplasmic reticulum (ER), the ubiquitin-proteasome system (UPS), and various forms of autophagy, can counterbalance the accumulation of misfolded proteins to some extent. Their ability to eliminate the neurotoxic effects of misfolded proteins, however, declines with age. A plausible explanation for the age-dependent deterioration of the quality control machinery involves compromise of these systems by excessive generation of reactive oxygen species (ROS), such as superoxide anion (O2-), and reactive nitrogen species (RNS), such as nitric oxide (NO). The resulting redox stress contributes to the accumulation of misfolded proteins. Here, we focus on aberrantly increased generation of NO-related species since this process appears to accelerate the manifestation of key neuropathological features, including protein misfolding. We review the chemical mechanisms of posttranslational modification by RNS such as protein S-nitrosylation of critical cysteine thiol groups and nitration of tyrosine residues, showing how they contribute to the pathogenesis of NDDs.
17
Quarrell, Oliver W. J., Helen M. Brewer, Ferdinando Squitieri, Roger A. Barker, Martha A. Nance, and G. Bernhard Landwehrmeyer, eds. Juvenile Huntington's Disease. Oxford University Press, 2012. http://dx.doi.org/10.1093/med/9780199236121.001.0001.
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Huntington's disease (HD) is an inherited progressive neurodegenerative disorder. Although onset of HD usually occurs in adulthood, a small percentage of cases develop symptoms before 20 years of age (juvenile-onset Huntington's Disease or JHD). This resource summarises the clinical and scientific knowledge available on JHD.
18
Walsh, Richard A. Falling All the Time. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780190607555.003.0010.
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Progressive supranuclear palsy is a four-repeat tauopathy that is confirmed, like all neurodegenerative disease, at postmortem examination. An expanding group of clinical syndromes are now linked with this pathology in its early stages, although with disease progression there tends to be greater clinical similarity with the classical Richardson’s syndrome, an akinetic rigid form of parkinsonism with a progressive supranuclear gaze palsy and prominent frontal cognitive impairment. Currently, there are no disease-modifying therapies for progressive supranuclear palsy; however, there continues to be interest in immunotherapies targeted at tau pathology. Liaison with colleagues with an interest in palliative neurology is appropriate for patients in the advanced stages of the disease.
19
Jaworska, Agnieszka. Ethical dilemmas in neurodegenerative disease: Respecting patients at the twilight of agency. Oxford University Press, 2017. http://dx.doi.org/10.1093/oso/9780198786832.003.0015.
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This chapter focuses on dilemmas faced by caregivers of Alzheimer’s patients in cases in which current preferences of such patients come into conflict with the attitudes and values the person held during better health. To which set of preferences should conscientious caregivers give priority? The chapter argues that many Alzheimer’s patients, at least up to the middle stages of the disease, are still capable of rudimentary autonomy and that they still have authority concerning their well-being. The capacity to value is often not completely lost in dementia, and insofar as it is not, respect for the immediate interests of a demented person compromises neither their well-being nor the respect for their autonomy. In the postscript, emerging neuroscience evidence is discussed that may suggest that, for a time, the capacity to value is not only preserved but even enhanced in the progression of Alzheimer’s disease.
20
Lleó, Alberto, and Rafael Blesa. Clinical course of Alzheimer’s disease. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780198779803.003.0004.
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Alzheimer’s disease (AD) is an age-related neurodegenerative disorder, with onset usually in late life, characterized by progressive cognitive impairment, a variety of behavioural symptoms, and impairment in the activities of daily living. The initial symptom in typical AD is episodic memory loss, which reflects hippocampal dysfunction. The memory deficits are very characteristic with low recall performance despite retrieval facilitations with cueing. These initial deficits can be identified by appropriate cognitive tests. Behavioural symptoms can be present at early stages of the disease (even in pre-clinical states), although the frequency increases as the disease progresses. In the past decade there has been a growing interest in characterizing these pre-clinical and prodromal stages as treatments are expected to be more effective in these phases.
21
Sedel, Frédéric. Niemann-Pick Disease Type C. Oxford University Press, 2016. http://dx.doi.org/10.1093/med/9780199972135.003.0053.
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Niemann-Pick disease type C (NPC) is a fatal neurovisceral lipid storage disease of autosomal inheritance resulting from mutations in either the NPC1 (95% of families) or NPC2 gene. The encoded proteins appear to be involved in lysosomal/late endosomal transport of cholesterol, glycolipids, and other molecules, but their exact function is still unknown. The clinical spectrum of the disease ranges from a neonatal rapidly fatal disorder to an adult-onset chronic neurodegenerative disease characterized prominently by psychiatric disorders, cerebellar ataxia, cognitive decline, and vertical supranuclear gaze palsy. Miglustat is the only treatment approved to date which has been demonstrated to slow or halt disease progression.
22
McShane, Tony, Peter Clayton, Michael Donaghy, and Robert Surtees. Neurometabolic disorders. Oxford University Press, 2011. http://dx.doi.org/10.1093/med/9780198569381.003.0213.
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Various disorders result from genetically determined abnormalities of enzymes, the metabolic consequences of which affect the development or functioning of the nervous system. The range of metabolic disturbances is wide, as is the resultant range of clinical syndromes. Although most occur in children, some can present in adult life, and increasing numbers of affected children survive into adult life. In some, specific treatments are possible or are being developed. The last 20 years has seen a considerable expansion in our understanding of the genetic and metabolic basis for many neurological conditions. Particular clinical presentations of neurometabolic disorders include ataxias, movement disorders, childhood epilepsies, or peripheral neuropathy. Detailed coverage of the entire range of inherited metabolic diseases of the nervous system is available in other texts (Brett 1997; Scriver et al. 2001; Menkes et al. 2005).Treatment is possible for some metabolic diseases. For instance, the devastating neurological effects of phenylketonuria have been recognized for many years. Neonatal screening for this disorder and dietary modification in the developed world has removed phenylketonuria from the list of important causes of serious neurological disability in children. This success has led to new challenges in the management of the adult with phenylketonuria and unexpected and devastating effect of the disorder on the unborn child of an untreated Phenylketonuria mother. More recently Biotinidase deficiency has been recognized as an important and easily treatable cause of serious neurological disease usually presenting with early onset drug resistant seizures. This and some other neurometabolic diseases can be identified on neonatal blood screening although a full range of screening is not yet routine in the United Kingdom. More disorders are likely to be picked up at an earlier asymptomatic stage as the sophistication of screening tests increases (Wilcken et al. 2003; Bodamer et al. 2007).Although individual metabolic disorders are rare, collectively such disorders are relatively common. In reality most clinicians will see an individual condition only rarely in a career. Furthermore, patients with certain rare conditions are often concentrated in specialist referral centres, further reducing the exposure of general and paediatric neurologists to these disorders. A recent study into progressive intellectual and neurological deterioration, PIND, gives some information about the relative frequency and distribution of some childhood neurodegenerative diseases in the United Kingdom (Verity et al. 2000; Devereux et al. 2004). Although primarily designed to identify any childhood cases of variant Creutzfeldt- Jakob disease, the study also provided much information about the distribution of neurometabolic disease in children in the United Kingdom. The commonest five causes of progressive intellectual and neurological deterioration over 5 years were Sanfilippo syndrome, 41 cases, adrenoleukodystrophy, 32 cases, late infantile neuronal ceroid lipofuschinosis, 32 cases, mitochondrial cytopathy, 30 cases, and Rett syndrome, 29 cases. Notably, geographical foci of these disorders were also found and correlate with high rate of consanguinity in some local populations.
23
Armstrong, Richard. Dementia. Edited by Patrick Davey and David Sprigings. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199568741.003.0228.
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Dementia is a syndrome defined by a persistent, progressive decline in multiple cognitive functions to a degree sufficient to detrimentally impact activities of daily living and social function. The syndromic diagnosis of dementia is useful, since the general management and economic burden of these patients remains similar, irrespective of etiology. However, a more precise etiological diagnosis must be sought, since disease-specific treatment is increasingly likely to be appropriate. The term ‘mild cognitive impairment’ (MCI) refers to an objective impairment in a cognitive function (usually memory) which does not impair activities of daily living. The aim underlying the use of this term is to identify those with the early pathophysiological changes of neurodegenerative disease. Nonetheless, ‘MCI’ is not synonymous with ‘early dementia’, as a significant proportion of those with MCI will not worsen. The term should therefore be used cautiously.
24
Pagnini, Francesco, and Zachary Simmons, eds. Amyotrophic Lateral Sclerosis. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780198757726.001.0001.
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Amyotrophic lateral sclerosis: Understanding and optimizing quality of life and psychological well-being presents a comprehensive and up-to-date review of the enhancement of the lives of people with amyotrophic lateral sclerosis (ALS) and their caregivers. ALS is a progressive, fatal neurodegenerative disorder. No current medical therapy can reverse or stop its progression, and the promotion of quality of life and psychological well-being is a central component of ALS care. Health care professionals who work in this field should incorporate attention to psychological, emotional, and relational aspects of the disease into their approach to care. This book provides some of the knowledge and direction necessary for optimizing the quality of care for individuals with ALS and their caregivers. Topics discussed include an ALS-centred view of quality of life, depressive features, anxiety, resilience, cognitive impairment, complementary and alternative medicines, and psychological research. Specific elements of ALS, such as end-of-life concerns and bulbar dysfunction, are described through the lens of their psychological impact. There is extensive discussion of the development of new psychological treatments, as well as the impact and incorporation of new technologies, with the goal of fostering optimal quality of life and psychological well-being as key parts of a holistic approach to care for the patients and for those who are close to such individuals.