Author: Bruce L. Miller, MD. Contemporary Neurology Series Editor: Sid Gilman, MD, FRCP
Publisher: Oxford University Press – 180 pages
Book Review by: Nano Khilnani

Frontotemporal Dementia (commonly referred to as FTD) was initially called Pick’s Disease, and was also associated in some ways with Alzheimer’s disease.

Dr. Bruce Miller writes at the outset about his initial interest in studying FTD in 1983 while working with two mentors: D. Frank Benson and Jeffrey Cummings. He writes that they taught him how to evaluate patients with dementia, and how to diagnose and separate those who had Alzheimer’s disease from those who had FTD.

They advised Dr. Miller: “don’t pick Pick’s Disease,” which meant that this is a rare condition and almost impossible to diagnose.

The author eventually learned that frontotemporal dementia is a “unique biological condition that offered an opportunity to understand how slow, progressive atrophy of the frontal lobes would subtly influence the personality, behavior and cognitive abilities of a previously healthy individual.”

Having realized that the development this disease is slow, but progressively gets worse, he decided to take what he calls a “bionarrative” approach. He began to carefully document a patient’s life story; his or her strengths, weaknesses, and eccentricities. He wanted to learn how the disease began, and how it changed his life’s trajectory. The course of the disease was often long and complex, he discovered, and that made him reconsider his assumptions about social behavior, language, and creativity.

“In some patients, the disorder began with genius in business, athletics, or the arts. Even once the illness was well-established, visual creativity could be maintained, particularly in patients with asymmetric left-sided disease. The experiences that I had working with my remarkable patients and their families are the inspiration for this book,” he writes.

For some 15 years from 1983 to 1998, Dr. Miller writes that “the clinical methodology for understanding FTD was sadly deficient.” But in 1998, “Michael Hutton and colleagues reported the exon and intron mutations in the tau gene were responsible for many familial forms of FTD,” Miller reports, and “suddenly, the rich biology of tau already studied intensely by neurologists interested in Alzheimer’s disease, was directly linked to a neurodegenerative condition,” he exudes.

Four new discoveries in the second of 2006generated further hope and excitement for researchers and patients. Since then, much more has been learned that has helped patients with FTD.

This book will be helpful to neurologists, neuropsychologists, neuropsychiatrists, those who specialize in geriatrics, and specially those who have patients with frontotemporal dementia.

This book takes you on an exciting historical journey of discoveries about FTD, clinical syndromes of related conditions; diagnoses, genes and treatments. Here is an overview of what you will find in it:

  • History and Nomenclature
  • The Clinical Syndrome of BVFTD
  • The Clinical Syndrome of SVPPA
  • The Clinical Syndrome of NFVPPA
  • Related Disorders: FTD-ALS
  • Related Disorders: Corticobasal Degeneration and Progressive Supranuclear Palsy
  • A Primer on FTLD Neuropathology
  • FTD Genes
  • FTD Reflections on Psychology and Philosophy
  • Treatments

This book authored by Dr. Bruce Miller is a very important source of information on FTD, and includes case studies and Reference lists at the end of chapters on other books and sources of information and research on similar subjects. Its value is enhanced with black-and-white and multicolor brain scans, boxes, charts, tables, and other features.

Bruce L. Miller MD, a behavioral neurologist, is A.W. and Mary Margaret Clausen Distinguished Professor of Neurology; and Director of the UCSF Memory and Aging Center at the University of California, San Francisco.