Frontotemporal Dementia

Overview

Frontotemporal Dementia provides an in-depth look at the history, various types, genetics, neuropathology and psychosocial aspects of one of the most common but least understood causes of dementia, frontotemporal lobar degeneration, from one of the world's leading centers for the study of dementia. Aided by the latest research in diagnosis, mechanism and treatment, this book captures the rich and quickly changing landscape of a devastating neurodegenerative disease, and offers up-to-date clinical advice for ...

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Overview

Frontotemporal Dementia provides an in-depth look at the history, various types, genetics, neuropathology and psychosocial aspects of one of the most common but least understood causes of dementia, frontotemporal lobar degeneration, from one of the world's leading centers for the study of dementia. Aided by the latest research in diagnosis, mechanism and treatment, this book captures the rich and quickly changing landscape of a devastating neurodegenerative disease, and offers up-to-date clinical advice for patient care. Frontotemporal dementia, in particular, raises psychological and philosophical questions about the nature of self, free will, emotion, art and behavior - important topics for practitioners and families to appreciate as they care for the sufferer. This book includes case studies, photographs and figures from the leaders in the field and personal communication from the researchers driving these developments.

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Editorial Reviews

From the Publisher

Frontotemporal Dementia by Bruce Miller is a remarkable book. Presenting the exciting science of this rapidly progressive field, the book is comprehensive, up-to-date, and clinically relevant. While introducing the latest advances in genetics, imaging, and neuropathology, Frontotemporal Dementia captures the author's important perspective on the philosophical issues raised by these unique disorders regarding how they impact our understanding of our sense of self and our role in the human community. This milestone volume is the definitive reference on frontotemporal dementias.
-- Jeffrey Cummings, MD, Director, Cleveland Clinic Lou Ruvo Center for Brain Health, Las Vegas, NV

"This is an exciting and authoritative book on frontotemporal dementia (FTD) that spans the biological and clinical features of a fascinating disease that sits directly in the intersection between neurology, psychiatry and neuroscience. It elucidates key principles around the biological basis for abnormal behavior. The superbly written book is a comprehensive guide for the physicians, psychologists, neuroscientists, and others who work with FTD and is very highly recommended."
-- Dilip V. Jeste, M.D., President, American Psychiatric Association

Frontotemporal lobar degenerations are being recognized with increasing frequency by the practicing neurologist. They are also offering unique research opportunities for exploring the molecular foundations of selective vulnerability and the neurobiology of cognitive networks. In this lavishly illustrated monograph, Bruce Miller integrates the history, clinical manifestations, cognitive patterns, neuropathology, genetics and even philosophical implications of FTD into a comprehensive account that is as readable as it is authoritative. This volume offers the single most coherent account of the topic that I have yet encountered.
-- M-Marsel Mesulam, MD, Ruth and Evelyn Dunbar Professor of Neurology, Psychiatry and Psychology, Director, The Cognitive Neurology and Alzheimer's Disease Center (CNADC), Feinberg School of Medicine, Northwestern University, Chicago, IL

"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 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." --Biz India

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Product Details

  • ISBN-13: 9780195380491
  • Publisher: Oxford University Press
  • Publication date: 9/3/2013
  • Series: Contemporary Neurology Series, #85
  • Pages: 200
  • Sales rank: 1,503,173
  • Product dimensions: 6.90 (w) x 10.00 (h) x 0.80 (d)

Meet the Author

Bruce L. Miller, MD is Professor of Neurology at the University of California, San Francisco (UCSF) where he holds the A.W. & Mary Margaret Clausen Distinguished Chair, teaches extensively and directs the busy UCSF dementia center. He is a behavioral neurologist focused in dementia with special interests in brain and behavior relationships as well as the genetic and molecular underpinnings of disease.

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Table of Contents

Table of Contents

1. History and Nomenclature

Pick's Disease: The Contributions of Arnold Pick

Maintaining Early Traditions and Refining Diagnosis: Delay, Brion, Escourolle, Malamud and Constantinidis

Splintering of Pick's Disease into Multiple Subtypes

Corticobasal Degeneration (CBD) - Rebeiz, Gibbs, Riley & Lang

Frontal Lobar Dementia of the Non-Alzheimer Type, Dementia of the Frontal Type, Frontotemporal Dementia & Frontotemporal Lobar Degeneration: Brun Gustafson, Neary, Bowen, Snowden, Mann

Primary Progressive Aphasia (PPA): Marsel Mesulam, Norman Geschwind, John Hodges, Julie Snowden, Karalyn Patterson and Marilu Gorno-Tempini

UCLA and UCSF Studies

The Biological Revolution

Genetics

Neuropathology

FTD Now: Where Are We?

The Future

References

2. The Clinical Syndrome of bvFTD

Overview

Case History

Comment

Clinical Features and Anatomy

Genetic and Pathological Correlates

Differential Diagnosis

Alzheimer's Disease

Psychiatric Disorders

Movement Disorders

Chronic Traumatic Encephalopathy

Other FTD Clinical Phenotypes

Vascular Dementia

Metabolic Causes

Structural Brain Abnormalities

Treatment

Medications used to alleviate bvFTD symptoms

bvFTD: Current State and Research Advances

References

3. The Temporal Variant of FTD

Overview

Case History

Comment

Clinical Features and Anatomy

Right Temporal Lobe Syndrome

Creativity

Genetic and Pathological Correlates

Differential Diagnosis

Treatment

svPPA: Current State and Research Advances

Semantic Knowledge and the Anterior Temporal Lobes

Autoimmunity, Left-Handedness, Head Trauma and svPPA

Summary

References

4. Progressive Nonfluent/Agrammatic Aphasia

Overview

Case History

Comment

Clinical Features and Anatomy

Genetic and Pathological Correlates

Differential Diagnosis

Treatment

nfvPPA: Current State and Research Advances

Diagnosis from Clinical Syndrome to Molecular Diagnosis

Neurolinguistic and Neuroplasticity Discoveries

References

5. Related Disorders: FTD-ALS

Overview

Amyotrophic Lateral Sclerosis

Case History

Comment

Clinical Features and Anatomy

El Escorial Criteria

Genetic and Pathological Correlates

Neuropathology

Differential Diagnosis

Treatment

FTD-ALS: Current State and Research Advances

Clinical-Pathological Considerations

Molecular Considerations

References

6. Related Disorders: Corticobasal Degeneration and Progressive Supranuclear Palsy

Overview

Corticobasal Syndrome (CBS) and Corticobasal Degeneration (CBD)

Case History

Clinical Features and Anatomy

Genetic and Pathological Correlates

Differential Diagnosis

Treatment

Progressive Supranuclear Palsy (PSP)

Case History

Clinical Features and Anatomy

Genetic and Pathological Correlates

Differential Diagnosis

Treatment

CBD and PSP: Current State and Research Advances

Clinical Dilemmas

New Discoveries on the Pathogenesis of CBD, PSP and other Tauopathies

Neuroimaging

Basic Biological Advances

Clinical Trials for Tauopathies

References

7. A Primer of FTLD Neuropathology

General Features

Overview of Tau-Related Neuropathology in FTLD

Overview of TDP-43 and FUS Related Neuropathology in FTLD

Gross Anatomical and Microscopic Changes in FTLD

Tau Aggregates

Pick's Disease

PSP and CBD

Argyrophilic Grain Disease

FTLD with Ubiquitin- and TDP-43-positive Inclusions

FTLD with Tau and TDP-43-Negative but FUS-Positive Inclusions

SQSTM1 and FTLD

Dementia Lacking Distinctive Histopathology (DLDH)

Special Features of FTLD Pathology

Hippocampal Sclerosis

Von Economo Neurons

FTLD Neuropathology: Current State and Research Advances

What is the Role of TDP-43 in Non-FTLD Degenerative Conditions: Alzheimer's Disease and Chronic Traumatic Encephalopathy?

Basic Mechanisms in TDP-43, FUS and C9ORF72

Does FTLD Spread in a Prion-like Fashion?

FTD Pathology Glossary

References

8. FTD Genes

Overview

Genes Implicated in FTD

MAPT

GRN

C9ORF72

Valosin-containing-Protein ATPase (VCP)

TARDBP

FUS

CHMP2B

SQSTM1 and Ubiquilin 2

Genes That Modify FTD Expression

Management and Genetic Counseling

References

9. FTD Reflections Upon Psychology and Philosophy

Emotions

The Self

Free Will

Religious Beliefs

Psychology

Social and Emotional Functioning

Executive Control

von Economo Neurons

References

10. Treatments

Diagnosis

bvFTD: Targeting Symptoms, Educating the Family

svPPA: Targeting Symptoms, Educating the Family

nfvPPA: Targeting Symptoms and Educating the Family

Late Stages of FTD

Early Interventions

Pharmacological Review

Selective Serotonin Reuptake Inhibitors (SSRIs)

Atypical Antipsychotics

Acetylcholinesterase inhibitors (AChEIs)

NMDA-Receptor Antagonist

Dextromethorphan Hydrobromide and Quinidine Sulfate for Pseudobulbar Affect

Valproic Acid

Gabapentin

Oxytocin

Non-Pharmacological Therapies

Complementary and Alternative Medicine

Language Impairment

Future Treatments

Tau

Progranulin

TDP-43, FUS, C9ORF72

Conclusions

References

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