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دانلود کتاب Neurology: A Queen Square Textbook
دانلود کتاب عصب شناسی: کتاب درسی مربع کوئین
مشخصات کتاب
Neurology: A Queen Square Textbook
ویرایش: 3 نویسندگان: Robin Howard, Dimitri Kullmann, David Werring, Michael Zandi سری: ISBN (شابک) : 1119715539, 9781119715672 ناشر: Wiley-Blackwell سال نشر: 2024 تعداد صفحات: 1280 زبان: English فرمت فایل : PDF (درصورت درخواست کاربر به PDF، EPUB یا AZW3 تبدیل می شود) حجم فایل: 87 مگابایت
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Cover Title Page Copyright Page Contents Contributors About the Editors Foreword to the Third Edition Preface to First Edition Preface to Second Edition Preface to Third Edition Acknowledgements Chapter 1 Global Burden of Neurological Disease and the Neurology of Climate Change Introduction The Global Burden of Neurological Diseases National Global Burden of Neurological diseases Specific Neurological Disorders Conclusion Neurology and Global Warming Global Warming Impacts on Neurological Diseases Neurological Diseases may Increase Susceptibility to Consequences of Climate Change Contributions to Climate Change from Neurology Actions References Further Reading Chapter 2 Approach to the Patient with Neurological Disease What Makes a Neurologist? Approaching the Neurological Patient The ‘Neurological Method’ The Neurological History The Neurological Examination Level of Alertness and Orientation Higher Cerebral Function Gait and Mobility Cranial Nerves Visual Acuity Colour Vision Examining the Fundus Visual Fields Eye Position and Movement Ocular Movements Pupils V (Trigeminal Nerve) VII Facial Nerve VIII Auditory and Vestibular IX, X Glossopharyngeal, Vagus Nerve Accessory Nerve XI Hypoglossal Nerve (XII) Muscles of the Neck Limb Examination Sensory System Remote Neurological Consultation Assessment of the Patient with Hearing Deficits Consultations with Patients for Whom English is not Their First Language Neurological Consultation with an Autistic Patient Neurological Consultation with a Person Who has a Learning Disability Neurological Assessment in an Emergency Setting, Including Hyperacute Stroke Conclusion Acknowledgements References Further Reading Chapter 3 Decision Making, Ethics and Law in Neurology Introduction Fundamentals The Scale of the Challenge of Decision Making Consent Part I: Towards Systematic Moral Decision Making A Case Ethical Systems Faith-Based Ethics Moral Universalism and Moral Relativism The Four Principles Ethical Framework The Intrinsic Factors – What We Should Be Thinking About A. Application of the Four Principles Ethical System to Clinical Decision Making B. The Role of the Law Part II: The Extrinsic Factors – the Enemies of Decision Making? A. Making Moral Judgements B. Unexamined Assumptions C. Bias and Implicit Bias D. Noise E. Mitigations Conclusion References Further Reading Chapter 4 Neuropathology: Introduction to History, Diagnostic Approaches, Techniques and their Interpretation Introduction Historical Aspects of Neuropathology Neuropathological Techniques Processing and Analysis of Histological Samples Histological Techniques Tinctorial Stains Immunohistochemical Techniques Molecular Pathology Techniques Techniques for the Visualisation of Tissue Components Digital Pathology Interpretation of Histological Samples and Systematic Approach to Diagnosis Common Techniques in Molecular Diagnostics in Neuro-Oncology Sanger Sequencing Fluorescence or Chromogenic In Situ Hybridisation Multiplex Ligation-Dependent Probe Amplification Next-Generation Sequencing Pyrosequencing Methylation Array Nanopore Technology Reference Further Reading Chapter 5 Neuroimaging Clinical Scenarios in Neuroradiology Neurological Imaging Plain Film Radiography Computed Tomography Magnetic Resonance Imaging Ultrasound Imaging Catheter Angiography Choosing the Right Imaging Modality Safety and Contraindications Radiation Protection Safety of Magnetic Resonance Imaging Intravenous Contrast Material Iodinated Intravenous Contrast for CT Gadolinium-Based Contrast for Magnetic Resonance Special Populations Pregnancy Patients with Claustrophobia or Anxiety Anaesthesia for Computed Tomography and Magnetic Resonance Imaging Examinations Choosing the Right (Magnetic Resonance) Protocol Advanced Techniques Timing of Follow-Up Imaging Examinations Brain Tumours Subarachnoid Haemorrhage Multiple Sclerosis Standardised Film Reading and Reporting Radiology Report – Conclusion Incidental Findings Incidental Findings in Research Studies Communicating in Clinical Neuroradiology Reference Further Reading Chapter 6 Neurogenetics Introduction Manifestations that Suggest a Neurogenetic Disorder Genetics Modes of Inheritance Autosomal Dominant Inheritance Autosomal Recessive Inheritance Sex-Linked Inheritance Mitochondrial Disorders Repeat Expansion Diseases How Heritable are Neurological Conditions? Lessons from Twins Mutation Versus Polymorphism Practical Approach to the Patient and Family Ethical Considerations: Predictive Versus Diagnostic Incidental Findings in Diagnostic Exome and Genome Sequencing Future Directions References Further Reading Chapter 7 Neuroimmunology Introduction Autoimmunity Fundamentals Components of the Immune System Innate Immune System Adaptive Immune System Protection of Neural Tissues: the Blood–Brain and Blood–Nerve Barriers Analysis of Cerebrospinal Fluid Solutes Autoantibody Testing in Central Nervous System Disease in Serum and Cerebrospinal Fluid Immunological diseases of the Nervous system T-Cell-Mediated Neurological Disease Cytokine-Driven Processes Antibody-associated Neurological Diseases Neurological Disease Associated with Systemic Disorders and Systemic Autoantibodies Autoimmune Encephalitis Other Neurological Disease with Pathogenic Autoantibodies Interfering with the Immune System as a Treatment for Disease Therapeutic Approach to Immune-Mediated Neurological Disorders References Further Reading Chapter 8 Stroke and Cerebrovascular Diseases Clinical Approach, Terminology and Classification of Stroke The Burden of Stroke Anatomy of Ischaemic Stroke The Heart and Great Vessels Pathophysiology of Stroke Thrombosis, Occlusion, Embolism and Hypoperfusion Microscopic and Metabolic Changes Intracerebral Haemorrhage Clinical Approach to Stroke Diagnosis The Clinical History Examination Risk Factors and Causes of Ischaemic Stroke Clinical Syndromes of Cerebral Ischaemia Transient Ischaemic Attacks Alternative Diagnoses: Transient Ischaemic Attack ‘Mimics’ Lacunar Syndromes and Subcortical Ischaemic Stroke Ischaemic Stroke Due to Large-Vessel Occlusion Anterior Circulation Posterior Circulation Vascular Cognitive Impairment and Dementia Treatment of Acute Ischaemic Stroke Service Design, Public Awareness and Early Diagnosis Organised Care in a Stroke Unit Reperfusion Therapies for Acute Ischaemic Stroke Antiplatelet Therapy in Acute Ischaemic Stroke Anticoagulation in Acute Ischaemic Stroke Neuroprotection Maintenance of Homeostasis Treatment of Cerebral Oedema and Hemicraniectomy in Acute Ischaemic Stroke Management of ‘Progressive’ Ischaemic Stroke Common Medical Complications of Stroke Investigation of Ischaemic Stroke, Intracerebral Haemorrhage and Transient Ischaemic Attack Basic Investigations for All Patients Imaging in Acute Stroke Guided Investigations Following Basic Profile Prognosis of Ischaemic Stroke and Transient Ischaemic Attack Secondary Prevention of Ischaemic Stroke Rarer Vasculopathies That Can Cause Stroke Cervicocephalic Carotid and Vertebral Artery Dissection Cerebral Vasculitis Other Vasculitides Thrombotic Thrombocytopenic Purpura CADASIL CARASIL CARASAL Fabry’s Disease Susac’s Syndrome Sneddon’s Syndrome Degos’ Disease Collagen Gene Mutations Reversible Cerebral Vasoconstriction Syndrome Behçet’s Disease Mitochondrial Disease Hypertensive Encephalopathy Migraine and Stroke Endocarditis Moyamoya Disease Causes and Treatment of Intracerebral Haemorrhage Risk Factors and Causes of Intracerebral Haemorrhage Clinical Syndromes of Intracranial Haemorrhage Management of Acute Intracerebral Haemorrhage Prevention of Venous Thromboembolism Neurosurgery for Intracerebral Haemorrhage Investigation of Intracerebral Haemorrhage Secondary Prevention After Acute Intracerebral Haemorrhage Subarachnoid Haemorrhage Risk Factors Clinical Features Investigation Initial Management of Aneurysmal Subarachnoid Haemorrhage Aneurysm Treatment Management of Complications Outcome Arteriovenous Malformations Presentation Natural History Management Cavernous Malformations Dural arteriovenous fistulae Cerebral Venous Thrombosis Aortic and Spinal Cord Vascular Disorders Anatomy Cerebral Ischaemia Resulting from Aortic Disease Spinal Cord Ischaemia Resulting from Aortic Disease Aortic Disorders Neurological Complications of Cardiac Surgery Building a Stroke Team Life After Stroke Post-Stroke Rehabilitation Stroke: Overall Conclusions References Further Reading Chapter 9 Movement Disorders Introduction Hypokinetic (Parkinsonian) Syndromes Parkinson’s Disease Dominant Genetic Causes of Parkinson’s Disease Recessive Genetic Causes of Parkinson’s Disease Premotor Features of Parkinson’s Disease ‘Typical’ Motor Presentation of Parkinson’s Disease Non-Motor Features of Parkinson’s Disease Ancillary Investigations Medical Treatment of Parkinson’s Disease Surgery for Parkinson’s Disease Disease-Modifying Treatments for Parkinson’s Disease Dementia in Association With Lewy Body Pathology Atypical Forms of Parkinsonism Multisystem Atrophy Progressive Supranuclear Palsy Corticobasal Degeneration Ancillary Investigations to Distinguish Between Conditions Vascular Parkinsonism Ethnic or Region-Specific Parkinsonism Other Causes of Parkinsonism Tremor Essential Tremor Dystonic Tremor Tremor Associated with Neuropathy Fragile X-Associated Tremor/Ataxia Syndrome Holmes Tremor Palatal Tremor Orthostatic Tremor Enhanced Physiological Tremor Tremor Combined with Parkinsonism Functional Tremor Treatment Options for Tremor Dystonia Epidemiology of Dystonia Classifying Dystonia Isolated Dystonia Combined Dystonia Syndromes Myoclonus Dystonia Dystonia Combined with Other Neurological or Systemic Features Dystonia and Neurodegenerative Disorders Wilson’s Disease Paroxysmal Dyskinesias Investigation of Dystonia Treatment of Dystonia Surgery for Dystonia Chorea Assessment of Chorea Huntington’s Disease Clinical Features of Huntington’s Disease Juvenile Huntington’s Disease Differential Diagnosis of Huntington’s Disease Neuroacanthocytosis Sydenham’s Chorea Autoimmune Chorea Benign Hereditary Chorea Drug-Induced Chorea Drug Management of Chorea Tic Disorders Tourette’s Syndrome Myoclonus Physiological Myoclonus Essential Myoclonus Hereditary Essential Myoclonus Primary Progressive Myoclonus of Ageing Epileptic Myoclonus Symptomatic (Secondary) Myoclonus Progressive Myoclonic Epilepsy and Progressive Myoclonic Ataxia Myoclonus in Neurodegenerative Disorders Non-Progressive Myoclonic Encephalopathies Opsoclonus–Myoclonus Syndrome Metabolic Causes of Myoclonus Post-Anoxic Action Myoclonus (Lance–Adams Syndrome) Startle Syndromes Propriospinal Myoclonus Segmental Myoclonus Peripheral Myoclonus Functional Myoclonus Physiological Classification of Myoclonus Approach to Patients with Myoclonus Treatment of Myoclonus Other Movement Disorders Functional Movement Disorders Movement Disorders Associated with Dopamine Receptor Blockade or Dopamine Depletion Restless Legs Syndrome Painful Legs and Moving Toes Stiff-Person Spectrum Disorders Neurophysiological Assessment of Movement Disorders Neurophysiological Assessment of Tremor Neurophysiological Assessment of Myoclonus Focal Motor Seizures Cortical–Subcortical Myoclonus Subcortical–Nonsegmental Myoclonus Reticular or Brainstem Myoclonus Spinal Segmental Myoclonus Functional Myoclonus Neurophysiological Assessment of Dystonia Functional Dystonia Neurophysiological Assessment of Rigidity References Further Reading Chapter 10 Epilepsy and Related Disorders What is Epilepsy? The Epidemiology of Epilepsy The Incidence of Epilepsy Epilepsy Prevalence Risk Factors and Causes Natural History Comorbidities of Epilepsy The Spectrum of Seizures and Epilepsies Seizure Classification: International League Against Epilepsy Classification of Seizure Type Semiological Seizure Classification Classification of Epilepsies Focal Seizures Generalised Seizures Risk Factors for Epilepsy Genetic Causes Structural Causes Neuroscience of Epilepsy Focal Seizure Mechanisms Thalamocortical Seizures Epileptogenesis Electroencephalography Basic Principles Interictal Electroencephalography Abnormalities: Epileptiform and Non-Epileptiform Abnormalities and Normal Variants Ictal Electroencephalography Role of Electroencephalography and Diagnostic Yield Electroencephalography in Specific Situations/Syndromes Long-Term Electroencephalographic Monitoring Electroencephalography in the Acute/Intensive Care Setting Electroencephalography and Evaluation for Epilepsy Surgery Imaging Magnetic Resonance Imaging Functional Imaging in Epilepsy Future Directions in Neuroimaging Neuropsychology Neuropsychological Assessment and Treatments in Epilepsy The Role of Neuropsychology in Epilepsy Surgery Differential Diagnosis: First Seizure Clinic and Care Plan The Diagnostic Process Seizures, Epilepsy and its Mimics Preceding Medical History Care Plan Medical Treatment Strategies Principles of Treatment of Newly Diagnosed Epilepsy Diagnosis Risk of Seizure Recurrence Type, Timing and Frequency of Seizure Treatment Protocol for People with Chronic Epilepsy Choice and Details of Antiseizure Medications Treatment of People with Epilepsy in Remission Antiseizure Medication Emergency Drug Treatment of Prolonged Tonic-Clonic or Serial Seizures Management of Epilepsy in Learning Disability Prevalence of Epilepsy in People with a Learning Disability Assessment Diagnostic Difficulties Treatment of Epilepsy in People with a Learning Disability Dietary, Complementary and Psychological Therapies Dietary Therapy Psychological, Complementary and Alternative Therapies Epilepsy in the Acute Medical Unit Status Epilepticus Treatment of Non-Convulsive Status Epilepticus Treatment of Convulsive Status Epilepticus Stage of Early Status Epilepticus Stage of Established Status Epilepticus Stage of Refractory Status Epilepticus Stage of Super-Refractory Status Epilepticus Epilepsy Surgery The Pathways of Epilepsy Surgery Evaluation Scalp Video Electroencephalographic Telemetry Brain Imaging Intracranial Electroencephalography Neuropsychological Evaluation Psychiatric Evaluation Prediction of Outcome of Epilepsy Surgery The Range of Epilepsy Surgery The Neurological and Surgical Complications of Epilepsy Surgery The Consequences of Epilepsy Surgery Postoperative Follow-Up Epilepsy and Reproduction Contraception Teratogenicity Pregnancy Epilepsy in Later Life Causes of Seizures in Later Life Diagnosis of Epilepsy in Later Life Treatment Considerations Sleep and Epilepsy Epilepsy and Circadian Rhythms Differential Diagnosis Epilepsy and Sleep Disorders Driving and Epilepsy Driving Accident Statistics Driving Restrictions The Future Epilepsy and the Law The Role of the Healthcare Professional Epilepsy and Criminal Behaviour Antiseizure Medication and Aggression Demonstrating an Association Between Epileptic Seizures and Criminal Behaviour Epilepsy and Climate Change Premature Mortality and Sudden Unexpected Death Sudden Unexpected Death of a Person with Epilepsy Neurodegeneration and Epilepsy Neuropsychometry Neuroimaging Neuropathology Psychiatric Disorders of Epilepsy Mood and Anxiety Disorders Psychotic Disorders Suicide Treatment-Related Psychiatric Symptoms Dissociative Seizures Epidemiology of Dissociative Seizures Clinical Features of Dissociative Seizures Diagnosis and Investigations Cause and Comorbidities Treatment of Dissociative Seizures Prognosis Artificial Intelligence Approaches to Diagnosis and Treatment of Epilepsy Machine Learning and Artificial Intelligence Unsupervised Machine Learning Future Prospects for Artificial Intelligence and Epilepsy Databases Future Prospects for Epilepsy Management Prevention Diagnostic and Treatment Prospects The Future of Epilepsy References Further Reading Chapter 11 Cognitive Impairment and Dementia Introduction Epidemiology: Delirium, Dementia and Cognitive Impairment Cognitive Functions and their Clinical Syndromes Attention Memory Paramnesias Transient Global Amnesia Perception Hallucinations Knowledge Voluntary Action Speech and Language Literacy and Numeracy Executive Function Emotion Investigation of the Patient with Cognitive Impairment Basic Principles Initial Investigation Neuropsychometry Brain Imaging Electroencephalography Cerebrospinal Fluid Examination Additional Investigations Major Neurodegenerative Dementias Suspecting Dementia and Psychological Factors Alzheimer’s Disease Frontotemporal Dementia Dementia with Lewy Bodies and Parkinson’s Disease Dementia Dementia with Other Movement Disorders Prion Disease Introduction and Disease Biology Aetiological Categories and Classification of Human Prion Disease Sporadic Prion Disease Acquired Prion Diseases Inherited Prion Diseases Prevention and Treatment Vascular Dementia and Vascular Cognitive Impairment Clinical Features Investigations and Differential Diagnosis Management Dementia in Young Adults Potentially Reversible Causes of Dementia Epilepsy and Dementia Neoplasms and Other Space-Occupying Lesions Limbic Encephalitis Infective, Metabolic, Toxic and Other Causes of Dementia Controversial Entities Alcohol Common Autoimmune Disease Normal Pressure Hydrocephalus Management of Dementia: General Principles Risk Factor Management Addressing Comorbidities Nonpharmacological and Behavioural Management Assessing and Ensuring Safety Caring for the Carer Planning for the Future and End of Life Issues References Further Reading Chapter 12 Infection in the Nervous System Introduction Bacterial Meningitis Epidemiology Pathogenesis Clinical Presentation Investigation Management of Bacterial Meningitis Complications of Bacterial Meningitis Specific Causes of Bacterial Meningitis Focal Central Nervous System Infection Cerebral Abscess Subdural Empyema Intracranial Epidural Abscess Spinal Epidural Abscess Spinal Subdural Abscess Spinal Cord Intramedullary Abscess Infective Endocarditis Granulomatous Infections of the Nervous System Central Nervous System Tuberculosis and Tuberculous Meningitis Parenchymal Central Nervous System Tuberculosis Leprosy (Hansen’s Disease) Clinical Features Peripheral Nerve Involvement Borderline Leprosy Diagnosis Management Brucellosis (Undulant Fever) Management Spirochaete Central Nervous System Infections Syphilis Diagnosis Treatment Zoonoses Lyme Neuroborreliosis Leptospirosis Anthrax Psittacosis Cat Scratch Disease Infections of the Nervous System Associated with Toxin Production Diphtheria Botulism Tetanus Miscellaneous Infections of the Nervous System Mycoplasma Whipple’s Disease Melioidosis Tickborne Disease Ehrlichiosis Tularaemia Rickettsial Disease Viral Infections of the Nervous System Viral Meningitis Chronic and Recurrent Meningitis Mollaret’s Meningitis Encephalitis Herpes Simplex Encephalitis Other Causes of Encephalitis Arboviruses (Arthropod-Borne Viruses) Viral Haemorrhagic Fevers Fungal Infections Risk Factors Differential Diagnosis Yeasts Candida Species Dimorphic Fungi Filamentous Fungi (Moulds) Screening for Invasive Fungal Infection Parasitic Diseases of the Nervous System Neurocysticercosis Schistosomiasis (Bilharzia) Protozoa American Trypanosomiasis (Chagas Disease) African Trypanosomiasis (Sleeping Sickness) Toxoplasma gondii Malaria Central Nervous System Opportunistic Infections Infections in Transplant Recipients Neurological Disorders Resulting From HIV Opportunistic Infections in HIV Tuberculous Meningitis and Brain Abscess Cryptococcal Meningitis Cytomegalovirus Infection Herpes Simplex Virus Varicella Zoster Virus Progressive Multifocal Leukoencephalopathy Primary Central Nervous System Lymphoma Neurological Complications Resulting Directly From HIV HIV-associated Neurocognitive Disorders Stroke Aseptic Meningitis HIV-Related Vacuolar Myelopathy HIV-Associated Peripheral Neuropathy Immune Reconstitution Inflammatory Syndrome CD8+ Encephalitis HIV Compartmentalisation and Central Nervous System Viral Escape Treatment of HIV: Neurological Aspects Conclusions Acknowledgement References Further Reading Chapter 13 Cranial Nerve Disorders Introduction Olfactory (I) Nerve Symptoms Examination Causes of Anosmia Other Causes of Olfactory Dysfunction Trigeminal Nerve (V) Functional Anatomy Examination of the Trigeminal Nerve Nuclear Vth Nerve Lesions Peripheral Vth Nerve Lesions Trigeminal Neuralgia Herpes Zoster Ophthalmicus Facial Nerve (VII) Functional Anatomy Examination Supranuclear Facial Weakness Cerebellopontine Angle Syndrome Facial Canal Syndrome Lesions at and Distal to the Stylomastoid Foramen Bell’s Palsy Recurrent Facial Palsy Hemifacial Spasm Other Involuntary Facial Movements Glossopharyngeal Nerve (IX) Functional Anatomy Examination IXth Nerve Lesions, Peripheral and Central Glossopharyngeal Neuralgia Vagus Nerve (X) Functional Anatomy Causes and Localisation of Lesions Investigation Accessory Nerve (XI) Functional Anatomy Examination and Localisation of Lesions XIth Nerve Lesions Hypoglossal Nerve (XII) Functional Anatomy Localisation of Lesions Causes of XIIth Nerve Lesions Investigation Bulbar and Pseudobulbar Palsy Normal Swallowing Bulbar Palsy Pseudobulbar Palsy Dropped Head Syndrome Multiple Cranial Neuropathies References Further Reading Chapter 14 Spinal Column and Spinal Cord Disorders Introduction Diagnosis of Spinal Column and Spinal Cord Disorders Clinical Assessment of Spinal Disorders Skeletal Disorders Affecting the Spine Idiopathic Scoliosis Congenital Scoliosis Neuromuscular Scoliosis Kyphosis and Lordosis Miscellaneous Causes of Spinal Deformity Management of Spinal Deformity Degenerative Scoliosis Craniocervical Junction Anomalies Syringomyelia Spinal Dysraphism Rheumatological Disorders Affecting the Spine and Spinal Cord Spinal Trauma Acute Management of Spinal Injury Surgical Management of Spinal Fractures Non-Surgical Management of Spinal Trauma Degenerative Disease of the Spine Cervical Spine Thoracic Spine Lumbar Spine Surgical Treatment of Lumbar Disc Disease Low Back Pain and Degenerative Disc Disease References Further Reading Chapter 15 Disorders of the Motor Cells: The Motor Neuron Diseases Introduction Motor Neuron Disease Amyotrophic Lateral Sclerosis Investigations and Diagnosis Disease Monitoring and Staging Pathogenesis of Amyotrophic Lateral Sclerosis Progressive Bulbar Palsy Flail Arm/Leg Syndrome and Hirayama’s Disease Primary Lateral Sclerosis Progressive Muscular Atrophy Other Forms Management Telling the Patient the Diagnosis Principles of Management Pharmacotherapy and Clinical Trials Respiratory Management Management of Bulbar Weakness Dysphagia Nutrition Communication Limb Dysfunction Cognitive Impairment in Amyotrophic Lateral Sclerosis Psychological Factors Other Symptoms Terminal Care Spinal Muscular Atrophy Genetics and Pathogenesis Clinical Features Diagnosis Rare Forms of Spinal Muscular Atrophy and Differential Diagnosis Supportive Care and Treatments Kennedy’s Disease Investigation Management References Further Reading Chapter 16 Diseases of the Peripheral Nerves Introduction Peripheral Nerve Disorders Macroanatomy of the Peripheral Nerve Microanatomy of the Peripheral Nerve Peripheral Nerve Compartments Immunology Pathophysiology of the Peripheral Nerve Diseases of the Peripheral Nerve History Examination Scoring Neurophysiology Nerve Biopsy Inherited Neuropathies Charcot–Marie–Tooth Disease and Related Disorders An Approach to the Diagnosis of Charcot–Marie–Tooth Disease and Related Disorders Classification of Charcot–Marie–Tooth Disease Autosomal Dominant Charcot–Marie–Tooth Disease Type 1 Autosomal Dominant Charcot–Marie–Tooth Disease Type 2 Distal Hereditary Motor Neuropathy Hereditary Sensory Neuropathy Hereditary Neuralgic Amyotrophy Importance of a Genetic Diagnosis in Charcot–Marie–Tooth and Related Disorders Familial Amyloid Polyneuropathy Transthyretin-Related Familial Amyloid Polyneuropathy Apolipoprotein A-1 Related Familial Amyloid Polyneuropathy Gelsolin-Related Familial Amyloid Polyneuropathy Beta2 Microglobulin Familial Amyloid Polyneuropathy Acquired Neuropathies Inflammatory Neuropathies Acute Neuromuscular Weakness and the Inflammatory Neuropathies Guillain–Barré Syndrome and its Variants Variants of Guillain–Barré Syndrome Chronic Inflammatory Demyelinating Polyradiculoneuropathy Chronic Inflammatory Demyelinating Polyradiculoneuropathy Variants Paraproteinaemic Neuropathies Acquired Amyloid Neuropathy Vasculitic Neuropathies Primary Vasculitides Secondary Vasculitides Non-Systemic Vasculitides Other Acquired Peripheral Nerve Disorders Endocrine Disorders Toxic, Nutritional and Metabolic Peripheral Neuropathies Metabolic Neuropathies Critical Illness Neuromyopathy Small-Fibre Neuropathies Idiopathic Axonal Neuropathy Focal and Compressive Neuropathies Median Nerve Compression and Carpal Tunnel Syndrome Carpal Tunnel Syndrome Ulnar Nerve Compression Other Ulnar Neuropathies Common Peroneal Neuropathies Plexopathies Acute Brachial Neuritis References Further Reading Chapter 17 Disorders of the Neuromuscular Junction Introduction Anatomy Physiology of Neuromuscular Transmission Myasthenia Gravis Epidemiology Clinical Features Course and Prognosis Muscle-Specific Kinase Myasthenia Gravis Diagnosis Treatments Myasthenic Crisis Cholinergic Crisis Drugs that May Induce or Cause Deterioration of Myasthenia Gravis Vaccination in Myasthenia Gravis Myasthenia Gravis in Pregnancy Myasthenia Gravis in Older Patients Anaesthesia and Perioperative Care Newer Treatments and Trial Treatments Lambert–Eaton Myasthenic Syndrome Clinical Features Neurophysiology Serology Screening for Malignancy Treatment Congenital Myasthenic Syndromes Presynaptic Congenital Myasthenic Syndromes Synaptic Congenital Myasthenic Syndromes Post-Synaptic Congenital Myasthenic Syndromes Disorders of Glycosylation Treatment of Congenital Myasthenic Syndromes Botulism Nerve Agents/Organophosphate Insecticides References Further Reading Chapter 18 Disorders of Muscle Introduction Basic Muscle Biology Clinical Assessment of the Patient with Muscle Disease History Examination Investigation of Muscle Diseases Creatine Kinase Neurophysiology Muscle Magnetic Resonance Imaging Muscle Biopsy Metabolic Testing Genetic Testing Genetic Muscle Diseases Muscular Dystrophies Congenital Myopathies Skeletal Muscle Channelopathies Metabolic Muscle Disease Mitochondrial Respiratory Chain Diseases Mitochondrial Myopathies Mitochondrial Neuropathies Diagnosis Treatment Glycogenoses and Lipid Storage Disorders Acquired Muscle Diseases Inflammatory Myopathies Investigation of Inflammatory Myopathy Treatment of Idiopathic Inflammatory Myopathies Other Rare Inflammatory Myopathies Myopathies Associated with Malignancy Endocrine Myopathies Drugs and Myopathy Rhabdomyolysis References Further Reading Chapter 19 Multiple Sclerosis and Demyelinating Diseases Introduction Epidemiology Migration Studies Health Economics Aetiology Genetic Susceptibility Environmental Factors Pathology Pathophysiology Immunopathogenesis Clinical Course Relapsing Remitting Multiple Sclerosis Secondary Progressive Multiple Sclerosis Primary Progressive Multiple Sclerosis Disease Activity and Progression Natural History and Prognosis Multiple Sclerosis Variants Health and Lifestyle Factors Affecting Disease Course Comorbidity Diet Gut Microbiota Infections Sex Hormones Smoking Stress Vaccines Clinical Features Diagnosis Diagnostic Investigations Diagnostic Criteria Differential Diagnosis The Diagnostic Process Management Education and Support Management of Acute Relapses Disease-Modifying Therapy for Relapsing Remitting Multiple Sclerosis Moderate Efficacy Disease-Modifying Therapies High Efficacy Disease-Modifying Therapies Other Disease-Modifying Therapies for Relapsing Multiple Sclerosis Disease-Modifying Therapy for Progressive Multiple Sclerosis Autologous Haematopoietic Stem-Cell Transplantation Overall Approach to Disease-Modifying Therapy Emerging Immunomodulatory Therapies Neuroprotection, Remyelination and Neurorestoration Symptomatic Treatment and Rehabilitation Multiple Sclerosis in Special Populations Paediatric-Onset Multiple Sclerosis Late-Onset Multiple Sclerosis Pregnancy COVID-19 Pandemic Neuromyelitis Optica Spectrum Disorder Epidemiology Pathogenesis Clinical Features Investigations Diagnostic Criteria Course and Natural History Management Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease Pathology Clinical Features and Course Investigations Diagnostic Criteria Management Acute Disseminated Encephalomyelitis Clinical Features Pathogenesis Differential Diagnosis Investigations Clinical Course and Prognosis Management Acute Haemorrhagic Leukoencephalitis Clinical Features Investigations Management Prognosis Acute Disseminated Encephalomyelitis and Acute Haemorrhagic Leukoencephalitis Following COVID-19 Infection References Chapter 20 Neuro-Oncology Introduction Primary Brain Tumours Epidemiology Clinical Features Histogenesis of Intrinsic Brain Tumours Genetic Components in Oncogenesis Epigenetic Events in Oncogenesis Tumour Classification Based on DNA Methylation Profile New Classification of Central Nervous System Tumours Imaging of Brain Tumours Applications of Physiological Imaging Multidisciplinary Management of Brain Tumours Improving Outcome and Quality of Life Surgical Management General Principles Surgical Instrumentation and Methods Intraoperative Diagnostic Ultrasound Intraoperative Magnetic Resonance Imaging Neuroendoscopy Radiotherapy High-Grade Gliomas Pathology Imaging Surgery Radiotherapy Chemoradiation Chemotherapy Relapsed Glioblastoma Grade 3 Oligodendrogliomas Low-Grade Gliomas Pathology Imaging Surgery Radiotherapy Chemotherapy Low-Grade Glioneuronal Tumours Ganglioglioma and Gangliocytoma Central Neurocytoma Dysembryoplastic Neuroepithelial Tumour Molecular Genetics Choroid Plexus Tumours Meningiomas Pathology Imaging Surgery Radiotherapy Chemotherapy Brain Metastases Pathology Imaging Treatment of Brain Metastases Primary Spinal Cord Tumours Intramedullary Spinal Tumours Intradural Extramedullary Tumours Radiotherapy for Intrinsic Cord Tumours Chemotherapy for Intrinsic Cord Tumours Metastatic Spinal Cord Tumours Skull-Base Tumours Chordoma Chondrosarcoma Neurofibromatosis and Schwannomas Vestibular Schwannomas Skull-Base Meningiomas Pituitary Tumours Biological Behaviour Size Histology Functional Criteria Clinical Presentation Pituitary Apoplexy Drug Therapy and Surgery Radiotherapy Craniopharyngioma Primary Central Nervous System Lymphomas Imaging Clinical Presentation Diagnosis Treatment Prognosis Primitive Neuroepithelial Tumours and Medulloblastomas Pathology Molecular Genetics and Histogenesis of Medulloblastoma Imaging Clinical Features Treatment Pineal Region Tumours Pathology Clinical Features Treatment Germ Cell Tumours Optic Pathway Glioma Treatment Ependymomas Pathology Imaging Treatment Dermoid and Epidermoid Cysts Imaging Colloid Cysts, Rathke’s Pouch Tumours and Neurenteric Cysts Neurological Complications of Cancer and Cancer Treatments Neurological Complications of Cancer Direct Effects and Infiltration Metastases Leptomeningeal Metastasis Indirect Effects of Cancer Neurological Complications of Cancer Treatments Peripheral Neuropathy Encephalopathy Neurological Complications of Radiotherapy Other Complications of Cranial Radiotherapy Neurological Complications of Immune-Mediated Therapies Immune Checkpoint Inhibitor Neurotoxicity Chimeric Antigen Receptor T Cell Neurotoxicity Paraneoplastic Neurological Disorders Incidence and Prevalence Immunopathogenesis Clinical Features Paraneoplastic Cerebellar Degeneration Paraneoplastic Encephalomyelitis and Limbic Encephalitis Brainstem Encephalitis Paraneoplastic Encephalomyelitis with Rigidity Paraneoplastic Opsoclonus–Myoclonus Paraneoplastic Retinal Degeneration Necrotising Myelopathy Motor Neuron Syndromes Paraneoplastic Sensory Neuronopathy Paraneoplastic Neuropathies Sensory and Sensorimotor Neuropathy Acute Inflammatory Demyelinating Polyradiculoneuropathy Motor Neuropathy Vasculitic Neuropathy Autonomic Neuropathy Neuromyotonia Lambert–Eaton Myasthenic Syndrome Myasthenia Gravis Polymyositis and Dermatomyositis Acute Necrotising Myopathy References Further Reading Appendix 20.1 World Health Organization 2021 Classification of Central Nervous System Tumours Chapter 21 Headache Introduction Evaluation of the Patient with Headache Headache History Taking Examination of the Patient with Headache Red Flags for Secondary Headaches Investigation of the Patient with Headache Secondary Headaches Medication Overuse Headache Headache Caused by Vascular Disorders Headache Caused by Disturbance of Intracranial Pressure Primary Headaches Anatomy and Physiology of Headache Migraine Tension-Type Headache Trigeminal Autonomic Cephalalgias Cluster Headache Short-Lasting Unilateral Neuralgiform Headache Attacks Hemicrania Continua Other Primary Headaches Chronic Daily Headache References Further Reading Chapter 22 Neuro-Ophthalmology Introduction Unilateral Visual Failure due to Optic Neuropathy History Examination Bilateral Visual Failure Special Investigations in Neuro-Ophthalmology Optical Coherence Tomography and Angiography Ultrasound and Duplex Ultrasonography Clinical Electrophysiology of the Eye Visual Field Testing Fundus Fluorescein Angiography Optic Neuropathy Inflammatory Optic Neuropathies (Optic Neuritides) Optic Neuritis Associated with Multiple Sclerosis Optic Neuritis Associated with Neuromyelitis Optica Spectrum Disorder Optic Neuritis: Chronic Relapsing Inflammatory Optic Neuropathy Optic Neuritis: Infective Disorders Optic Neuritis: Sarcoid-Related Optic Neuropathy Optic Neuritis: Neuroretinitis Optic Neuritis: Optic Perineuritis Ischaemic Ocular Syndromes Classification of Ischaemic Syndromes Central and Branch Retinal Artery Occlusion Central and Branch Retinal Vein Occlusion Non-Arteritic Anterior Ischaemic Optic Neuropathy Anterior Ischaemic Optic Neuropathy in Giant-Cell Arteritis and Other Vasculitides Posterior Ischaemic Optic Neuropathy Chronic Ocular Ischaemic Syndromes Tumours Affecting the Optic Nerve Compressive or Infiltrative Optic Neuropathy Primary Optic Nerve Sheath Meningiomas Other Forms of Meningiomas Optic and Optochiasmal Glioma Hereditary Optic Neuropathies Autosomal Dominant Optic Atrophy Leber’s Hereditary Optic Neuropathy Toxic and Nutritional Optic Neuropathies Traumatic Optic Neuropathy Radiation-Induced Optic Neuropathy Swollen Optic Disc Specific Optic Disc Anomalies Papilloedema Idiopathic Intracranial Hypertension Ocular Involvement in Other Neurological Disease Uveomeningeal Syndromes Neoplasia The Phakomatoses in Neuro-Ophthalmology Neurofibromatosis Types 1 and 2 Von Hippel–Lindau Disease Tuberous Sclerosis Sturge–Weber Syndrome (Encephalotrigeminal Angiomatosis) Abnormalities of Eye Movements Diplopia Orbital Disease Thyroid Ophthalmopathy Orbital Inflammatory Syndromes Cavernous Sinus Thrombosis Caroticocavernous Fistula Myopathy Mitochondrial Disease Oculopharyngeal Dystrophy Neuromuscular Junction Abnormalities Cranial Nerve Palsies Oculomotor Nerve (IIIrd Nerve Palsy) Abducens (V1th) Nerve Palsy Trochlear (IVth) Nerve Palsy Painful and Combined Ophthalmoplegia Central Disorders of Eye Movements Saccadic Eye Movements Supranuclear Gaze Palsy Horizontal Gaze Palsy Vertical Gaze Palsy Oculogyric Crises Internuclear Ophthalmoplegia One and a Half Syndrome Wall-Eyed Bilateral Internuclear Ophthalmoplegia Internuclear Ophthalmoplegia Of Abduction Disconjugate Vertical Gaze Palsy Skew Deviation and Ocular Tilt Reaction Opsoclonus Nystagmus Horizontal Nystagmus Nystagmus in Normal Individuals Jerk Nystagmus Pendular Nystagmus Nystagmus in Childhood Pendular Nystagmus from Visual Loss Spasmus Nutans Monocular Nystagmus Vestibular Jerk Nystagmus Gaze-Evoked or Gaze Paretic Jerk Nystagmus Caloric Nystagmus Torsional Nystagmus Central Vestibular Horizontal Nystagmus Vertical and Other Forms of Nystagmus Nystagmus in Oculopalatal Tremor Seesaw Nystagmus Oculomasticatory Myorhythmia Periodic Alternating Nystagmus Convergence–Retraction Nystagmus in Parinaud Syndrome Voluntary Nystagmus Eyelid Nystagmus Medical Treatment of Nystagmus Chiasmal and Retrochiasmal Visual Pathways Chiasmal Disease Homonymous Hemianopia Optic Tract Lateral Geniculate Nucleus Optic Radiation Visual Cortex Bilateral Homonymous Hemianopia Blindsight and Statokinetic Dissociation Visual Association Areas (Extrastriate Cortex Areas V2–6) Disorders of Higher Visual Function Visual Hallucinations Visual Hallucinations Associated with Impaired Vision (Charles Bonnet Syndrome) Peduncular Hallucinosis Polyopia Palinopsia Other Disorders of Visual Perception Disorders of Colour Vision Cerebral Metamorphopsia Visual Simultanagnosia Cortical Visual Impairment Neglect Alexia Abnormalities of the Pupil Disorders of the Light Reflex Complete Afferent Pupillary Defect Relative Afferent Pupillary Defect Central (Midbrain) Lesions of the Light Reflex Efferent Parasympathetic Defects of the Light Reflex Holmes–Adie Syndrome Disorders of the Sympathetic Nervous Supply to the Pupil References Chapter 23 Neuro-Otology: Dizziness, Balance and Hearing PART I DIZZINESS, VERTIGO, AND IMBALANCE Introduction Epidemiology Anatomy and Physiology Labyrinths and the Vestibular Nerve The Thalamus, Basal Ganglia and Cerebrum The Blood Supply Three-Dimensional Spatial Orientation Neuro-Otological Assessment Clinical Examination The Clinical Presentation of Balance Disorders Commonly Used Vestibular Investigations Clinical Disorders Benign Paroxysmal Positional Vertigo Unilateral Peripheral Vestibulopathy Bilateral Peripheral Vestibulopathy Ménière’s Disease Vestibular Migraine Motion Sickness Vestibular Paroxysmia Acute (Central) Vestibular Syndrome Chronic Vestibular Disorders Dizziness in the Elderly Management of Vestibular Disorders Drug Treatment Symptomatic Treatment of Acute Vestibular Symptoms Specific Treatment of Vestibular Disorders Treatment of Chronic Peripheral Vertigo PART II HEARING DISORDERS Anatomy and Physiology Definitions of Hearing Loss Types of Hearing Impairment Clinical Examination of the Ear and Hearing Otoscopy Auricle (Pinna) Tympanic Membrane and Middle Ear Audiological Investigations Baseline Audiometric Tests Aetiology of Hearing Loss Conductive Hearing Loss Sensorineural Hearing Loss Auditory Processing Disorders Aetiology of Auditory Processing Disorders Management of Auditory Disorders Hearing Aids Conductive Hearing Loss Sensorineural Hearing Loss VIIIth Nerve Disorders Amplification and Rehabilitation Strategies Rehabilitation of Auditory Processing Disorders Acknowledgements References Further Reading Chapter 24 Cerebellar Ataxias and Related Conditions Introduction Approach to the Patient with Ataxia Symptoms Physical Signs Additional Signs Investigations The Ataxic Disorders Inherited Ataxia Syndromes Other Metabolic Causes of Ataxia Diagnostic Testing Treatment Genetic Forms of Episodic Ataxia X-Linked Ataxia Syndromes Mitochondrial Ataxia Syndromes Next-Generation Gene Sequencing Acquired Ataxia Syndromes Infective Disease Inflammatory Disease Vascular Disease Vascular Anomalies Acquired Metabolic Disorders Toxins and Physical Agents Autoimmune and Paraneoplastic Cerebellar Degeneration Late-Onset Cerebellar Degenerations General Summary Reference Further Reading Chapter 25 Restorative Neurology, Rehabilitation and Brain Injury Introduction The Nature of Disability and its Optimal Management Key Aspects of Multidimensional Rehabilitation Natural Histories of Neurological Damage Resources, Integrated Care Pathways, Effectiveness and Evidence Base Specialist Neurorehabilitation Services Neural Reorganisation and Restoration Treatment of Neurological Impairments and Functional Limitations Restorative and Compensatory Approaches, Skill Learning and Task-related Training Physical Therapeutic Interventions for Motor Disorders Language Disorders: Speech and Language Therapy and Communication Aids Cognitive Disorders Psychological Disorders Neurobehavioural Disorders Family Wellbeing Telerehabilitation Vocational Rehabilitation Barriers to Work Vocational Rehabilitation Resources Approaches to Vocational Rehabilitation Vocational Rehabilitation Interventions Future Priorities Medical Treatments for Specific Problems Spasticity Pharmacological Management of Multiple Sclerosis-Related Walking Impairment Ataxia Pain Bladder, Bowel and Sexual Dysfunction Fatigue Dysphagia Neuropsychiatric Problems Single-Incident Brain Injury Stroke Traumatic Brain Injury Mild Traumatic Brain Injury, Sports Concussion and Minor Head Injury Sports-Related Head Injury and Mild Brain Injury Moderate to Severe Traumatic Brain Injury Clinical Diagnostics and Treatment Over Time Post Injury Medical Treatments Over Time Post Injury Effectiveness of Modules in the Traumatic Brain Injury Care Pathway Prolonged Disorders of Consciousness Mechanism of Brain Injury and Prognostication Definitions of Disorders of Consciousness Subcategorisation of the Minimally Conscious State Emergence from a Minimally Conscious State Cognitive Motor Dissociation Prognostication in Prolonged Disorder of Consciousness Factors Affecting the Probability of Recovery of Consciousness Pathophysiology of Prolonged Disorder of Consciousness Interventional Programmes for Patients with Prolonged Disorder of Consciousness Service Delivery Organisational Behaviours and Interdisciplinary Assessment Assessment Goal Setting Outcome Measurement Indices of Outcome Service Quality Conclusions References Further Reading Chapter 26 Toxic, Metabolic and Physical Insults to the Nervous System Introduction Neurological Disorders Associated with Exposure to Toxic Substances Organic Solvents Nitrous Oxide Organophosphates and Carbamates Carbon Monoxide Occupational Health Aspects Marine Toxins Ciguatera Tetrodotoxin Scombroid Shellfish Other Biological Toxins Snake Venom Spider Toxins Scorpion Toxin Ticks Fungal Poisons Lathyrism Konzo Subacute Myelo-Optic Neuropathy Tropical Ataxic Myeloneuropathy (Strachan Syndrome) Radiation-Induced Neurological Disease Lightning and Electrical Damage to the Nervous System Lightning: Initiation and Pattern of Contact Electrical Injuries: High and Low-Voltage Nervous System Complications of Lightning and Electrical Injury Non-Nervous System Complications of Lightning and Electrical Injury Heat Stroke Hypothermia and Non-Freezing Cold Injury Diving Decompression Sickness Arterial and Venous Gas Emboli Altitude Medicine Acute Mountain Sickness and Cerebral Oedema High-Altitude Cerebral Oedema High-Altitude Pulmonary Oedema Chemical and Biological Weapons Vitamin and Mineral Deficiencies and Toxicity Vitamin A Vitamin B1 (Thiamine) Vitamin B2 (Riboflavin) Vitamin B3, Niacin (Nicotinic Acid and Nicotinamide) Vitamin B6 (Pyridoxine) Vitamin B12 Deficiency (Chapter 14) Subacute Combined Degeneration of the Cord Folate Deficiency Vitamin C (Ascorbic Acid) Vitamin D Vitamin E Vitamin K Other Micronutrients Alcohol Abuse Metabolism of Alcohol Effects of Acute Intoxication Effects of Alcohol Substitutes Withdrawal Syndromes Chronic Disorders Associated with Prolonged Alcohol Abuse Other Neurological Complications of Alcohol Abuse Other Deficiency States Associated with Neurological Manifestations Copper Deficiency Magnesium Deficiency Drugs of Abuse Epidemiology Opioids Sedatives/Hypnotics Psychostimulants Conditions Caused by Stimulant Abuse Hallucinogens Cannabis and Synthetic Cannabinoids Inhalants/Solvents Anticholinergics Neurological Complications of Drug Abuse Investigation of Suspected Substance Abuse Adverse Reactions to Drugs Toxic Leukoencephalopathy Specific Agents Biological Response Modifiers Severe Generalised Drug Reactions Malignant Hyperthermia Neuroleptic Malignant Syndrome Serotonin Syndrome Malignant Catatonia and Opistotonus Tyramine Cheese Reaction Anticholinergic Syndrome Caused by Medication Toxicity References Further Reading Appendix 26.1 Drugs Associated with Toxins Drugs That May Provoke Seizures Drugs That May Cause Headache Drugs Associated with Confusional State Drugs Associated with Encephalopathy Drugs That May Be Associated with Memory Disturbance Drugs That May Be Associated with Toxic Leukoencephalopathy Drug-Induced Movement Disorders Drugs That May Cause Disorders of the Neuromuscular Junction Drugs That May Cause Muscle Disease Chapter 27 Inherited Disorders of Metabolism Introduction Porphyria Acute Intermittent Porphyria Disorders of Amino Acid Metabolism Phenylketonuria Maternal Phenylketonuria Syndrome Organic Acidaemias Maple Syrup Urine Disease Isovaleric Acidaemia Propionic and Methylmalonic Acidaemias Disorders of Homocysteine Metabolism Transsulfuration Defects Defects in Intracellular Cobalamin Metabolism Remethylation Defects Urea Cycle Disorders Arginase Deficiency Disorders of Carbohydrate Metabolism Glycogen Storage Diseases Hepatic Involvement in Glycogen Storage Diseases Skeletal/Cardiac Muscle Involvement in Glycogen Storage Diseases Central Nervous System Involvement in Glycogen Storage Diseases Fatty Acid Oxidation Defects Neurotransmitter Disorders Clinical Presentations Laboratory Diagnosis Lysosomal Storage Disorders Glycosphingolipidoses Primary Mitochondrial Diseases Genetics Clinical Manifestations Diagnosis Adult-Onset Inherited Leukodystrophies X-Linked Adrenoleukodystrophy Krabbe Disease Cerebrotendinous Xanthomatosis Metachromatic Leukodystrophy Hereditary Diffuse Leukoencephalopathy with Neuroaxonal Spheroids Alexander Disease Vanishing White Matter Disease Pelizaeus–Merzbacher Disease and Pelizaeus–Merzbacher-Like Disease Peroxisomal Disorders Refsum Disease 2-Methylacyl-CoA Racemase Deficiency Disorders of Phospholipid and Glycosphingolipid Biosynthesis Disorders of Phospholipid Synthesis Disorders of Glycosphingolipid Synthesis Inherited Disorders of Metabolism Associated with Epilepsy An Overview of Dietary Principles for Inherited Disorders of Metabolism Acute Neurological Emergencies Caused by Inherited Metabolic Disease References Further Reading Chapter 28 Disorders of Consciousness and Intensive Care Neurology Consciousness States of Impaired Consciousness Coma Causes of Coma Initial Assessment and Management of Coma Distinction of Metabolic and Toxic Coma from Structural Coma Outcome From Coma Locked-In Syndrome Unresponsive Wakefulness Syndrome Minimally Conscious/Aware States Diagnosing Death by Neurological Criteria Apnoea Test Ventilatory Failure Associated with Neurological Disease Neurological Indications for Tracheal Intubation and Mechanical Ventilation Mechanical Ventilation Weaning Conditions Requiring Neurointensive Care Support Intracranial Pressure Cerebral Herniation Herniation of the Temporal Lobe Through the Tentorium Central Herniation of the Brain Stem Subfalcine Herniation Upward Transtentorial Herniation Tonsillar Herniation Indications for Intracranial Pressure Monitoring Pressure Reactivity Index Multimodal Neuromonitoring Optic Nerve Sheath Diameter Transcranial Doppler and Transcranial Colour Doppler Near-Infrared Spectroscopy Brain Tissue Oxygenation Jugular Venous Oxygen Saturation Cerebral Microdialysis Electroencephalography and Processed Electroencephalography Automated Pupillometry Traumatic Brain Injury and the Management of Raised Intracranial Pressure Primary Injury Secondary Injury Skull Fractures Diffuse Axonal Injury Haemorrhagic Contusions and Lacerations Extradural Haematoma Subdural Haematoma Traumatic Subarachnoid Haemorrhage Intraventricular Haemorrhage Intracerebral Haematoma Intensive Care Management of Traumatic Brain Injury Conditions of Particular Concern on Neurological Critical Care Artery of Percheron Stroke Subarachnoid Haemorrhage Seizures Status Epilepticus Encephalitis Limbic Encephalitis Associated with Neuronal Surface Antigens Brainstem Encephalitis Morvan Syndrome Encephalitis Due to N-Methyl-D-Aspartate Receptor (NMDAR) Antibodies Other Immune-mediated Encephalopathies Metabolic Encephalopathy Septic Encephalopathy Uraemic Encephalopathy Hepatic Encephalopathy Hypernatraemia Hyponatraemia Posterior Reversible Encephalopathy Syndrome Hypoxic–Ischaemic Brain Injury Prognostic Factors Following Cardiac Arrest Investigations Prognosis of Brain Injury After Hypoxic–Ischaemic Brain Injury Clinical Examination Longer-Term Complications of Hypoxic–Ischaemic Brain Injury Carbon Monoxide Poisoning Delayed Post-Hypoxic Encephalopathy Movement Disorders Movement Disorder Emergencies Status Dystonicus Stiff-Person Syndromes and Progressive Encephalopathy with Rigidity and Myoclonus Acute Cervical Cord Disorders Anterior Horn Cell Disease Neuromuscular Disease Guillain–Barré Syndrome on the Intensive Care Unit Miller Fisher Syndrome Bickerstaff’s Brainstem Encephalitis Acute Intermittent Porphyria Phrenic Nerve Neuropathies Neuromuscular Junction Disease Muscle Disease Intensive Care Unit Presentation of Chronic Neuromuscular Disease Intensive Care Unit Acquired Weakness General Medical Care on the Neurological Intensive Care Unit Nosocomial Infection and Infection Surveillance Anticoagulation Delirium Agitation Pain Sleep Communication Cognition Mental Health Family Support Follow-Up End of Life Issues on Intensive Care References Further Reading Appendix 28.1 Causes of Coma With Intact Brainstem Function, without Meningism and without Lateralising Signs Typical Causes of Coma with Meningism, with or without Brainstem Signs and with or without Lateralising Signs Typical Causes of Coma with Intact Brainstem Function and Asymmetrical Lateralising Signs with or without Meningism Causes of Coma with Intact Brainstem Function and Symmetrical Lateralising Signs with or without Meningism Causes of Coma with Signs of Focal Brainstem Dysfunction (with or without Meningism, with or without Lateralising Signs) Chapter 29 Disorders of Sleep Structure of Normal Sleep Rapid Eye Movement Sleep Patterns of Sleep Regulation of Wakefulness and Sleep Functions of Sleep Sleep and Breathing Classification of Sleep Disorders Insomnia Management Sleep-Related Breathing Disorders Obstructive Sleep Apnoea/Hypopnoea Syndrome Central Sleep Apnoea Central Disorders of Hypersomnolence Narcolepsy Cataplexy Hypnogogic/Hypnopompic Hallucinations Sleep Paralysis and Automatic Behaviours Pathophysiology of Narcolepsy Primary (Idiopathic) Hypersomnia Recurrent Hypersomnia Idiopathic Recurring Stupor Excessive Daytime Somnolence in Neurological Disease Circadian Rhythm Disorders Delayed Sleep–Wake Phase Disorder Advanced Sleep–Wake Phase Disorder Non-24-Hour Sleep–Wake Rhythm Disorder Shift Work and Jet Lag Parasomnias Non-Rapid Eye Movement Parasomnias Rapid Eye Movement Sleep Disorders Other Forms of Parasomnia Sleep-Related Movement Disorders Restless Legs Syndrome Periodic Limb Movements of Sleep Rhythmic Movement Disorders Sleep Starts Sleep Disturbances in Neurological Disorders Extrapyramidal Disease Epilepsy Syndromes Associated with Sleep Multiple Sclerosis Dementia Traumatic Brain Injury and Sleep References Further Reading Chapter 30 Neuropsychiatry Introduction The Mental State Examination Appearance and Behaviour Speech Mood and Affect Thoughts Perceptions Cognition Insight Formulation Identifying and Managing Risk Secondary to Abnormal Mental States Agitation and Aggression Lack of Insight Suicidal Ideation/Suicidal Behaviour Legal Issues: Use of Mental Health and Capacity Acts Psychiatric Symptoms Commonly Seen in Neurological Disorders Personality Disorder and Personality Change Obsessions, Compulsions and Impulsivity Anxiety Mood Apathy Pseudodementia Psychosis Catatonia Pseudohallucinations, Déjà Vu, Depersonalisation and Derealisation Functional Neurological Symptoms Terminology Diagnosis Specific Types of Functional Neurological Symptoms Functional Seizures Fixed Dystonia Treatment of Functional Neurological Symptoms Dissociative Disorders Dissociative Amnesia Dissociative Fugue Depersonalisation/Derealisation Disorder The Neuropsychiatry of Movement Disorders Parkinson’s Disease Iatrogenic Neuropsychiatric Symptoms in Parkinson’s Disease Other Neurodegenerative Movement Disorders Neuropsychiatric Symptoms in White Matter Disorders Autoimmune Encephalitis Neuropsychiatric Presentations of N-Methyl-D-Aspartate Receptor Encephalitis The Significance of Neuronal Surface Antibodies in Chronic Psychotic Illness Management of Psychosis in Autoimmune Encephalitis Neuropsychiatric Aspects of Inherited Metabolic Disorders References Further Reading Chapter 31 Pain in Neurological Disorders Introduction Definitions and Taxonomy Neuropathic Pain Neuroanatomy of Pain Pathways Peripheral Central Neurophysiology of Neuropathic Pain Pathways Ectopic Discharges Central Sensitisation Supraspinal Influences Role of Inflammation and the Immune System Neurological Diseases Presenting with Pain Peripheral Nervous System Diseases Small-Fibre Neuropathies Large-Fibre Neuropathies Brachial Plexopathies Specific Conditions of the Peripheral Nervous System Central Nervous System Diseases Spinal Cord Brain Orthopaedic Conditions that may Present to a Neurologist Glomus Tumour Osteoid Osteoma Conditions of Uncertain Aetiology that may Present to Neurology Complex Regional Pain Syndrome Fibromyalgia Burning Mouth Syndrome Assessment of the Patient with Painful Neurological Disease Treatment History Physical Examination Management of Pain Systemic Drug Treatments Topical Treatments Botulinum Toxin Intrathecal Drugs Biological Therapies Neuroablative Procedures Neurostimulation Procedures Other Physical Methods of Treatment Acupuncture Behavioural and Psychological Approaches to Management of Chronic Pain Conclusions Acknowledgements References Chapter 32 Autonomic Aspects of Neurology Introduction Classification of Autonomic Dysfunction Clinical Approach to Autonomic Disorders Clinical History Clinical Features in Patients with Autonomic Failure Manifestations of Orthostatic Hypotension Hypertension Heart Rate Disturbances Sudomotor Dysfunction and Thermodysregulation Gastrointestinal and Secretomotor Dysfunction Genitourinary Dysfunction Ocular, Pupillary and Lacrimal Glands Dysfunction Facial and Peripheral Vascular Changes Psychological and Psychiatric Disturbances Clinical Features in Patients with Autonomic Intermittent Disorders Autonomic Mediated Syncope Orthostatic Intolerance with Posturally Induced Tachycardia Facial and Peripheral Vascular Changes Sudomotor Dysfunction in Intermittent Autonomic Disorders Gastrointestinal Dysfunction Genitourinary Dysfunction Psychological Disturbances Clinical Examination and Diagnostic Approach to Autonomic Disorders Investigations Autonomic Testing and Laboratory Tests in Patients with Autonomic Failure Autonomic Testing in Patients with Intermittent Autonomic Disorders Sudomotor Function Testing Punch Skin Biopsy Management of Autonomic Disorders Treatment of Orthostatic Hypotension Hypertension Diagnostic and Treatment Approach to Autoimmune Autonomic Ganglionopathy Autonomic Mediated Syncope Postural Tachycardia Syndrome Sudomotor Disorders Gastrointestinal and Secretomotor System Urinary Tract Sexual Function and the Reproductive System Respiratory System Eye and Lacrimal Glands References Further Reading Chapter 33 Uro-Neurology Introduction The Lower Urinary Tract and its Neurological Control Lower Urinary Tract Dysfunction Following Neurological Disease Cortical Disease Cerebrovascular Disease Dementia Parkinson’s Disease Multiple System Atrophy Hereditary Ataxias Brainstem Lesions Spinal Cord Disease Multiple Sclerosis and Other Demyelinating Diseases Other (Non-Traumatic) Spinal Cord Diseases Sacral and Infrasacral Lesions Conus or Cauda Equina Lesions Diabetic Neuropathy Autonomic Disorders Other Neuropathies Pelvic Nerve Injury Myotonic Dystrophy Urinary Retention Fowler Syndrome Management of Lower Urinary Tract Dysfunction Management of Storage Dysfunction Medications Botulinum Toxin Neuromodulation Invasive Surgery Long-Term Follow-Up Management of Voiding Dysfunction Sexual Functions and Their Neurological Control Physiology Sexual Dysfunction Following Neurological Disease Management of Sexual Dysfunction References Further Reading Chapter 34 Systemic Conditions and Neurology Introduction Spinal Cord Ischaemia Resulting from Aortic Disease Aortic Surgery Aortic Dissection Fibrocartilaginous Embolism Systemic Hypotension Neurological Complications of Cardiac Surgery Neurological Complications of Acquired Cardiac Disease Heart Failure Cardiac Embolism Cardiomyopathies Atrial Myxoma Cystic Fibrosis Endocrine Conditions Thyroid Disorders Diabetes Mellitus Pituitary Disorders Parathyroid Disorders Adrenal Disorders Electrolyte Disturbances Acid-Base Disorders Haematological Disorders Anaemia Proliferative Conditions Histiocytic Disorders Langerhans Cell Histiocytosis Erdheim–Chester Disease Rosai–Dorfman Disease Haemophagocytic Lymphohistiocytosis Autoinflammatory Syndromes Familial Mediterranean Fever Cryopyrin-Associated Periodic Syndromes Deficiency of Adenosine Deaminase 2 Gastrointestinal Disorders Hepatic Encephalopathy Neurological Complications of Nutritional Disease Malabsorption Gluten Sensitivity Inflammatory Bowel Disease Renal Disease Conditions Affecting Both Renal and Neurological Function Neurological Consequences of Renal Disease and its Treatment Neurological Aspects of Organ Transplantation Central Nervous System Infections Neurological Sequelae of Transplantation Complications Related to Specific Allograft Transplantation Neurological Involvement in Systemic Vasculitides and Related Disorders Polyarteritis Nodosa Granulomatosis with Polyangiitis Eosinophilic Granulomatosis with Polyangiitis Giant-Cell Arteritis Isolated Cerebral Angiitis Rheumatoid Arthritis Seronegative Spondyloarthropathies Systemic Lupus Erythematosus Antiphospholipid Syndrome Sjögren Syndrome Sarcoidosis Behçet Syndrome Immunoglobulin G4-Related Disease (IgG4-RD) Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) Neurocutaneous Syndromes Neurofibromatosis Type 1 Neurofibromatosis Type 2 Xeroderma Pigmentosa Tuberous Sclerosis Von Hippel–Lindau Disease Ataxia Telangiectasia Sturge–Weber Syndrome Cerebrotendinous Xanthomatosis Neurological Aspects of Pregnancy Cerebrovascular Disease in Pregnancy Cerebrovascular Disorders in Pregnancy Pregnancy and Other Neurological Diseases Complications of Obstetric Anaesthesia Frailty in Neurological Disease Acknowledgements References Further Reading Chapter 35 Palliative Care in Neurology What is Palliative Care? Core Palliative Care Issues Symptomatic Management Communication Family Members and Caregivers Social and Economic Needs of Patients and Caregivers Psychosocial Support Religious, Spiritual and Existential Care Coordination of Care Triggers for Referral to Palliative Care Surrogate Decision Making Advance Care Planning and Advance Decisions Cardiopulmonary Resuscitation Palliative Care in Neurology Symptom Management Physical Symptoms Specific Neurological Conditions Needing Palliative Care Support Parkinson’s Disease and Other Movement Disorders Multiple Sclerosis Dementia Motor Neuron Disease Neuromuscular Disease Brain Tumours Stroke Critical Care Neurology Coronavirus End of Life Care Conclusion References Index EULA
