Dementia is a clinical syndrome characterized by the impairment of multiple cognitive domains that is severe enough to interfere with one’s usual social and occupational functioning. The impairment must represent a decline from a previously higher level of functioning and not occur exclusively during the course of delirium. Knowledge of the functional link between neuroanatomy and neurophysiology of neurodegenerative processes and neuropsychological outcomes is critical for clinical practice. This chapter describes the neuropsychological, neuroanatomical, and neurophysiological features of several of the more common “cortical dementias”. It reviews Alzheimer’s disease (AD), the most common form of dementia, and then compares and contrasts the features of AD with those of other disorders that involve significant cortical pathology including dementia with Lewy bodies (DLB), frontotem-poral dementia (FTD), and cortical vascular dementia (VaD). The chapter provides shorthand descriptions of the typical disease onset, course, neuropsychology, neuroimaging, and neuropathology associated with each cortical dementia syndrome.

This chapter provides a brief guide to the main brain imaging techniques from the point of view of their usefulness in differentiating the principal forms of cortical dementias, that is, Alzheimer’s disease (AD), posterior cortical atrophy (PCA), frontotemporal lobar degeneration (FTLD), and dementia with Lewy bodies (DLB), which have relatively specific imaging findings. It considers mild cognitive impairment (MCI) because it is a clinical condition that is often prodromal to cortical dementias, in particular AD. The chapter discusses in detail other dementias associated with movement disorders, that is, the corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP), because they belong to the FTLD spectrum, and vascular dementia (VaD), which is frequently considered in differential diagnosis of cortical dementias. It also offers a quick look at future directions in neuroimaging, which will most likely be based on the combination of different techniques of structural, molecular, and functional imaging.

With the elderly population rapidly increasing worldwide, neuropsychologists are often called upon to conduct assessments of possible neurodegenerative disorders. This chapter provides an overview of the differential diagnostics via neuropsychological methods of cortical dementia syndromes. Over the last several decades, clinicians have seen significant changes with regard to daily practice in interprofessional settings, which are becoming more commonplace for practicing neuropsychologists. The chapter provides guidance/input/assistance for practitioners working in such settings. Mini-Mental State Examination (MMSE) is the most commonly administered psychometric screening assessment of global cognitive functioning. The Clinical Dementia Rating scale (CDR) is a widely used rating scale for measuring dementia severity. Alzheimer’s disease (AD) is characteristic of a progressive decline in memory, executive functioning, visuospatial abilities, language, and behaviors as a result of neurodegeneration in the brain. Progressive supranuclear palsy (PSP) is a neurodegenerative akinetic rigid disorder.

Alzheimer’s disease (AD) presents one of the most urgent health care issues of our time. AD is a disease of the brain and mind, and as such, neuropsychology has an essential and evolving role to play in addressing this growing public health concern. Measurement of key cognitive functions, such as delayed recall of recently presented information, is crucial in the diagnosis and monitoring of the disease. In addition to the importance of advancing scientifically informed disease-specific measurement of cognition, neuropsychology has a growing role to play in the design and implementation of nonpharmacological interventions for AD. The neuropathological hallmarks of AD are senile plaques (SP), neurofibrillary tangles (NFTs), and cell and synapse loss in multiple brain areas. Granulovacuolar degeneration (GVD) has long been recognized to be present in the brains of AD patients.

Vascular dementia (VaD) is an umbrella term representing a clinical grouping with inherent heterogeneity in its clinical manifestations reflecting a variability in its underlying etiology. This chapter discusses specific presentations that can fall under the VaD heading. It includes discussion of multi-infarct dementia (MID) and dementia associated with lacunar states (LSs), as well as Binswanger’s disease (BD), which remains embroiled in controversy. The chapter discusses cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and moyomoya disease due to their clinical overlap. The etiology of MID is in many ways the same as the etiology of cerebrovascular disease (CVD) in general and even late-life dementia. The term MID itself is used to describe a disorder characterized by a stepwise deterioration of cognitive functioning associated with strokes or accumulated transient ischemic attacks (TIAs).

This chapter suggests that the dysexecutive syndrome associated with vascular dementia (VaD) is caused by impairment in separate but related cognitive concepts; that is, pathological inertia, mental bradyphrenia, disengagement, and temporal reordering. During the late 19th and early 20th centuries, cerebrovascular dementia was a well-established clinical syndrome. Multi-infarct dementia (MID) generally became associated with all types of vascular syndromes. Recent research suggests the presence of considerable overlap between the neuropathology underlying Alzheimer’s disease (AD) and VaD. Patients diagnosed with VaD tend to produce hyperkinetic/interminable perseverations, suggesting an inability to appropriately terminate a motor response. Other aspects of the dysexecutive syndrome associated with VaD revolve around constructs related to interference inhibition, flexibility of response selection, and sustained attention. From the view point of diagnosis, the neuropathology of VaD often differentially impacts the frontal lobes, whereas the neuropathology associated with AD revolves more around circumscribed temporal lobe involvement.

Dementia with Lewy bodies (DLB) is a clinical syndrome characterized by progressive dementia, cognitive fluctuations, visual hallucinations (VH), and parkinsonism. In 1961, Okazaki, Lipkin, and Aronson reported two patients with dementia and parkinsonism with cortical neuronal inclusions similar to the brain-stem Lewy bodies (LB) seen in Parkinson’s disease (PD). LBs are intra-cytoplasmic neuronal inclusions containing α-synuclein and ubiquitin. There are other associated pathological features in DLB such as spongiform change neuronal loss, and Alzheimer’s disease (AD) pathology includes amyloid plaques and neurofibrillary tangles (NFTs). DLB and other entities such as PD and multiple system atrophy (MSA) have been grouped under the term synucleinopathies due to the existence of &#945-synuclein inclusions in the brain. The central feature required for a diagnosis of DLB is the presence of dementia: a progressive cognitive decline of sufficient magnitude to interfere with normal social or occupational function.

Chronic alcohol use has been related to various linked disorders when used in excess, particularly when this excessive use becomes chronic. It is important for clinicians to clarify the amount and type of alcohol being consumed and the frequency of this consumption when considering its potential role in any neuropsychological profile. The most commonly reported terms found in the literature include alcohol-induced persisting dementia (APA), alcohol-related dementia, and Korsakoff’s syndrome (KS). This chapter provides some synthesis of this literature to offer some clarity on cognitive dysfunction as it relates to alcohol and the manifestation of dementia as a result of chronic use, including discussion of the classic KS and related presentations. Alcohol dependency is commonly associated with a number of neurological impairments including deficits in abstract problem solving, visuospatial and verbal learning, memory function, perceptual-motor skills, and even motor function.

Dementia is an umbrella term for conditions such as Alzheimer’s disease (AD), dementia with Lewy bodies (DLB), vascular dementia (VaD), and frontotemporal dementia (FTD). Under that umbrella, FTD, also known as frontotemporal lobar degeneration (FTLD), can be further categorized to define a group of neurodegenerative disorders resulting from a progressive deterioration of the cells in the anterior temporal and/or frontal lobes of the brain. More specifically, ventromedial-frontopolar cortex is identified with metabolic impairment in FTD. This chapter elaborates on the history, epidemiology, pathophysiology, clinical features, treatment, and outcomes of FTD. The history and background section of each of the FTD categories highlights the evolution of the disease conceptualization. The FTD subtypes are conceptualized in three categories: neurobehavioral variant, motor variant, and language variant. The chapter illustrates the features of all three categories of FTD.