
Neurology
Description
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A fully updated and authoritative neurology resource
The Queen Square Textbook has established itself as a favourite companion to clinical neurosciences training and teaching around the world, whilst retaining its role as an invaluable reference guide for physicians and other healthcare professionals working in neurology, general medicine and related specialties.
The book continues to reflect the core values essential to the practice of clinical neurology in the 21st century. The third edition has been extensively revised and updated to take account of the rapid pace of progress in the neurosciences and patient care. Contemporary neurology has been changed by the COVID-19 pandemic, the climate emergency and the growing inequalities in healthcare resources. The new edition has been extensively revised to reflect these challenges and affords a greater emphasis on management and rehabilitation whilst continuing to reflect the coherence of a text produced from a single, closely-knit, centre of excellence.
Highlights of the new edition include:
* An updated approach to clinical examination, decision-making and diagnosis
* New developments in neuroimmunology, pathology and genetics
* Neuropalliative care
* Ethical and legal issues in clinical neurology
* The latest developments in the understanding and management of stroke, movement disorders, epilepsy, cognitive impairment, multiple sclerosis, infections, myelopathy, anterior horn cell disease, disorders of nerve and muscle, neuro-oncology, neurological disorders of hearing, balance and vision, and the neurological care of critical illness, sleep, neuropsychiatry, pain, autonomic and urological disorders.
* An emphasis on treatment and rehabilitation of the person with a neurological disease
The new edition marks a significant transition to reflect contemporary neurological practice during uncertain times. It mirrors the enormous changes in investigation, diagnosis and treatment that have occurred in recent years whilst maintaining the underlying principle that we do not treat diagnoses but, rather, we care for people affected by neurological disease.
More details
Other editions
Additional editions

Previous edition

Persons
Dimitri Kullmann, FMedSci, FRS
David Werring, PhD, FRCP, FESO
Michael Zandi, PhD, FRCP
Content
- 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
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