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In 1885, the French neurologist Georges Gilles de la Tourette, describing the first nine patients with Gilles de la Tourette syndrome, stated, "Everything is extraordinary in this disease: the name is ridiculous, its symptoms peculiar, its character equivocal, its cause unknown, its treatment problematical." More than a century later, this statement still holds, although the name of the disease has been changed to Tourette's syndrome (or Tourette's disorder). Considered rare and exotic at that time, Tourette's syndrome is a relatively common childhood-onset disorder characterized by multiple motor and vocal tics.
Often lifelong in duration, the full syndrome affects up to 1 person in 2500 and the partial forms two or three times that many. In addition to tics, most patients have other symptoms such as obsessions, compulsions, motor hyperactivity, impulsivity, and distractibility, and many meet the criteria for the diagnosis of obsessive-compulsive disorder or attention-deficit-hyperactivity disorder. A variety of sensory and mental states, including urges and a buildup of tension, precede the tics and subside after the tics occur. Vulnerability to Tourette's syndrome is transmitted within families, although the gene or genes conferring vulnerability have yet to be fully identified. Although much has been learned about the symptoms, character, and course of this fascinating disorder since Gilles de la Tourette's original description, its cause is still unknown and its treatment remains challenging, even with modern pharmacotherapy.
No one is more qualified to contribute a comprehensive textbook on the current understanding of the development of Tourette's syndrome than James Leckman and Donald Cohen of the Yale Child Study Center. With the help of their colleagues, Leckman and Cohen have broadened the scope of their last major book, Tourette's Syndrome and Tic Disorders: Clinical Understanding and Treatment (edited by D.J. Cohen, R.D. Bruun, and J.F. Leckman. New York: Wiley, 1988). That book was considered by clinicians and investigators to be a classic, but an update was needed, since the past 10 years have seen an exponential growth in research on neuroimaging techniques and the neuroscience and genetics of Tourette's syndrome.
This book will appeal to clinicians, investigators, and students and to the families of persons afflicted with Tourette's syndrome. The book is comprehensive in scope; its goal was to review what the authors and their colleagues have learned from their studies and clinical care of patients with Tourette's syndrome and to frame the important questions that remain. A central tenet of the book, derived from the editors' and contributors' developmental and psychoanalytic orientation, is that persons with Tourette's syndrome are whole people whose inner worlds are marked by the struggle to maintain a sense of autonomy and integrity in the face of constant unwanted urges, images, and thoughts -- a siege against the self. This developmental understanding, informed by the authors' research in neurobiology, imaging, neuropsychology, and clinical care, is the foundation of the book's structure.
The book contains three sections. The first, "Individuals, Symptoms, and Diagnoses," reviews the natural history of Tourette's syndrome, the phenomenology of tics and comorbid disorders, the neuropsychology of the disorder, and patients' adaptation to the disease and relationships with their peers. The second section, "Causes and Determinants," presents an evolving model of the pathogenesis of Tourette's syndrome. The authors hypothesize an interaction among neurobiologic substrates, genetic factors, and epigenetic or environmental factors that may confer susceptibility to or protection against Tourette's syndrome and related obsessive-compulsive disorders. They offer the concept that Tourette's syndrome and obsessive-compulsive disorder are the result of heightened but selective sensitivity to alterations in the internal milieu of the body and the external environment. This sensitivity, they argue, results from an escape from the inhibitory pathways in the brain, which link deep basal ganglionic structures with specific cortical areas. In Tourette's syndrome this enhanced sensitivity causes the exaggerated, uncoordinated expression of fragments of movement, cognition, and behavior that belong to the repertoire of ordinary, voluntary action in normal people.
Also included in section 2 are reviews of the epidemiology, genetics, neuroanatomical circuitry, and neurochemistry of Tourette's syndrome. Section 3, "Partnerships for Making the Best of Tourette's," focuses on the Yale approach to evaluation and treatment and includes chapters on rating instruments, testing, and pharmacologic and psychosocial treatment. A final chapter reviews the role of voluntary organizations in clinical care, research, and public policy.
This outstanding book is a model of comprehensive care for clinicians. Its strengths include the authors' successful integration of basic principles and approaches to clinical care from neurobiologic, developmental, and psychodynamic perspectives. The discussion of adaptive aspects of Tourette's syndrome and the emphasis on evaluation of the strengths and resilience of individual patients are also important features. Although treatment options are reviewed thoroughly, in part in separate chapters on pharmacotherapy and psychosocial therapy, the book would have been strengthened by a discussion of treatment recommendations based on the spectrum of Tourette's syndrome and obsessive-compulsive disorders.
Reviewed by Barbara J. Coffey, M.D.
Copyright © 1999 Massachusetts Medical Society. All rights reserved. The New England Journal of Medicine is a registered trademark of the MMS.
Review
"...an excellent guide..." (Ageing & Society, Vol 20, 2000)
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