Handbook of Medical Neuropsychology (eBook)

Carol L. Armstrong is a neuropsychologist conducting cognitive neuroscience research. Her long-term interests are the study of memory and attention, and the cognitive processes affected by white matter disease or injury. Investigations have focused on resource-limited memory and attention processes, and on the structural distribution of the white matter measure in normal and injured brains. Recent investigations include the longitudinal damaging effects of radiotherapy and brain tumors in both children and adults, as shown by longitudinal, prospective studies of cognition and neuroimaging of white matter integrity, and response of the hippocampus to radiotherapy. Current studies are being conducted on the functional and structural significance of cerebellar connections with the cortex.

Foreword 6
References 9
Preface 10
Contents 12
Contributors 15
Part I Primary Nervous System Disease 20
1 Epilepsy and Cognitive Plasticity 21
Introduction: Why Study Cognition in Epilepsy 21
Biological Bases for Epilepsy 22
General Cognitive Characteristics of Epilepsy 22
Chronicity of Seizures 23
Seizures Initiate Neuroplasticity 24
Cognitive Deficits Outside the Epileptogenic Zone and the Development of Neural Networks 25
Diaschisis and Inhibition 25
Seizure Propagation 25
Secondary Epileptogenesis 26
Seizures as an Example of Maladaptive Plasticity 26
Cognitive Reorganization from Epilepsy 27
The Role of Neuropsychology in Epilepsy 28
The Changing Surgical Algorithm and Neuroimaging 29
FMRI and Other Neurocognitive Tools in Epilepsy 31
Future Directions 32
Conclusion 32
References 32
2 Traumatic Brain Injury 35
Epidemiology 35
Etiology 35
Mechanisms of Injury 35
Primary and Secondary Injuries 36
Types of Injury 36
Penetrating Head Injury 36
Closed Head Injury 36
Rating Severity of TBI 37
Loss of Consciousness 37
Posttraumatic Amnesia 37
Severity Classifications 37
Neuroimaging and TBI 39
Structural Imaging 39
Diffusion Tensor Imaging 39
Magnetic Resonance Spectroscopy 39
Functional Imaging 40
Frontal Systems, Cognition, and Behavior 40
Dorsolateral Prefrontal Circuit 40
Orbitofrontal Circuit 41
Anterior Cingulate 41
Neuropsychological Assessment of TB I 42
Attention 42
Memory 42
Executive Functions 43
Executive Functions: The Need for Subcategories 45
Neuropsychological Assessment as a Dynamic Process 46
Summary 46
References 47
3 Neuropsychological Problems in Neuro-oncology 51
Introduction and History 51
Incidence of CNS Tumors 52
Biological Processes of Brain Neoplasms 52
The Genesis of Brain Tumors 52
Diagnosis of Brain Neoplasms 55
Risks for Developing a Brain Tumor 55
Brain Tumor Classification and Histologic Groupings 55
Tumor Grading 55
Neuropsychological Mechanisms 56
Effects of Tumors on Cognitive Function 56
Do Tumors Cause Regional Cognitive Effects? 57
Conclusions 59
Regional Effects in Individuals 59
Individualized Approach to Neuropsychological Evaluation and Case Examples 59
Sensitivity of Neuropsychological Evaluation 60
Effects of Neurosurgery on Cognitive Function 61
Syndromal Neuropsychiatric Disturbances and Treatments Associated with Brain Tumors 61
Effects of Adjuvant Treatments on Cognition 64
Radiation Therapy (Radiotherapy) 64
Conclusions 70
References 70
4 Multiple System Atrophy, Orthostatic Hypotension, and Autonomic Dysfunction and Cognition 75
Introduction 75
Clinical Features 75
Diagnosis 76
Pathology 79
Cognition, Attention, and Depression in MSA 79
Mood 80
Treatment 81
Parkinsonism 81
Cerebellar Dysfunction 81
Dysautonomia 82
Orthostatic Hypotension 82
Conclusions and Future Directions 82
References 83
Part II Vascular System Disease 85
5 Cardiovascular Disease and Neurocognitive Function 86
Cardiovascular Disease Classification 87
Cardiovascular Risk Factors and Neurocognitive Function 87
Genetics 87
Traditional Biomedical Risk Factors 87
Hypertension/Antihypertensives 88
Lipids/Statins 89
Obesity 90
Diabetes, the Metabolic Syndrome, Glucose, Insulin 91
Biomarkers 93
Inflammation 93
Oxidative Stress 94
Biomarkers of Chronic Kidney Disease (CKD) 94
Homocysteine 95
Behavioral Risk Factors 95
Smoking 95
Alcohol 96
Diet/Antioxidants 96
Physical Activity, Exercise 96
Psychosocial Risk 97
Psychophysiological Risk 97
Autonomic Nervous System 97
Hypothalamic--Pituitary--Adrenocortical (HPA) Axis 97
Summary 98
Cardiovascular Diseases and Neurocognitive Function 99
Cardiac Arrhythmias, Cardiac Arrest 99
Subclinical Cardiovascular Disease 100
Atherosclerosis 100
Arterial Stiffness 101
Endothelial Dysfunction 101
Left Ventricular Hypertrophy 102
Mechanisms 102
Coronary Heart Disease (CHD) 102
Coronary Artery Bypass Grafting (CABG) 103
Peripheral Arterial Disease 103
Heart Failure/Heart Transplantation 104
Summary 105
Discussion 105
References 107
6 Cerebrovascular Disease and Disorders 117
Introduction 117
Medical Information Regarding Cerebrovascular Disorders 117
Risk Factors for Cerebrovascular Disorders 117
Clinical Presentation of Cerebrovascular Disorders 118
Diagnosis of Cerebrovascular Disorders 118
Treatment of Cerebrovascular Disorders 120
Mechanisms Underlying Cognitive Dysfunction in Cerebrovascular Disorders 121
Neuropsychological Assessment Following Stroke 122
Assessment in the Acute Phase 122
Intellectual Functioning 123
Language 124
Memory 125
Attention and Neglect 126
Executive Functioning 128
Higher-Order Visual Processing Skills 130
Fine Motor and Sensory Functioning 131
Emotional and Behavioral Functioning 131
Long-Term Neuropsychological Outcome 132
Treatment Approaches to Cognitive Impairment Due to Cerebrovascular Disease 133
References 134
7 Cognitive Declines During Migraine and Cluster Headaches Are Caused by Cerebral 5HT Neurotransmitter Dysfunction 138
Introduction and Neurological Mechanisms of Headaches 138
History 139
Experimental Studies of Treatments 139
Participants 140
Hypotheses to Be Tested 141
Ethical Treatment 141
Results 141
References 142
8 Respiratory Disorders: Effects on Neurocognitive and Brain Function 144
Introduction 144
Effects of Hypoxia 144
Mechanisms of Brain Injury 145
Neuroimaging Findings 145
Neurological and Neuropsychological Sequelae 145
Chronic Obstructive Pulmonary Disease 146
Neurocognitive Morbidity 146
Neuropsychiatric Morbidity 147
COPD Summary 148
Acute Respiratory Distress Syndrome 148
Mechanisms of Injury 148
Neurocognitive Morbidity 149
Neuropsychiatric Morbidity 149
ARDS Summary 150
Carbon Monoxide Poisoning 150
Neurocognitive Morbidity 150
Neuropsychiatric Morbidity 151
CO Summary 152
Obstructive Sleep Apnea 152
Neurocognitive Morbidity 152
Neuropsychiatric Morbidity 153
OSA Summary 153
Rehabilitation Outcomes Following Anoxia 153
Conclusions 154
References 154
Part III Developmental, Genetic, and Structural Disorders 161
9 Cerebral Palsy: Effects of Early Brain Injury on Development 162
Introduction 162
Etiology 162
Neuroimaging 163
Diagnosis 163
Prevalence 164
Neuropsychological Functioning 164
Intelligence 164
Visual-Perceptual Impairments 165
Attention Dysfunctions 166
The Crowding Hypothesis 166
Language and Speech Impairments 166
Arithmetic Difficulties 167
Limitations in Theory of Mind 167
Longitudinal Follow-Up of the Gross Motor and Cognitive Development 168
Problems with Cognitive Assessment in the Clinic 168
Intervention 169
Functional Limitations 169
Types and Efficacy of Intervention 170
Conclusions and Future Directions 172
References 172
10 Autism and Asperger's Syndrome: A Cognitive Neuroscience Perspective 177
History and Background 177
Biological Underpinnings 177
Neuroanatomic Abnormalities 177
Postmortem Studies 178
MRI Studies 178
White Matter Connectivity 179
EEG Abnormalities and Seizures 180
Diagnosis 181
Screening Guidelines 181
Clinical and Research Criteria 182
Increased Prevalence of Autism: It Is Not the Vaccine 182
Neurocognitive Mechanisms 183
Major Cognitive Models 183
Cognitive Profiles 184
Attention 184
Sensation/Perception 186
Language 188
Social/Emotional 189
Treatment 192
Behavioral 193
Pharmacologic 193
Summary and Conclusion 194
References 194
11 Genetic Syndromes Associated with Intellectual Disabilities 204
Down Syndrome 205
Genetics, Prevalence, and Overview 205
Behavioral Phenotype 206
Linguistic Dimensions of the Phenotype 207
Neural Bases of the Phenotype 209
Summary 211
Fragile X Syndrome and Related Conditions 211
Genetics, Prevalence, and Overview 211
Full Mutation 211
Premutation 215
Summary 215
Williams Syndrome 215
Genetics, Prevalence, and Overview 215
Behavioral Phenotype 216
Linguistic Dimensions of the Behavioral Phenotype 217
Neural Bases of the Phenotype 219
Summary 220
Issues in the Neuropsychological Assessment of Individuals with Intellectual Disabilities of Genetic Origin 220
Implications 220
Limitations 222
Families and the Neuropsychological Assessment of Individuals with Intellectual Disabilities of Genetic Origin 223
References 224
12 An Introduction to Hydrocephalus: Congenital and Late-Life Onset 233
Introduction 233
The Ventricular System 233
Neuropsychological Outcomes in Congenital Hydrocephalus 234
Intelligence 235
Attention and Executive Functioning in Children 236
Memory 236
Language 236
Visuospatial 237
Motor 237
Emotional Functioning 237
Overall Profile in Congenital Hydrocephalus 238
Etiology of Profile 239
Late-Life Onset: Idiopathic Normal Pressure Hydrocephalus 239
Clinical Presentation 239
Cognitive Profile 240
Traditional Treatment: Shunting 240
New Treatment Approach: Endoscopic Third Ventriculostomy 241
Summary 242
References 243
13 Learning Disabilities 247
History and Background 247
Prevalence Rates of LD 248
The Process of Diagnosis 249
Evaluating Children at Risk for LD 250
Biological and Neuropsychological Mechanisms 252
Genetic Influences 252
Brain Mechanisms and Correlates of Dyslexia 252
Brain Mechanisms and Correlates of Dyscalculia 254
Brain Mechanisms and Correlates of Dysgraphia 255
Treatment 255
Summary and Future Directions 256
References 257
14 Frontal Lobe Disorders in Pediatric Neuropsychology: Attention-Deficit Hyperactivity Disorder and Tourette Disorder 261
Introduction 261
Attention-Deficit Hyperactivity Disorder 261
Definition 261
Etiology 262
Neuroanatomy/Pathophysiology 263
Comorbidity 264
Assessment 265
Treatment: Overview 266
Psychoeducation 266
Medical Treatment 266
Behavior Management 268
Long-Term Outcome 270
Tourette Disorder 271
Definition 271
Phenomenology 271
Prevalence and Etiology 272
Neurobiology 273
Associated Conditions/Comorbidity 275
Neuropsychological Functions 276
Evaluation 277
Treatment 277
Psychosocial Interventions 278
Medications 279
Somatic Treatments 280
Prognosis/Long-Term Outcomes 280
References 281
Part IV Aging 284
15 Dementia 285
Introduction 285
Neuropsychological Testing of Dementias 286
Alzheimers Disease 287
Neuropsychology of AD 288
Areas Relatively Preserved 291
Symptoms Associated with Age of Onset in AD 291
Emotional and Psychotic Symptoms 291
Posterior Cortical Atrophy 292
Dementia with Lewy Bodies (DLB) 293
Parkinsons Disease Dementia (PDD) 295
Binswangers Dementia 296
Subcortical Arteriosclerotic Encephalopathy 296
Transmissible Spongiform Encephalopathies (Prion Diseases) 297
Mild Cognitive Impairment [Similar to Cognitively Impaired, No Dementia (CIND)] 298
Conclusion 299
References 299
16 Theoretical Perspectives on Cognitive Aging 304
Historical Perspective 304
Neurological Changes Associated with Cognitive Aging 305
Theories of Cognitive Aging 306
Speed of Processing 306
Inhibitory Deficit Hypothesis 307
Self-Initiated Processing Deficits 309
What Do We Know So Far 311
Neuropsychological and Neurocognitive Variability in the Older Adult Population 311
Gender 311
Frontal Functioning 311
Mild Cognitive Impairment 313
Treatment in Light of Cognitive Interactions 313
Conclusions and New Directions 315
References 316
17 Neuropsychology of Movement Disorders and MotorNeuron Disease 321
Introduction 321
Parkinson's Disease 321
Biological Underpinnings and Diagnosis 321
Neuropsychological Mechanisms 322
Attention and Executive Functions 322
Motor Skills and Information Processing Speed 322
Language 323
Learning and Memory 323
Visuoperception 323
Neuropsychiatric Factors 324
Progressive Supranuclear Palsy 324
Biological Underpinnings and Diagnosis 324
Neuropsychological Mechanisms 324
Attention and Executive Functions 325
Motor Skills and Information Processing Speed 325
Language 325
Learning and Memory 325
Visuoperception 325
Neuropsychiatric Features 325
Essential Tremor 326
Biological Underpinnings and Diagnosis 326
Neuropsychological Mechanisms 326
Attention and Executive Functions 327
Motor Skills and Information Processing Speed 327
Language 327
Learning and Memory 327
Visuoperception 327
Neuropsychiatric Factors 328
Huntington's Disease 328
Biological Underpinnings and Diagnosis 328
Neuropsychological Mechanisms 328
Attention and Executive Functions 328
Motor Skills and Information Processing Speed 329
Language 329
Learning and Memory 329
Visuoperception 329
Neuropsychiatric Features 329
Amytrophic Lateral Sclerosis (Lou Gehrig's Disease) 330
History and Diagnosis 330
Biological Underpinnings 330
Neuropsychological Mechanisms 330
Attention and Executive Functions 330
Motor Skills and Information Processing Speed 331
Language 331
Learning and Memory 331
Visuoperception 331
Neuropsychiatric Factors 331
Conclusions 332
References 332
Part V ImmuneMediated Disease 340
18 Cognitive and Affective Neuroscience Theoriesof Cognition and Depression in Multiple Sclerosisand Guillain--Barre Syndrome 341
Multiple Sclerosis 341
Pathophysiology, Clinical Presentation, and Course 341
Cognitive Neuroscience Theories 342
Possible Causes of Cognitive Deficits 344
Neuropsychological Assessment Tools 345
Relationship Between Cognitive Deficits and Illness Variables 348
Neurocognitive Theories of Depression 348
Family and Social Issues 351
GuillainBarr Syndrome 352
Pathophysiology, Clinical Presentation, and Course 352
Neuropsychological Factors 353
Summary and Conclusions 353
References 354
19 Neurocognitive Function in Systemic Autoimmune and Rheumatic Diseases 358
Introduction 358
Systemic Lupus Erythematosus 358
Definitions and Epidemiology 358
Classification of Neuropsychiatric SLE 358
Pathophysiology of Cognitive Dysfunction Is Elusive 359
Risk Factors for Cognitive Dysfunction 360
The Role of Neuropsychological Testing in the Diagnosis of Cognitive Dysfunction in SLE 361
Neuroimaging Modalities in Studies of Cognitive Dysfunction 366
Rheumatoid Arthritis 367
Sjgrens Syndrome 369
Family and Social Issues 371
Treatment 371
Summary and Conclusions 372
References 372
20 HIV-AIDS: The Neurologic and Cognitive Consequences of HIV-1 Infection 376
Neuropathological Consequences of HIV Infection 376
Common HIV Staging Criterion 378
Cognitive Domains Affected 382
Attention 382
Memory 384
Language 384
Psychomotor Abilities 385
Executive Functions 385
Visuospatial Deficits 385
Cognitive Sequelae 386
Cognitive Batteries Used to Assess HIV-Associated Cognitive Dysfunction 387
Cognitive Confounds 388
Alcohol and Drug Abuse Issues 388
Co-infection with Hepatitis C 390
Advanced Age, HIV, and the Brain 391
Additional Host Risk Factors 392
Psychiatric Illness 392
Effects of HIV Treatment on Brain Function 393
Relevant Family or Social Issues 394
Future Directions 394
References 395
21 Rheumatologic Conditions: Sjogren's Syndrome, Fibromyalgia, and Chronic Fatigue Syndrome 400
Fibromyalgia 400
Working Memory 400
Episodic Memory 404
Verbal Fluency 405
Attention and Concentration 405
Neuroimaging and Brain Activity 406
Chronic Fatigue Syndrome 407
Summary of Neuropsychological Findings 408
Neuroimaging and Brain Activity 409
Other Rheumatologic Conditions 410
Sj'gren's Syndrome and Cognitive Function 410
Chronic Pain and Cognitive Function 411
Summary 411
References 411
Part VI Endocrine Disease 415
22 Neuropsychological Sequelae of Type 1 and Type 2 Diabetes 416
Type 1 Diabetes 416
Overview of Type 1 Diabetes 416
T1D Cognitive Effects in Adults 416
Hypoglycemic Effects on Adult T1D Cognition 417
Hyperglycemic Effects on T1D Cognition 418
Cognitive Effects of Pediatric T1D 418
Hypoglycemia in T1D Youth 419
Hyperglycemia in T1D Youth 419
Early Onset T1D (EOD) and the Developing Brain 419
Cerebral Microvascular Effects in T1D 420
Depression and Cardiovascular Disease in T1D 421
T1D Conclusions and Implications 421
Type 2 Diabetes 421
Overview of Type 2 Diabetes 421
Cognitive Function in T2D 422
Cognitive Decline in T2D 422
Effects of Age and Disease Duration on T2D Cognitive Status 422
Hyperglycemic Effects in T2D 423
T2D and Hypoglycemia 423
Cerebral Microvascular Disease in T2D 423
Comorbid Macrovascular Disease 424
Depression and T2D 425
Alzheimer's Disease 425
Implications and Future Directions in T2D 425
T1 and T2 Diabetes: Overview and Conclusions 426
References 427
23 Neuropsychological Functioning of Endocrinology Disorders: Gonadotropic Hormones and Corticosteroids 431
Sex Hormones 431
Overview of Sex Hormones 431
Estrogen 431
Alzheimer Disease and HRT in Women 432
Turner Syndrome 433
Testosterone 434
Klinefelter Syndrome 435
Corticosteroids, Cushing Syndrome, and Addison Disease 436
Overview of Corticosteroids 436
Effects of Administration of Exogenous Steroids 436
Cushing Syndrome 437
Adrenal and Corticosteroid Insufficiency 438
Sex Hormones and Corticosteroids: Summary and Future Directions 439
References 440
24 Neuropsychological Assessment of Posttraumatic Stress Disorder (PTSD) 446
Description of the Disorder 446
Diagnostic Criteria and Prevalence 446
Course and Associated Clinical Features 447
Neurobiological Basis of PTSD 447
Neuroimaging Findings 448
Structural Imaging and Magnetic Resonance Spectroscopy (MRS) 448
Functional Imaging 448
Summary and Related Literatures 448
Neuropsychological Functioning in PTSD 449
Empirical Findings 449
Implications for Clinical Evaluation 450
Neurobehavioral Instrument Selection 452
The Emotional Stroop Paradigm 453
Treatment Implications 454
Pharmacological Treatment 454
Psychotherapy 454
Family Considerations 455
Conclusions 455
References 456
Part VII Metabolic Disease 465
25 Hepatic Encephalopathy 466
Neurocognitive and Neuroscience Theory and Background 466
Review of the Attempts to Identify the Most Specific Neuropsychological Assessment for Diagnosis 467
Altered Mental Status in Patients with Cirrhosis It Is Not All HE
Physical Examination 468
Motor Exam 468
Neuropsychological Examination and Psychometric Testing 468
Neurophysiologic Testing 469
Limitations of Currently Available Psychometric and Neurophysiologic Tests 469
Additional Helpful Information About the Diagnostic Question 469
Population to Be Tested 470
State-of-the-Art Diagnostic, Open Access, and Treatments 470
State of the Art and Open Access Diagnostic Strategies 470
Treatments for HE 471
A Brief Section on Relevant Family or Social Issues 471
Summary 472
References 472
26 Toxic Disorders and Encephalopathy 475
Organic Solvents 475
Cognitive Changes 476
Emotional/Behavioral Changes 476
Neuroimaging 477
Lead 478
Cognitive Function 478
Emotional Functioning 479
Neuroimaging 479
Carbon Monoxide 480
Cognitive Symptoms 481
Emotional Symptoms 481
Neurological Changes 481
Neuroimaging 482
Additional Issues in the Assessment of Toxic Exposure 483
References 483
27 Neurocognition in Mitochondrial Disorders 486
Background 486
The Process of Diagnosing a Mitochondrial Disorder 487
Biological Underpinnings of Mitochondrial Disorders 488
Mitochondrial Encephalomyopathy with Lactic Acidosis and Stroke-Like Episodes (MELAS) 488
Myoclonic Epilepsy with Ragged-Red Fibers (MERRF) 489
Kearns--Sayre Syndrome (KSS) 490
Leigh Syndrome (LS) and Neurogenic Muscle Weakness, Ataxia, Retinitis Pigmentosa (NARP) 490
Leber Hereditary Optic Neuropathy (LHON) 491
Barth Syndrome 491
Non-syndromic Mitochondrial Disease 491
Treatment 492
Neurological Similarities Among Mitochondrial Disorders 492
Basal Ganglia Calcification 492
White Matter Anomalies 492
Neuropsychological Assessment in Mitochondrial Disorders 493
Conclusions/Future Directions 493
References 494
Part VIII Rehabilitation 497
28 Current Approaches to Cognitive Rehabilitation 498
Current Approaches to Rehabilitation 498
Compensatory Approaches 498
Restorative Approaches 498
Metacognitive Approaches 498
Lessons from Plasticity 499
Sensory and Motor Functions 499
Cognitive Domains 499
Attention 500
Memory 501
Environmental Modification 501
Vanishing Cues 501
Errorless Learning 502
Distributed Practice 502
Prospective Memory Training 502
Other Techniques 502
Academic Strategies 503
Language 503
Dyslexia and Developmental Reading Disorders 504
Executive Functions 504
Compensatory Strategies 504
Behavioral Treatments 504
Direct Training 505
Metacognitive Strategies 505
Normal Aging 506
Virtual Reality 506
Brain--Computer Interface 507
Conclusions 507
References 508
29 Sensory Reweighting: A Rehabilitative Mechanism0 511
History and Background 511
Sensory Reweighting in Older Adults 512
Balance Training 513
Multisensory Integration: The Light-Touch/Vision Paradigm 514
Sensory Reweighting in the Fall-Prone Elderly Population 515
A Multisensory Intervention 517
Conclusions 519
References 519
Subject Index 522