Neuropsychologists working with dementia patients can readily access many resources dedicated to Alzheimer's Disease (AD). Harder to find is a book devoted exclusively to frontotemporal dementia (FTD), particularly a recent publication. Since the consensus paper on the diagnostic criteria of the FTDs (Neary et al., Reference Neary, Snowden, Gustafson, Passant, Stuss, Black, Freedman, Kertesz, Robert, Albert, Boone, Miller, Cummings and Benson1998), there has been an upsurge in research into this disease and its related syndromes. Many advances have been made in the areas of neuroimaging, neuropathology, and genetics, as well as neuropsychology. Frontotemporal Dementia Syndromes, edited by John R. Hodges, offers a much-needed review of the current status of the field. The authors are researchers and clinicians at the forefront of the discipline, and all have collaborated at one time at the specialist FTD clinic at Addenbrooke's Hospital in Cambridge, UK. However, the text offers a thorough survey of the broader literature and is targeted to both clinical and scientific audiences.
Chapter 1 begins with an overview of frontotemporal dementia, including the evolution of the terms for this spectrum of dementing disorders. According to Hodges, Arnold Pick in 1892 described patients with progressive language difficulty, all with marked unilateral temporal atrophy. Later, he focused on patients with prominent behavioral and personality changes as a result of bilateral frontal pathology, who were discovered to have argyrophilic intracytoplasmic inclusions (Pick bodies) histologically. In the 1920s, the term “Pick's disease” centered on these frontal lobe changes whilst neglecting temporal lobe degeneration. By the 1980s, cases of frontal lobe atrophy without Pick bodies came to be known as “frontal degeneration of the non-Alzheimer type.” This preceded the Neary et al. (Reference Neary, Snowden, Gustafson, Passant, Stuss, Black, Freedman, Kertesz, Robert, Albert, Boone, Miller, Cummings and Benson1998) paper, which divided frontotemporal lobar degeneration (FTLD) into FTD, the primarily behavioral variant, and two aphasic variants: progressive non-fluent aphasia (PNFA) and semantic dementia (SD). In this chapter, Hodges further describes the subforms of FTLD used in Cambridge, introducing the term “frontal/behavioral variant of FTD” (bv-FTD) in addition to PNFA and SD. He also discusses how FTD (or FTLD) is unique amongst dementias in its selective involvement of brain areas involved in social cognition, language, and semantic memory.
Graham, the author of Chapter 2, addresses the epidemiology of FTD. While FTD is reportedly much less common than AD, its incidence depends on whether you are looking at a community based sample (which has a base rate of approximately 5%) or a clinic based sample (with a more biased 15–30% incidence rate). He also describes the age of onset, which is earlier than for AD, and prognosis.
The next three chapters are probably the most interesting and helpful to those neuropsychologists who assess dementia patients and routinely make differential diagnoses. In Chapter 3, Kipps, Knibb, and Hodges describe the clinical presentations of FTD, including behavioral, psychiatric and language manifestations, and suggest behavioral rating scales and cognitive screens. Since neuropsychological assessments may be normal in bv-FTD, behavioral rating scales become paramount. Case examples from the contributors' clinical experiences highlight the presentations. This chapter also outlines useful diagnostic criteria to differentiate FTD from AD and other neurodegenerative disorders (including dementia with Lewy Bodies (DLB), vascular dementia, and some of the “overlap” syndromes discussed in more detail in the next chapter). Overlap syndromes involve a mixture of symptoms of two different diseases in one patient. Chapter 4, by Bak, reviews the overlap syndromes with FTD, including motor neuron disease (MND), corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), Parkinson's linked to chromosome 17, and amyotropic lateral sclerosis (ALS). Again, case examples illustrate the diagnoses. The neuropsychology of FTD is discussed in Chapter 5 by Hodges and Patterson. The cognitive profiles of bv-FTD, PNFA, and SD are covered, including dysexecutive, memory and language impairments, as well as less commonly assessed areas such as decision-making, social cognition, emotional processing, and empathy.
Chapters 6, 7, 8, and 9 likely represent the areas of greatest research progress. In Chapter 6, Nestor writes about the neuroimaging (MRI, PET, SPECT, and EEG) of the three clinical subtypes of FTD. The majority of the references cited here are less than ten years old. Comparative color figures clearly demonstrate structural and functional changes in FTD. Davies and Xuereb, in Chapter 7, cover histopathology. They elucidate how the pathological subtypes differ from the clinical entities, and are divided by the presence or absence of tau abnormalities (taupathology). Chapter 8, by Gasparini and Spillantini, is a more technically challenging chapter on molecular neuropathology, and Brown, in Chapter 9, summarizes the genetics of FTD. The latter focuses on the role of chromosome 17 in FTD, although concluding that FTD is likely polygenic. Brown further states that genetic factors appear important in FTD, probably more so than in AD.
Readers craving information on interventions with FTD patients and their families will find it hard to wait until Chapter 10 (Lough and Garfoot). This last chapter addresses psychological interventions. In it, the authors suggest that the psychologist's role is threefold: diagnosis, behavior management, and assessment of the needs of caregivers and families. Diagnosis is also covered in earlier chapters but, in Chapter 10, practical suggestions for behavior management and work with caregivers are offered through detailed case studies. The techniques used vary in many ways from work done with AD patients and their families. Behavior management, for example, can rely on environmental dependency, cognitive behavioral therapy (CBT), or techniques based on a model of social self-awareness and self-regulation. Family work focuses on education about FTD as well as reducing the caregiver's stress; each family is very different. The emphasis throughout is on a multidisciplinary approach, although noticeably absent is any discussion on pharmacological treatments, if only to augment psychological interventions. Perhaps this could have been addressed in a separate chapter. Lastly, even the book's appendices are thoughtful, and include the Cambridge Behavioral Inventory, resources for caregivers (admittedly, mainly for patients with AD), and website addresses. Unfortunately, the latter are specific to the U.K.
In conclusion, Frontotemporal Dementia Syndromes is a comprehensive resource for neuropsychologists, particularly those specializing in dementia, and would be a useful reference text in their libraries. This rarer form of dementia has experienced many definitional changes and research strides in the past decade, but a review of the literature was sorely needed. This edited text brings together many of the authoritative leaders in the field of FTD, is well-written, well organized, and very accessible with numerous tables, illustrations, figures, and case studies. Its chapter on psychological interventions in FTD may be a first, and leaves us wanting more. Both clinically and scientifically, it is a recommended and welcome addition.
