Search Results

Patients with frontotemporal dementia (FTD) manifest severe behavioral and personality alterations associated with orbitofrontal cortex (OFC) dysfunction. This chapter provides a review of the clinical features, neuropathology, neuroimaging, genetics, and neuropsychology of FTD as well as presenting two prototypical cases that provide a clinical picture of the disorder. Neuropathological and neuroimaging studies have identified the OFC as the brain region most prominently involved in the frontal-variant of FTD. The neuroanatomy, circuitry, and functions of the OFC are summarized, emphasizing its role in emotional and social cognition. Theories involving deficits in recognition of emotional expression, decision-making, and theory of mind have been proposed to explain the mechanism underlying the clinical expression of FTD, and the OFC is intimately involved in studies examining the neural basis underlying these deficits.

Frontotemporal lobar degeneration (FTLD) is a syndrome of focal, often asymmetric, neurodegeneration presenting clinically as a progressive dementia associated with focal atrophy of the frontal and/or temporal lobes. The disease is capable of producing striking changes in personality, behaviour, and language, with a diverse range of clinical presentations. Two major clinical presentations of FTLD are recognized. In the frontal variant — usually designated frontotemporal dementia (FTD) — the emergence of disinhibition, mental rigidity, stereotyped and perseverative behaviour, hyperorality, and loss of insight give rise to marked changes in personality. An aphasic variant — designated primary progressive aphasia (PPA) — is also recognized. The fluent variant appear to have difficulty expressing and understanding meaning, a syndrome that has been given the name semantic dementia (SD). In contrast, the nonfluent variant — progressive nonfluent aphasia (PNFA). Because FTLD can be associated with degeneration of cortical and bulbar motor neurons and anterior horn cells of the spinal cord, some patients with FTD, SD, or PNFA also meet clinical criteria for amyotrophic lateral sclerosis (ALS).

Magnetic resonance imaging (MRI) has opened up the way to diagnose dementia in vivo. It provides clear evidence for hippocampal atrophy in Alzheimer's disease (AD), lobar atrophy in frontotemporal lobar degeneration (FTLD), vascular changes in VaD, and specific findings in some rare forms of dementia. In addition, the traditional role of excluding space-occupying lesions has been kept and the combination of both aspects has rendered MRI indispensable in the diagnostic work-up.

The clinical syndrome of dementia can occur in a range of conditions. Some dementia causing diseases are clearly genetic in origin, such as Huntington's disease (HD) or familial Alzheimer's disease (AD). However, most people with dementia have no obvious cause for their condition. The clearest risk factor for dementia is increasing age, with prevalence approximately doubling with each increase of 5.1 years. This chapter provides an overview of familial AD and its causative genes, familial frontotemporal dementias, and related conditions with non-Alzheimer pathology: these are the most common conditions likely to be seen in memory clinics. It then mentions rarer dementia causing diseases, including HD and familial prion diseases, and concludes by considering some practical questions that occur in clinical settings.

Philosophers have hypothesized that one of the defining characteristics of being human is the production and appreciation of art. Beginning in the early 20th century, observations following isolated strokes or brain surgery supported theories that artistic behaviors were mediated by complex interactions between the frontal and parietal lobes. While these findings were instrumental in establishing the foundations of functional neuroanatomy, the association between artistic behavior and brain circuitry remained crude at best. With advances in molecular biology, improved clinical measures, and sophisticated neuroimaging techniques, the study of neurodegenerative disease has become a powerful method for investigating behavior. Already, the study of neurodegenerative disease has yielded significant insights into the process of artistic behavior. In this chapter we present an overview of visuospatial functional anatomy, how neurodegenerative disease affects artistic sensibility, and detail the paradoxical functional facilitation of artistic ability in patients diagnosed with frontotemporal dementia. Specifically, the behaviors we document developed in the setting of profound and isolated speech dysfunction, a condition known as primary progressive aphasia. Based on the specific aphasia subtype - semantic dementia versus progressive nonfluent aphasia - the quality of these patient’s artistic interest appeared to differ. Taken together, we believe that patients suffering from the language variants of frontotemporal lobar degeneration are uniquely positioned to provide the greatest insights yet into aesthetic choice and artistic creation.

This chapter reviews the neuropsychological assessment of orbitofrontal functions. It starts by describing the major categories of neurological disorders that result in lesions of the orbitofrontal cortex (OFC). Traditional neuropsychological test batteries are generally insensitive to OFC dysfunction. A number of experimental tasks have been proposed as tests of OFC functions. These include objection alternation and object reversal learning tasks, gambling tasks, go/nogo tasks, olfactory recognition, theory of mind and social processing measures, and self- or family-rating scales of the patient's behavior. The sensitivity and specificity of these measures are discussed, with a particular emphasis on issues of functional localization. The chapter concludes with a description of rehabilitation programs aimed at addressing problems associated with OFC dysfunction.

Frontotemporal dementia (FTD) is a family of neurodegenerative diseases and syndromes that most commonly involve the frontal and temporal lobes, producing dramatic alterations of personality, behavior, language, and other cognitive abilities (McKhann et al., 2001). Age of onset tends to be younger than in Alzheimer’s disease (AD), with most patients becoming symptomatic in the sixth decade of life. Although population-based epidemiologic studies of FTD have found a prevalence of approximately 5–10 per 100,000 in patients 50 years of age and older, autopsy-based case series have found that approximately 5% to15% of people with dementia have FTD, a discrepancy suggesting that many cases go undiagnosed during life (Rosso et al., 2003). Recent advances in clinicopathologic correlation have revealed that a number of neurologic conditions previously conceived of as independent disease entities such as progressive supranuclear palsy (PSP), corticobasilar degeneration (CBD), and hippocampal sclerosis dementia are in many cases better classified in the FTD family (McKhann et al., 2001; Blass et al., 2004). Many patients with FTD develop other neurologic syndromes as well, including parkinsonism and amyotrophic lateral sclerosis (ALS). FTD is actually a group of clinical syndromes with overlapping neuropathologies. The clinical expression of the disease relates primarily to the anatomic location of disease involvement rather than the neuropathologic subtype; there are many such subtypes. Clinical variants are most distinct early in the disease course, when the degree of anatomic involvement may be limited to discrete regions. As the disease spreads through the brain, many patients have symptoms that become complex and take on char acteristics of other variants. The first clinical FTD variant is one in which behavioral abnormalities and personality changes dominate the clinical presentation. This syndrome is usually associated with disease involvement of the frontal and anterior temporal lobes. In addition, there are two language presentations: primary progressive aphasia and semantic dementia (McKhann et al., 2001). The neurologic syndromes of PSP, CBD, and ALS with dementia are familiar to the neurologist because of their neurologic symptoms; it is noteworthy that patients with any of the previously mentioned syndromes routinely develop the psychiatric symptoms reviewed below.

This chapter examines how music knowledge is affected in non-Alzheimer’s dementias, with a focus on frontotemporal dementia syndromes. It discusses the clinical and neurobiological rationale for studying music knowledge in non-Alzheimer’s dementia. It describes some of the ways in which music knowledge has been investigated in these patients, what musical abilities are lost or preserved in non-Alzheimer’s dementia, and how this information helps us improve our knowledge of how the brain processes music. The social role of music in evolution is briefly discussed. The chapter examines how emotions generated by and recognized in music are processed differently in frontotemporal dementia compared to healthy individuals and Alzheimer’s disease patients, including the phenomenon of musicophilia, the abnormally enhanced craving for music. Finally it explains how the differences in emotion processing between dementia diseases highlight the need for some selectivity in designing music-based therapies.

This chapter presents a comprehensive understanding of dementia as a commonly encountered condition/syndrome in the nursing care of older adults and offers insights into the health challenges faced by people living with dementia. It will provide nurses with the knowledge to be able to assess, manage, and care for people with dementia in an evidence-based and person-centred way. After a comprehensive overview of the causes, risk factors, and impact of dementia, it will outline best practice to deliver care, as well as to prevent or minimize further ill-health. Nursing assessments and priorities are highlighted throughout, and the nursing management of the symptoms and common health problems associated with dementia can be found in Chapters 14 and 17, respectively. In the past, dementia was most often described in terms of mental disability. However, it is now more often described in terms of neurological disability (i.e. changes in the brain). For example, the Mental Health Foundation describes dementia as:…A decline in mental ability which affects memory, thinking, problem-solving, concentration and perception….The NHS Choices website states:…Dementia describes the effects of certain conditions and diseases on a person’s mental ability, personality and behaviour….Dementia is generally classified according to two international classification systems: the American Psychiatric Association Diagnostic and Statistical Manual of Mental Disorders fourth edition (DSM-IV); and the International Classification of Diseases tenth edition (ICD-10). Dementia can be defined as a syndrome whereby there is gradual death of brain cells, resulting in a loss of brain ability that is severe enough to interfere with normal activities of living for more than 6 months. Problems with brain function should not have been present at birth and it is not associated with a loss or alteration of consciousness. This latter point distinguishes dementia from delirium, which is a state of mental disorientation that can happen if you become medically unwell, also known as an ‘ acute confusional state’ (Royal College of Psychiatrists, 2009). (See Chapter 11). It is vital that nurses hold central what dementia means for people living with it. For example, people will commonly experience changes to their perception, senses, memory, and the range of skills they need to carry out everyday activities.

Progressive neurodegenerative disease that disrupts the interactions between the frontal lobes and the temporal, parietal and occipital lobes, or between the dominant and non-dominant hemispheres has been shown to affect creativity in a myriad of ways. The importance of studying the paradoxical facilitation of behaviors that can help patients achieve ‘flow’ is underscored by the observation that patients who engage in creative activities display many signs of improved quality of life. It is important to note, however, that while the art created by patients with neurodegenerative disease might be hailed as more creative by the artistic community, the brain networks underlying the drive to make these choices may not necessarily be the same as those upon which healthy artists rely.

In a relatively small percentage of ALS patients a frontotemporal-type dementia may occur. A significant percentage of non-demented patents may show mild to moderate cognitive change, predominantly of a dysexecutive nature. Memory deficits may occur, and language involvement has been noted. Disease-related factors, mood disorder, and medication may compound cognitive impairment and must be accounted for when assessing cognition. There has been no research into the effectiveness of psychological interventions for cognitive dysfunction in ALS, although cognitive neuropsychological rehabilitation techniques may be applicable. The development of interventions for behavioural changes that may occur may be guided by approaches used in other neurodegenerative diseases, especially frontotemporal dementias. People with ALS should have access to clinical neuropsychologists who can assess the presence of cognitive dysfunction, undertake behavioural assessments, and work alongside other members of the clinical team to ensure the best outcome for ALS patients and their carers.

Based on neuro-imaging findings, we present a brain model for intelligence and a model for creativity and discuss how genius may emerge from the overlapping and the unique aspects of these models. Intelligence is associated with integrity of the dorsolateral prefrontal cortex and creative achievement with lower volumes of the orbitofrontal cortex. Increased creative drive sometimes associated with frontotemporal dementia is related to damage in the left anterior temporal lobe. Intelligence is also associated with integrity of white-matter tracts including the arcuate fasciculus and corpus callosum. Divergent thinking and openness to experience are associated with lower measures of integrity within white-matter tracts linking the thalamus with frontal projection zones. Although there is some overlap, intelligence and creativity appear to involve largely different brain networks. The intelligence findings suggest the importance of network integrity that may facilitate knowledge acquisition and retention. The creativity findings suggest a disinhibition of networks that facilitates the generation of novel associations among knowledge stores. Whether there is a specific network for genius is not yet apparent. Complex phenomena like intelligence, creativity, and genius can be studied scientifically with modern neuroscience methods even as their definitions evolve with better empirical observations.

This chapter presents two cases of individuals with frontotemporal dementia: a 60-year-old man arrested for child molestation whose personality had changed remarkably over four years, and a woman in her fifties who demonstrated progressive changes in her personality over the course of 18 months. These neurological patients demonstrate many of the characteristics of psychopathic individuals, including pervasive immoral behavior and reduced concern for the harm that this behavior may have on others. In relation to these cases, the chapter explains how neuropsychology has been able to provide information about the brain regions that likely function differently in psychopathy in two ways. Specifically, neuropsychology compares the behaviors and personality features of patients with damage to specific brain regions to those of people with psychopathic traits.

Memory is one of the most important cognitive functions in a person’s life. Memory is essential for recalling personal memories and for performing many everyday tasks, such as reading, playing music, returning home, and planning future actions, and, more generally, memory is crucial for interacting with the world. Determining how humans encode, store, and retrieve memories has a long scientific history, beginning with the classical research by Ebbinghaus in the late 20th century (Ebbinghaus, 1964). Since this seminal work, the large number of papers published in the domain of memory testifies that understanding memory is one of the most important challenges in cognitive neurosciences. With population growth and population aging, understanding memory failures both in the healthy elderly and in neurological and psychiatric conditions is a major societal issue. A substantial body of evidence, mainly from double dissociations observed in neuropsychological patients, has led researchers to consider memory not as a unique entity but as comprising several forms with distinct neuroanatomical substrates (Squire, 2004). With reference to long-term memory, episodic memory may be described as the conscious recollection of personal events combined with their phenomenological and spatiotemporal encoding contexts, such as recollecting one’s wedding day with all the contextual details (Tulving, 2002). Episodic memory is typically opposed to semantic memory, which is viewed as a system dedicated to the storage of facts and general decontextualized knowledge (e.g., Paris is the capital of France), including also the mental lexicon. Episodic memory was initially defined by Tulving as a memory system specialized in storing specific experiences in terms of what happened and where and when it happened (Tulving, 1972). Later, phenomenological processes were associated with the retrieval of memories (Tulving, 2002). Episodic memory is assumed to depend on the self, and involves mental time travel and a sense of reliving the original encoding context that includes autonoetic awareness (i.e., the awareness that this experience happened to oneself, is not happening now, and is part of one’s personal history).

Empirical research apparently suggests that emotions play an integral role in moral judgment. The evidence for sentimentalism is diverse, but it is rather weak and has generally been overblown. There is no evidence that our moral concepts themselves are partly composed of or necessarily dependent on emotions. While the moral/conventional distinction may partly characterize the essence of moral judgment, moral norms needn’t be backed by affect in order to transcend convention. Priming people with incidental emotions like disgust doesn’t make them moralize actions. Finally, moral judgment can only be somewhat impaired by damage to areas of the brain that are generally associated with emotional processing (as in acquired sociopathy and frontotemporal dementia). While psychopaths exhibit both emotional and rational deficits, the latter alone can explain any minor defects in moral cognition.