Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders Book

Acknowledgements ix

Introduction 1

1 Cognitive function, neuropsychological evaluation, and syndromes of cognitive impairment 6

1.1 Attention 8

1.2 General intelligence, IQ 10

1.3 Memory 11

1.4 Language 15

1.5 Perception 16

1.6 Praxis 19

1.7 Executive function, 'frontal function' 20

1.8 'Bedside' neuropsychological test instruments 22

1.9 Normal aging 32

1.10 Dementia, delirium, depression 33

1.11 Cortical versus subcortical dementias, thalamic dementia 35

1.12 Disconnection syndromes 37

2 Neurodegenerative disorders 39

2.1 Alzheimer's disease (AD) 40

2.2 Frontotemporal lobar degenerations (FTLD) 49

2.3 Motor neurone disease (MND), amyotrophic lateral sclerosis (ALS) 63

2.4 Parkinson's disease dementia (PDD) and dementia with Lewy bodies (DLB) 69

2.5 Prion diseases 82

2.6 Mild cognitive impairment (MCI) 87

3 Cerebrovascular disease: vascular dementia and vascular cognitive impairment 90

3.1 Cortical vascular dementia, multi-infarct dementia (MID), post-stroke dementia 93

3.2 Subcortical vascular dementia, Binswanger's disease, lacunar state, subcortical ischaemic vascular disease (SIVD) 95

3.3 Strategic infarct dementia, strategic strokes 97

3.4 Subarachnoid haemorrhage (SAH) 101

3.5 Intracranial vascular malformations 104

3.6 Vasculopathies 106

3.7 Other cerebrovascular disorders 112

4 The epilepsies 115

4.1 Epilepsy and cognitive impairment 115

4.2 Cognitive decline and epilepsy: shared aetiology 116

4.3 Seizures causing acquired cognitive impairment 120

4.4 Antiepileptic drug therapy causing cognitive impairment 122

4.5 Treatment of cognitive problems in epilepsy 123

5 Neurogenetic disorders 125

5.1Hereditary dementias 126

5.2 Hereditary ataxias 135

5.3 Hereditary spastic paraplegia 140

5.4 Hereditary movement disorders 141

5.5 Hereditary metabolic disorders 146

5.6 Hereditary neurocutaneous syndromes (phakomatoses) 155

6 Inflammatory, immune-mediated, and systemic disorders 157

6.1 Multiple sclerosis (MS) 157

6.2 Acute disseminated encephalomyelitis (ADEM) 165

6.3 Sarcoidosis 165

6.4 Systemic lupus erythematosus (SLE) 166

6.5 Sjogren's syndrome 168

6.6 Behcet's disease 169

6.7 Rheumatoid arthritis (RhA) 170

6.8 Scleroderma, systemic sclerosis 170

6.9 Relapsing polychondritis 171

6.10 Cerebral vasculitides 171

6.11 Sydenham's chorea, paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) 173

6.12 Limbic encephalitis 174

6.13 Hashimoto's encephalopathy (HE) 176

6.14 Erdheim-Chester disease 176

6.15 Bilateral vestibulopathy 177

7 Structural brain lesions 178

7.1 Brain tumours and their treatment 178

7.2 Hydrocephalic dementias 182

7.3 Other structural lesions 186

8 Endocrine, metabolic, and toxin-related disorders 188

8.1 Endocrine disorders 188

8.2 Metabolic disorders 193

8.3 Toxin-related disorders 200

9 Infective disorders 204

9.1 Encephalitides and meningoencephalitides 205

9.2 Meningitides 209

9.3 Human immunodeficiency virus (HIV) and related conditions 210

9.4 Other disorders of infective aetiology 211

10 Neuromuscular disorders 215

10.1 Myotonic dystrophy (Steinert disease) 215

10.2 Myasthenia gravis (MG) 216

Index 218