Non-Alzheimerʼs and Atypical Dementia

This deceptively slim volume, looking more like an atlas than a textbook, is actually a thorough, carefully organized, and well-referenced text on atypical dementias. Unlike most multiauthor medical textbooks, this one is divided into discrete chapters on individual syndromes, with overlap only in the general introductory chapter, a chapter on multidisciplinary evaluation, and a concluding chapter on management of patients with all types of atypical dementias. I would recommend the book both as useful background reading and as a ready reference when a neurologist, psychiatrist, or internist encounters a patient with any of the atypical dementias. The introductory chapter outlines the variety of diagnoses that will be discussed later and briefly introduces each disorder. The chapter on multidisciplinary evaluation is a detailed clinical roadmap. Authors Geschwind and Belkoura start with what to look for in the patient history: early symptoms, memory function, behavioral and neuropsychiatric symptoms, speech and language, visuospatial function, executive function, the motor system, and activities of daily living. The history continues with the review of systems, past medical and surgical history, medications, and family and social history. Then the authors cover the neurologic examination, neuropsychological testing, laboratory testing, and neuroimaging. This chapter is literally a “how to” for the approach to the cognitively impaired patient. Each chapter on a specific disorder or set of disorders begins with a case example, followed by a discussion with useful figures and tables, and ends with a complete reference list. Almost all of these chapters are excellent, although I found a few minor omissions in some of them. For example, Chapter 3, on atypical Alzheimer disease, opens with a mention of posterior cortical atrophy and primary progressive aphasia, but omits “frontal variant” Alzheimer disease until much later, in Case 4. Chapter 4, on vascular cognitive impairment, is an excellent summary. I would have liked a general definition at the beginning, rather than a table with multiple sets of criteria for diagnosis. This is ironic since the first author, Helena Chui, wrote the very useful California criteria for diagnosis of “ischemic vascular dementia” (Chui et al, 1992). Chapter 5 is an excellent discussion of frontotemporal dementia, well coordinated with the chapter on atypical Alzheimer disease. The section on treatment is deservedly pessimistic, although I would point out that the memantine trials cited in the book were quite small, and clinical experience has shown that some patients seem to benefit from memantine and/or cholinesterase inhibitors. Chapter 6, on Lewy body dementia, states that this is the second most common cause of dementia. Chapter 3 notes that vascular pathology is the second most common cause of cognitive impairment in the elderly and points out that vascular disease interacts with late-life neurodegenerative pathology to produce cognitive impairment. Perhaps the best way to eliminate this apparent contradiction (rare in this book) would be to say that Lewy body dementia is the second most common neurodegenerative cause of dementia. Chapter 7 offers useful summaries of progressive supranuclear palsy and corticobasal degeneration, both tauopathies. I would have liked to see the “look of perpetual astonishment” or the “hummingbird sign” on magnetic resonance imaging mentioned with regard to progressive supranuclear palsy. Chapter 8, on repeat expansion diseases and dementia, is an excellent summary not only of Huntington disease, but also of the less common syndromes of spinocerebellar ataxia 17 (SCA17) and fragile X-associated tremor/ataxia syndrome (FXTAS). Chapter 9 is a brief but complete summary of the prion-related dementias by two international experts on these syndromes, Leonel Takada and Dr Geschwind. My only complaint is that the “alphabet soup” of abbreviations for the subsyndromes made reading somewhat hard going. Chapters 10 through 12 deal with autoimmune dementias, toxic and metabolic dementias, and leukoencephalopathies and leukodystrophies. Although the summaries are very brief, I found these to be useful chapters on topics not usually included in books on dementia. I would have liked to see more discussion of Hashimoto encephalopathy in either the autoimmune or the metabolic dementia chapter. Chapter 13, on infectious causes of dementia, and Chapter 14, on rheumatologic dementias, also cover syndromes not usually explored in textbooks on cognitive impairment or dementia. The final chapter, 15, covers overall management of the patient with an atypical dementia, including, among many concerns, behavioral and pharmacologic management, safety, and end-of-life issues. The discussions are somewhat individualized for the specific disorders. Overall, the book is a very useful volume to have on hand when questions arise about a patient with cognitive issues, as well as a book to read from cover to cover. As I noted at the outset, the book is exceptionally cohesive, with all chapters similarly outlined and topics skillfully organized, thus minimizing repetition. I have also found several of Dr Geschwind’s articles about atypical and rapidly progressive dementing illnesses to be very useful, especially proposing the mnemonic VITAMINS to outline the causes of dementias The reviewer declares no conflicts of interest. BOOK REVIEW