Frontotemporal Dementia

I highly recommend Bruce Miller’s extraordinary book, Frontotemporal Dementia, to all psychiatrists for two reasons: because it is a fun, interesting, and informative read and because it is important. Unlike most medical books, which are a compilation of chapters contributed by different authors, this book is written entirely by Dr. Miller, a behavioral neurologist who has done more than anyone to treat, study, and bring this fascinating disorder to international attention. Having a single author, the book has a cohesiveness and continuity that many multiauthor medical books lack. With his extensive expertise on the topic, the book is authoritative and current. One of my favorite things about this book is how Dr. Miller’s clinical wisdom and expertise complement the discussions of research findings. There are many important issues in the field with a dearth of data, for example, the topic of slowly progressive frontotemporal dementia, and Dr. Miller supplements his discussion of the limited research on these topics with his own extensive clinical experience.

The book is an engaging and well-written overview of frontotemporal dementia and related disorders. It has something to offer for clinicians and researchers at any level, from beginner to experts on this topic. Readers will come away with a broad base of knowledge about the disorders that make up the frontotemporal dementia spectrum. Dr. Miller skillfully integrates important recent research findings with the background knowledge about these disorders to give a sense of where the field is going and why those with these disorders are such an interesting group of patients to care for and study. He covers frontotemporal dementia and related disorders, including language variants and the variants that present with prominent motor symptoms such as corticobasal syndrome, progressive supranuclear palsy, and frontotemporal dementia-amyotrophic lateral sclerosis, from the genetics and neuropathological findings to the imaging findings and clinical presentation.

Sections of particular interest to psychiatrists describe the insights to be gained from these patients on how emotion, empathy, the self, and social cognition are represented in the brain and what happens when these functions are disrupted. The frontal and anterior temporal lobes, more than any other part of the brain, make us “human,” and the dysfunction of these systems can lead to fascinating symptoms. Dr. Miller describes a series of his patients with frontotemporal dementia with right frontal degeneration who had dramatic alterations of self, including changes in political views and religion, and patients with right anterior temporal damage who become rigid, unbending, and lost their empathy for others. He describes creative experiments, such as one in which frontotemporal dementia patients watched a videotape of themselves singing the song “My Girl” but did not show the signs of embarrassment that healthy people show when doing this. He also reviews genetic findings with important implications for psychiatric disorders, including the recent evidence that mutations in the C9ORF72 gene frequently initially present with psychiatric syndromes including psychosis and personality disorders.

I have made the case that Frontotemporal Dementia is an entertaining and informative book to read. But why is it particularly important for psychiatrists to read? Psychiatry should be especially concerned with disorders such as frontotemporal dementia that affect frontal and limbic functions, including emotion, social cognition, and behavior regulation. To date, the field of psychiatry has shown little interest in frontotemporal dementia and other neurodegenerative disorders that affect these brain regions. This creates a fundamental contradiction: We wish psychiatry to be taken seriously as a brain-based medical specialty, but we exclude patients with brain disorders that manifest with psychiatric symptoms such as frontotemporal dementia. Patients with frontotemporal dementia usually present to mental health settings and are commonly misdiagnosed with psychiatric disorders. The study of patients with frontotemporal dementia can provide unique insights into the neuroanatomy and genetics of psychiatric disorders. Having more psychiatrists read Dr. Miller’s excellent book will not fix the problem of integrating the study of frontal lobe disorders into psychiatry, but it would be a good start.