Hodges' Frontotemporal Dementia by Dickerson, Bradford C. -- Read -- Imperial Library of Trantor

Index

Half title Title page Imprints page Epigraph Contents Contributors Editor biographies Foreword Preface Section 1 Introduction to and brief history of FTD

Chapter 1 Historical introduction to FTD

Introduction What did Arnold Pick actually describe? Rediscovering Pick's disease: from dementia of the frontal type and progressive aphasia to frontotemporal dementia

Progressive aphasia and semantic dementia Frontotemporal dementia and frontotemporal lobar degeneration

Familial chromosome 17-linked frontotemporal dementia and the discovery of unique tau pathology Frontotemporal dementia with motor neuron disease Discovery of the C9orf72 mutation Corticobasal syndrome Conclusions References

Chapter 2 Overview of frontotemporal dementia and its relationship to other neurodegenerative disorders

Introduction to terminology and historical aspects Clinical syndromes of FTD/Pick complex and their assessment

Frontotemporal dementia: behavioral variant (bvFTD) Primary progressive aphasia (PPA) Semantic dementia (svPPA, semantic aphasia) Logopenic variant of PPA (lvPPA) Corticobasal degeneration (CBD) and progressive supranuclear palsy PSP) Motor neuron disease and FTD (FTD-MND)

Neuropathology and molecular genetics

Pathology Genetic relationships

Clinicopathologic correlations in FTD/Pick complex Differential diagnosis

Alzheimer's disease Vascular dementia Prion disease Psychiatric phenocopies

Conclusions References

Section 2 Clinical phenotypes

Chapter 3 Overview of frontotemporal dementia and the variety of its clinical presentations

Introduction Epidemiology Classical features of frontotemporal dementia spectrum syndromes

Behavioral variant FTD (bvFTD) Semantic dementia (SD) Progressive non-fluent aphasia (PNFA)

Atypical presentations and the differential diagnosis of FTD

Differentiating FTD from Alzheimer's disease Differentiating FTD from other neurodegenerative conditions Differentiation from cerebrovascular disease Differentiation from psychiatric conditions

Clinical course Managing FTD

Caregivers Patients The future

Case studies References

Chapter 4 Behavioral variant frontotemporal dementia

Introduction Diagnosis of bvFTD Behavior in bvFTD

Behavioral disinhibition Apathy and inertia Loss of empathy Perseverative, stereotyped, and compulsive behaviors Hyperorality and dietary changes

Measuring behavior in bvFTD Cognition in bvFTD

Executive and generation deficits Relative preservation of visuospatial and constructional abilities Relative preservation of episodic memory

Measuring cognition in bvFTD Emotion, social cognition, and decision-making in bvFTD

Emotion and social cognition Decision-making Experimental tasks in clinical practice

Conclusions Acknowledgment References

Chapter 5 Primary progressive aphasia

Introduction The role of neuropsychological assessment The variants of primary progressive aphasia

Non-fluent/agrammatic variant of primary progressive aphasia Semantic variant of primary progressive aphasia Logopenic/phonologic variant of primary progressive aphasia Atypical and mixed cases of primary progressive aphasia

Genetic studies Treatment and management References

Chapter 6 The FTD-ALS spectrum

FTD-ALS spectrum: the evolution of the concept Profile of cognitive change in FTD-ALS Executive dysfunction in FTD-ALS Language dysfunction in FTD-ALS Social cognition in FTD-ALS Behavior change in FTD-ALS Psychiatric symptoms in FTD-ALS Assessing cognitive and behavior dysfunction in FTD-ALS Brain imaging and cognition in FTD-ALS Pathology of FTD-ALS Genetics of FTD-ALS Conclusions References

Chapter 7 Progressive supranuclear palsy and corticobasal degeneration in the FTD spectrum

Introduction Neuropathology and genetics of PSP and CBD Clinical phenotypes associated with PSP and CBD Brain imaging in PSP and CBD Biomarkers for the diagnosis of PSP and CBD Conclusions References

Section 3 Approach to the diagnosis of FTD

Chapter 8 Overview of clinical assessment of frontotemporal dementia syndromes

Introduction Clinical characteristics of FTLD spectrum diseases

Major FTLD clinical syndromes Other clinical phenotypes

Differential diagnosis of FTLD

Differential diagnosis with diseases traditionally considered “neurologic” Differential diagnosis with diseases traditionally considered “psychiatric”

Diagnostic assessment

History of symptoms The office-based cognitive examination The neurologic examination in suspected FTLD syndromes Neuropsychological testing in FTLD syndromes Neuroimaging, fluid biomarkers, and other diagnostic tests

Clinical course of FTD: the value of longitudinal reassessment of diagnostic classification A recommendation for the diagnostic terminology used in FTLD References

Chapter 9 Neuropsychological assessment of frontotemporal dementia

Behavioral variant FTD

Screening tests Attention Memory Visuospatial function Executive functions

Frontal screening tools Classical executive function tests Social cognition tests

Theory of mind tests

More complex ecologically valid executive function tests

Language

Language variants of FTD

Semantic memory Semantic fluency Synonym judgments Naming Reading Repetition Oral production

Conclusions References

Chapter 10 Imaging of frontotemporal dementia

Clinical syndromes

Behavioral variant FTD (bvFTD) Frontotemporal dementia with motor neuron disease/amyotrophic lateral sclerosis (FTD-MND/ALS) Primary progressive aphasia (PPA)

Semantic variant PPA (svPPA) Non-fluent variant PPA (nfvPPA) Logopenic variant PPA (lvPPA) Other forms of PPA

Comparison of bvFTD and the PPA syndromes

Genetic syndromes Pathologic syndromes

Comparison of FTD with other neurodegenerative disorders

Brain–behavior correlation

Behavioral variant FTD Primary progressive aphasia

References

Chapter 11 Cerebrospinal fluid biomarkers of frontotemporal lobar degeneration

Introduction Hypothesis-driven CSF research in FTLD

Progranulin and TDP-43 in CSF of FTLD patients CSF tau and P-tau in FTLD patients CSF amyloid in FTLD patients Neurofilaments in FTLD patients

Non-hypothesis-driven research in FTLD: biomarker discovery Summary References

Chapter 12 Genetic counseling for FTD

Introduction Conclusions References

Section 4 Pathology and pathophysiology

Chapter 13 Neuropathology of frontotemporal dementia and related disorders

Introduction FTLD-tau

Normal tau expression and function Tau in disease Neuropathologic subtypes of FTLD-tau

Pick's disease Progressive supranuclear palsy Corticobasal degeneration Globular glial tauopathies FTD and parkinsonism caused by MAPT mutations Other tauopathies

Models and pathogenic mechanisms

FTLD-TDP

Normal TDP-43 expression and function TDP-43 in disease Pathologic subtypes of FTLD-TDP

FTLD-TDP type A FTLD-TDP type B FTLD-TDP type C FTLD-TDP type D

Familial FTLD-TDP

FTLD-TDP due to GRN mutations FTLD-TDP due to C9orf72 repeat expansion mutation FTLD-TDP due to VCP mutations FTLD-TDP due to TARDBP mutations

TDP-43 pathology in other neurodegenerative disease Pathogenesis of FTLD-TDP

FTLD-FET

Normal FET expression and function FET proteins in disease FTLD-FET subtypes

Atypical FTLD-U Neuronal intermediate filament inclusion disease Basophilic inclusion body disease

FET proteins in other neurodegenerative diseases FTLD-FET pathogenesis

FTLD-UPS Other pathologic causes of FTD Molecular correlates of FTD phenotypes Conclusions References

Chapter 14 Genetics of frontotemporal dementia and related disorders

Introduction Mendelian FTLD genes

MAPT GRN C9orf72 VCP CHMP2B Mendelian ALS genes TARDBP and FUS

Susceptibility genes and risk loci in FTLD

TMEM106B Risk variations in Mendelian FTLD genes

Concluding remarks Note Acknowledgments References

Chapter 15 Pathophysiology and animal models of frontotemporal dementia

C9ORF72

Pathophysiology Animal models

Tau

Pathophysiology Animal models

Progranulin

Pathophysiology Animal models

TDP-43 and FUS

Pathophysiology Animal models

VCP

Pathophysiology Animal models

CHMP2B

Pathophysiology Animal models

Conclusions References

Section 5 Treatment

Chapter 16 Functional disability and the impact of frontotemporal dementia in everyday life

How activities of daily living are defined and evaluated Patterns of functional impairment in FTD subtypes, and their progression

Behavioral variant FTD Primary progressive aphasia: semantic variant Primary progressive aphasia: non-fluent variant Primary progressive aphasia: logopenic variant Corticobasal degeneration and progressive supranuclear palsy

Relationship between cognitive deficits and behavioral changes with ADLs Impact of functional disability and disease progression on family carers Improving functional abilities in FTD: therapeutic recommendations Conclusions/summary Cases References

Chapter 17 Practical management of frontotemporal dementia

Initial clinic contact and early-stage management Management at middle stages Pharmacologic management Non-pharmacologic interventions

Cognitive impairments Communication impairments Motor impairments Behavioral disturbances and neuropsychiatric impairments

Management at advanced stages Conclusions References

Chapter 18 Pharmacologic therapy for FTD and related disorders

Introduction Neurotransmitter-based symptomatic treatments

Antidepressants Acetylcholinesterase inhibitors Antipsychotics and dopaminergic therapies Anti-epileptics Memantine Parkinsonism treatment

Potential disease-modifying treatments

Targets

Tau

Tau gain-of-function therapies Tau loss-of-function therapies

TDP-43 Progranulin Immunogenicity

C9ORF72

Models

Pluripotent stem cells Mouse Other

Future directions

Industry cooperation Clinical trial considerations

Conclusions References

Chapter 19 The family's perspective on FTD

Stage I. Something is wrong Stage II. Maybe there is a medical problem Stage III. A series of medical professionals and opinions/diagnoses Stage IV. Referral to a physician familiar with FTD Stage V. The diagnosis is FTD Managing the disease through partnership

Avoid nihilism after the diagnosis Identify a collaborative team Recognize the impact of disrupted social relationships and obligations among younger individuals Suggest anticipatory legal and financial planning Assist with community placement Discuss research opportunities

Creating a broader community for resources and support

AFTD initiatives and programs

Promoting and funding research Supporting and informing families Educating medical and healthcare professionals Promoting awareness Advocating for support and research funding Facilitating international partnerships

A force for change

Index

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