Contents. How to Use This Book? General Background Main Classification System A Route-Map or Classification Tree...

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1 1 How to Use This Book? General Background Main Classification System A Route-Map or Classification Tree Dementia: Clinical Background What Is Dementia? Prevalence and Incidence Prevalence Incidence Nosological Approach Genetic/Protein Classification Clinical and Pathological Uncertainty Differential Diagnosis Diagnostic Evaluation Multidisciplinary Integration The Toolbox Introduction: Choosing the Right Tools Structural Imaging Data Acquisition Feature Extraction Introduction: Structured Reporting Visual Rating Linear Measurements for MRI and/or CT Quantitative Techniques (contribution by Giorgos Karas) Advanced MR Techniques Diffusion Tensor Imaging (DTI) MR Perfusion Imaging MR Spectroscopy Brain Activation Functional MRI (fmri) SPECT and PET (contribution by Bart van Berckel) Data Acquisition Introduction SPECT Technique and Tracer Generation PET Technique and Scanners Feature Extraction

2 x 4 Normal Ageing Introduction Don t Worry, It is Normal? Successful Ageing Versus Usual Ageing Brain Volume Loss Global Brain Atrophy Medial Temporal Lobe Atrophy Versus Hippocampal Cavities Enlarged Perivascular (Virchow Robin) Spaces and Lacunes Microbleeds Age-Related White Matter Hyperintensities Iron Accumulation Age-Related Changes in Normal Appearing Brain Tissue Metabolic and Microstructural Changes Functional Changes in Normal Ageing Is It Normal or Not? Primary Grey Matter Loss Introduction: Primarily Grey Matter Loss Alzheimer s Disease History Histopathology Genetics Clinical Presentation Differential Diagnosis of AD Neuroimaging Strategy and Findings in AD Recommendations for Further Imaging Frontotemporal Lobar Degeneration (Jonathan D. Rohrer) Introduction and Synonyms History and Nosology Epidemiology, Aetiology and Genetics Histopathology Clinical Features and Imaging Strategy Differential Diagnosis, Imaging Follow-up (Including PET/SPECT), Other Tests (CSF/EEG) Dementia with Parkinsonism Introduction: Neurodegeneration, Parkinsonism and Dementia Dementia with Lewy bodies and Parkinson s Disease Dementia (John O Brien and Michael Firbank) Progressive Supranuclear Palsy (PSP) (Dominic Paviour) Multiple System Atrophy (MSA) (Dominic Paviour) Cortico-Basal Degeneration (CBD) and Other FTLD Tauopathies (Dominic Paviour) Dementia in Other Movement Disorders Introduction Huntington s Disease (Ed Wild, Nicola Hobbs and Susie Henley)

3 xi 6..3 Metal Metabolism Disorders Dentatorubral-Pallidoluysian Atrophy Prion-Linked Dementias Introduction History, Nosology and Transmission Biochemistry, Genetics and Histopathology Creutzfeldt Jakob Disease Gerstmann Sträussler Scheinker (GSS) Syndrome Fatal Familial Insomnia (FFI) Recreational Drugs and Alcohol Introduction Alcohol-Induced Dementia Marchiafava Bignami Disease Cannabis, Ecstasy and Cocaine Nicotine Vascular Dementia Introduction History and Nosology Binswanger s Disease, État lacunaire, Multi-infarct Dementia, Strategic Infarct Dementia Current Concepts Current Diagnostic Criteria and Limitations Towards More Homogeneous Criteria Large Vessel Vascular Dementia Aetiology, Pathology and Genetics Clinical Presentation, Epidemiology and Treatment Neuroimaging Strategy and Key Findings in Large Vessel VaD Differential Diagnosis Small Vessel Vascular Dementia Age-Related White Matter Changes, Lacunes, État criblé What Is Abnormal for Age? Subcortical Ischaemic Vascular Dementia Specific Types of Microangiopathy Vasculitis Primary CNS Vasculitis Secondary CNS Involvement Systemic Lupus Erythematosus (SLE) Susac s Syndrome Systemic Hypoxia Anaemia Transient Global Amnesia Mixed Dementia and Interrelationship of Vascular Pathology and Alzheimer s Disease (Wiesje van der Flier) Introduction The Contribution of Cerebrovascular Pathology to Alzheimer s Disease The Contribution of Neurodegenerative Pathology to VaD

4 xii How Do Neurodegenerative and Cerebrovascular Changes Interact? Shifting Paradigms Suggestions for Further Reading Disorders Mainly Affecting White Matter Introduction Focus on White Matter Involvement Infections Introduction Viral Infections Leading to Dementia Bacterial Infections: Whipple s Disease Spirochetal Infections and Dementia Inflammatory Disorders Introduction Multiple Sclerosis and ADEM Neurosarcoidosis Coeliac Disease Inborn Errors of Metabolism Introduction Fragile X-Associated Tremor/Ataxia Syndrome (FXTAS) Mitochondrial Dementia Diffuse Neurofibrillary Tangles with Calcification (DNTC) Cerebrotendinous Xanthomatosis (CTX) Adult Polyglucosan Body Disease (APBD) (Contribution by Bert-Jan Kerklaan) Neuroaxonal Leukodystrophy (NAL) Late-Onset Metachromatic Leukodystrophy Adrenomyeloneuropathy (AMN) Vanishing White Matter Disease (VWM) Globoid Cell Leukodystrophy (Krabbe s Disease) Toxic Leukoencephalopathy and Dementia (Mike P. Wattjes) Introduction and Classification Vitamin B Deficiencies Chronic Kidney Disease and Dialysis Carbon Monoxide Poisoning Delayed Post-hypoxic Demyelination Organic Solvent Inhalation Heroin Vapour Inhalation Cognitive Dysfunction Associated with Cancer Therapy (Mike P. Wattjes) Trauma Dementia Associated with Traumatic Brain Injury (Mike P. Wattjes) Dementia Pugilistica

5 xiii 8 Dementias with Associated Brain Swelling Introduction Giant Virchow Robin Spaces (VRS) Infections Herpes Simplex Encephalitis Progressive Multifocal Leukoencephalopathy (PML) Neoplastic Disease and Radiation Necrosis Eloquent Areas and Common Tumours Gliomatosis Cerebri Diffusa Intravascular Lymphomatosis Radiation Necrosis Autoimmune Limbic Encephalitis (Jonathan Schott) History and Nosology Clinical Presentation, Pathology and Differential Diagnosis Paraneoplastic Limbic Encephalitis Non-neoplastic Autoimmune Encephalitis Normal Pressure Hydrocephalus (NPH) History and Nosology Clinical Presentation Neuropathology and Aetiological Considerations Neuroimaging Strategy and Findings Differential Diagnosis Shunt Response Arteriovenous Malformations (AVM) and Dural Arteriovenous Fistula (davf) Reversible Posterior Leukoencephalopathy (RPLS) Amyloid-Targeted Therapy Index

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