Оглавление
Charles H. Clarke. Neurology
Table of Contents
List of Tables
List of Illustrations
Guide
Pages
Neurology: A Clinical Handbook
Preface
Foreword
1 Neurology Worldwide : Public Health and Essential Neuro‐epidemiology
Basic Data
Practical Neurology
Causation
Genetic
Genetic and Environmental
Evident and Preventable
Mortality, Life Expectancy and Quality of Life
Quality of Life
Other Important Measures
Burden of Illness
Cost of Illness Studies
Stigma
Costs and Impact
Treatment Gaps
Improvements
Acknowledgements
References
Further Reading and Information
2 Movement, Sensation and The Silent Brain
ABC of Movement: Cortical, Extrapyramidal and Cerebellar Function
Cortex: Movement Force, Direction and Synergy
Pyramidal System Anatomy
Extrapyramidal System and Basal Ganglia Region
Basal Ganglia Circuits
Cerebellar System
Afferent and Efferent Cerebellar Pathways
Sensory Pathways
Sensory Pathways in the Cord and Brain
Dorsal Root Ganglia
Posterior Column→Medial Lemniscus Pathway
Spinothalamic Pathway
The Silent Brain
Brainstem
Reticular Formation
Gate Control: Sensory Modulation
Limbic System and Hippocampus
Insula and Cingulate Cortex
Amygdala and Kindling
Nucleus Accumbens, Septal Region and Basal Forebrain
Thalamus
Hypothalamus and Pituitary. Hypothalamic Region
Neuroendocrine Cells
Sympathetic and Parasympathetic Hypothalamic Activity (See Chapter 24) Water Intake, Thirst, Appetite and Satiety
Mood, Sexual Arousal, Wakefulness and Memory
Anterior/Posterior Pituitary Axes and Circumventricular Organs
Acknowledgements
Further Reading and Information
3 Aetiologies and Mechanisms: Genetics, Immunology and Ion Channels
Genetics
Essential Embryology of the Spine
Genetic Control of Spinal Development
Chromosomal Abnormalities, Repeat Expansions and Mutations
Autosomal Dominant Inheritance
Autosomal Recessive Inheritance
X‐Linked Inheritance
Mitochondrial Disorders
Expanded Repeat Disorders
Practical and Ethical Considerations
Immune Mechanisms: Concepts and Components
Blood–Brain and Blood–Nerve Barriers
Cerebrospinal Fluid
CSF Solutes
CSF Cells and Other Constituents
Immune Nervous System Diseases
Antibody‐Mediated Diseases
Neurological Disease with Pathogenic Autoantibodies
Neurological Disease with Systemic Disorders and Autoantibodies
T‐Cell‐Mediated Neurological Disease
Cytokine‐Driven Processes
Immunomodulation, Immunosuppression and Replacement Therapy
Targeted Ablative Therapies
Specifically Targeted Molecules
Ion Channels and Inherited Mutations
Migraine, Epilepsy, Movement Disorders and Ataxias
Nerve, Muscle and Neuromuscular Junction Diseases
Disease Causation
Voltage‐Gated Potassium Channels
Transient Receptor Potential Channels
Sodium Channels
Calcium Channels
Chloride and Ligand‐Gated Ion Channels
Acetylcholine Receptors
GABAA, Glycine and Glutamate Receptors
Acquired Channelopathies
Acknowledgements
Further Reading
4 Examination, Diagnosis and the Language of Neurology
Elements of Diagnosis
History
Nature of Symptoms
Neurological Examination: Preliminary Assessment
Brief Neurological Examination
Detailed Examination
Cognition and Mental State
Skull, Scalp and Spine
Cranial Nerves. I: Olfaction
II: Vision, Pupils and Fundi
III, IV and VI Diplopia: 4 Patterns and 4 Formal Rules
VI: Abducens Palsy
III: Oculomotor Palsy (complete)
Internuclear Ophthalmoplegia (INO)
IV: Trochlear Palsy
V: Trigeminal, Sensory and Motor
VII: Facial
VIII: Auditory
VIII: Vestibular
IX and X: Glossopharyngeal and Vagus
XI: Accessory
XII: Hypoglossal
Gait and Movement Disorders
Motor System
Posture of Outstretched Upper Limbs
Tone
Power, Muscle Bulk, Consistency
Cerebellar Signs
Tendon Reflexes
Extensor Plantar (Babinski)
Superficial Abdominal Reflexes
Respiration, Diaphragm
Lower and Upper Motor Neurone Lesions
Sensory System
Formulation
Difficulties
Diagnostic Tests
Imaging
Clinical Neurophysiology. Electroencephalography
Alpha, Theta, Delta and Beta Activity in Normal Subjects
Epilepsy
EEG Artefacts
Diffuse and Focal Brain Disorders
Brainstem Death
Clinical Neurophysiology: Nerve and Muscle
Electromyography (EMG)
Normal Motor Unit Recruitment
Chronic Partial Denervation
Fibrillation, Fasciculation and Positive Sharp Waves
Myopathic EMG
Myotonic EMG Changes
Hemifacial Spasm, Cramps, Myokymia and Stiff Person Syndrome
Peripheral Nerve Conduction Studies
Polyneuropathy
Entrapment Neuropathies
F waves
Hoffman (H) Reflexes
Neuromuscular Transmission. Repetitive Stimulation: Myasthenia and Myasthenic–Myopathic Syndromes
Cerebral‐Evoked Potentials
Specialised Investigations
Cerebrospinal Fluid Examination
Indications for LP and CSF Examination
Informed Consent, LP Risks, CSF Removal
LP: Contraindications
Biopsy: Brain, Nerve and Muscle
Neuropsychological Testing
The Vocabulary of Neurology
Focal Cortical Disorders
Language and Speech Disorders
Temporal Lobes
Frontal Lobes
Occipital Lobes
Parietal Lobes
Dominant Parietal
Non‐dominant Parietal
Motor Abnormalities: Brain and Spinal Cord
Hemiparesis
Cerebellar Syndromes
Movement Disorders
Paraparesis
Brainstem Syndromes
Anterior Horn Cell Disease
Sensory Abnormalities: Patterns at Different Levels
Peripheral Nerve Lesions
Polyneuropathy
Sensory Root and Root Entry Zone
Cord Lesions: Sensory Changes. Posterior Columns
Spinothalamic Tracts
Brainstem Lesions and Sensation
Thalamic Lesions
Mononeuropathy, Polyneuropathy
Neurogenic Muscle Wasting
Root Lesions
Cauda Equina Syndrome
Myopathy
Subacute Paralysis
Abnormal Illness Behaviour and Somatic Symptom Disorder
Acknowledgements
References
Further Reading
5 Cognition, Cortical Function and Dementias
Cognitive Functions and Clinical Practice
Attention and Neglect
Memory – Its Subdivisions
Anatomy
Amnesias
Paramnesias
Perception and Its Disorders
Hallucinations – False Perceptions
Voluntary Action Failure: Apraxias
Speech and Language
Reading, Writing and Numeracy
Reading
Writing
Numeracy
Knowledge and the Cognitive Executive
Emotion
Dementias
Investigation of dementia
Brain Imaging
Electroencephalography
CSF Examination
Additional Investigations
Alzheimer’s Disease
Neuropathology
Genetics
Clinical Features
Imaging
Therapy
Cholinesterase Inhibitors in Alzheimer’s
Frontotemporal Dementia
Genetics and Prognosis
Parkinson's Disease Dementia (PDD) and Dementia with Lewy Bodies (DLB)
Dementia with Other Movement Disorders (Chapter 7)
Vascular Dementia (VaD) – Vascular Cognitive Impairment (VCI)
Imaging
Differential Diagnosis of Probable VCI
Prion Diseases
Aetiology and Classification
Sporadic Creutzfeldt–Jakob Disease
Iatrogenic Creutzfeldt–Jakob Disease
Variant CJD
Secondary (Iatrogenic) vCJD
Kuru
Inherited Prion Diseases
Prevention
Dementia in Young Adults
Controversial Entities. Alcohol
Traumatic Brain Injury
Autoimmune Hashimoto and Coeliac Disease
Normal Pressure Hydrocephalus
Other Causes of Dementia
Management of Dementia
Safety, Legal Aspects and Financial Planning
Carers
Behavioural Management
Co‐morbidity
Autopsy and Brain Donation
Acknowledgements
Further Reading
Websites
Broca’s aphasia
Wernicke’s aphasia
6 Stroke and Cerebrovascular Disease
Pathophysiology: Ischaemic Stroke
Thrombosis, Embolism, Collaterals and the Ischaemic penumbra
Metabolic Changes and Raised Intracranial Pressure
Risk factors: Age, Hypertension, Tobacco, Lipids, Alcohol
Cardiac Disease
Ischaemic Stroke and the Blood
Stroke and TIA
Transient Ischaemic Attack (TIA)
Thrombo‐embolic TIA Patterns, Carotid Stenosis and Alternative Diagnoses
Lacunes
Typical Stroke Patterns. Internal Carotid Artery (ICA) Disease
Total and Branch Middle Cerebral Artery (MCA) Occlusion
Anterior Cerebral (ACA) and Posterior Cerebral Artery (PCA) Occlusion
Vertebral Artery
Basilar Artery
Border Zone Ischaemia
Vascular Dementia
Intracerebral Haemorrhage (ICH)
Deep Haemorrhage
Lobar Haemorrhage
Pontine and Cerebellar (Infratentorial) Haemorrhage
Stroke & TIA management. NICE Guidance: Stroke & TIA – Diagnosis and Initial Management
Rapid Recognition of Symptoms and Diagnosis
Imaging for Suspected TIA or Non‐disabling Stroke
Specialist Care
Alteplase (Thrombolysis) and Thrombectomy et al
Maintenance/Restoration of Homeostasis, Nutrition and Hydration
Emergency Neurosurgery, Early Mobilisation, Positioning
Stroke and TIA Investigations
Progressive Stroke
Common Complications of Stroke
Secondary Prevention
Intracranial Aneurysms
Clinical Features: Thunderclap Headache
Investigation and Management
SAH Complications, Treatment & Outcome
Cerebral Venous and Venous Sinus Thrombosis
Imaging, Treatment and Outcome
Arteriovenous Malformations
Cavernous Malformations
Non‐atherosclerotic Vascular Disease and Other Causes of Stroke
Carotid and Vertebral Artery Dissection
Vasculitis and Other Rarities
Infective Vasculitis
Thrombotic Thrombocytopenic Purpura
Behçet’s Disease, Susac’s and Sneddon’s Syndrome
Mitochondrial Disease and Fabry’s
CADASIL and CARASIL
Hypertensive Encephalopathy and Eclampsia
Migraine and Stroke
Moyamoya Angiopathy
Reversible Cerebral Vasoconstriction and Posterior Reversible Encephalopathy Syndromes (RCVS & PRES)
Acknowledgements
Further Reading
Personal References
Websites
7 Movement Disorders
Parkinsonian Syndromes and Parkinsonism. Parkinsonism: Motor Features
Parkinson’s Disease
PD: Premotor Features
PD: Typical Motor Presentation
PD: Non‐motor Features
Investigations
PD Treatment: Levodopa + DDIs: Madopar and Sinemet
Other Side Effects of Levodopa
Monoamine Oxidase B Inhibitors
Catechol‐O‐Methyl Transferase Inhibitors
Dopamine Agonists
Adverse Effects: Dopamine Agonists
Anticholinergics and Amandatine
Surgery for PD
Parkinson’s, Dementia and Lewy Body Pathology
Multiple System Atrophy (MSA)
Progressive Supranuclear Palsy (PSP)
Corticobasal Degeneration
Investigations: MSA, PSP, CBD
Ethnic and/or Region‐Specific Parkinsonism
Other Causes of Parkinsonism
Tremor
Benign Essential Tremor (ET)
Dystonic Tremor and Tremor Associated with Dystonia
Neuropathic Tremor and Fragile X Tremor Ataxia Syndrome (FXTAS)
Cerebellar (Pathway) Tremor and Holmes Tremor
Palatal Tremor
Orthostatic Tremor
Drug, Metabolic, Toxin‐Induced Tremors and Functional Tremor
Dystonia
Classification
Primary Generalised Dystonia
Primary Focal Dystonia
Secondary Heredo‐Degenerative Dystonias
Wilson’s Disease
Presentations
Diagnosis
Treatment
Secondary Symptomatic Dystonia
Dopa‐Responsive Dystonia (DRD)
Myoclonus‐Dystonia (MD)
Paroxysmal Dyskinesias
Management of Secondary Dystonia
Chorea
Huntington’s Disease
Features and Diagnosis
Juvenile Huntington’s
Neuro‐Acanthocytoses
Post‐Streptococcal Autoimmune Disorders
Benign Hereditary Chorea
Drug‐Induced Chorea
Chorea: Drug Management
Tics
Gilles de la Tourette Syndrome
Other Tic Disorders
Management of Tics
Myoclonus and Startle Syndromes
Functional Movement Disorders
Movement Disorders and Dopamine Receptor Blockade
Restless Legs Syndrome and Painful Legs and Moving Toes
Stiff Person and Stiff Limb Syndromes Fatal Encephalomyelitis with Rigidity
Acknowledgements
Further Reading
Websites
8 Epilepsy and Related Disorders
International League Against Epilepsy Classification
Simple and Complex Partial Seizures
Generalised Seizures
Typical and Atypical Absence Seizures, and Myoclonic Seizures
Classification and Causes of Epilepsy
Single‐Gene and Other Disorders
Hippocampal Sclerosis and Perinatal Disorders
Trauma
Tumours and Neurosurgery
Infection
Cerebrovascular
Degenerative
Immune‐Mediated
Provoked and Reflex Epilepsies
Differential Diagnosis
Loss of Awareness
Generalised Movements ‐ Epilepsy and Non‐Epileptic Attack Disorder (NEAD)
Focal Seizures, Tics, TIAs, MS, Movement Disorders
Drop Attacks
Focal Sensory, Visual and Vestibular Symptoms
Transient Psychological Experiences
Aggressive Outbursts and Criminal Activity
Sleep Phenomena
Prolonged Confusional and Fugue States
Intermittent Psychosis and Fugues
Investigation
EEG in Epilepsy Diagnosis – Epileptiform and Normal Phenomena
Idiopathic Generalised Epilepsy and Asbsence seizures
Some Specific Epilepsies
Partial Epilepsies
Prediction of Seizure Recurrence and Drug Effects
EEG Monitoring
Imaging
Management: Newly Diagnosed Cases
Management: Established Epilepsy
Antiepileptic Drugs
Acetazolamide
Benzodiazepines
Carbamazepine
Cenobamate
Eslicarbazepine Acetate
Ethosuximide
Felbamate
Gabapentin
Lacosamide
Lamotrigine
Levetiracetam
Oxcarbazepine
Perampanel
Phenobarbital
Phenytoin
Piracetam
Pregabalin
Primidone
Retigabine
Rufinamide
Stiripentol
Tiagabine
Topiramate
Valproate
Vigabatrin
Zonisamide
Emergencies: Prolonged Convulsions, Serial Seizures, Status Epilepticus
Treatment
Epilepsy Surgery
Driving Regulations: UK
Acknowledgements
Further Reading
Websites
9 Infections
Acute Bacterial Meningitis
The Meningitic Syndrome
Management
Antbiotics in the Community?
Immediate Hospital Management?
Should CT (Brain) Precede LP and Antibiotics?
Outcome
Neisseria Meningitidis
Streptococcus Pneumoniae
Brain Abscess
Subdural Empyema
Intracranial Epidural Abscess
Spinal Epidural, Subdural and Intramedullary Abscess
Infective Endocarditis: Neurology
Tuberculosis
Tuberculoma, TB Brain Abscess and Spinal TB
Leprosy (Hansen’s Disease)
Syphilis and Neurosyphilis (Treponema pallidum pallidum)
Lyme Disease and Neuroborreliosis
Leptospirosis (Weil’s Disease)
Brucellosis (Malta Fever, Undulant Fever)
Psitticosis and Cat Scratch Disease
Anthrax
Diphtheria
Botulism
Tetanus
Whipple’s Disease
Tick‐Borne Diseases
Other Infections
Viral Infections
Varicella Zoster Virus: Chickenpox and Shingles
Viral Meningitis
Viral Encephalitis
Herpes Simplex Encephalitis
Japanese Encephalitis and Viral Haemorrhagic Fevers
Poliomyelitis
Subacute Sclerosing Panencephalitis (SSPE)
Rabies
HTLV‐1
Epstein–Barr Virus (EBV)
COVID‐19
Fungal Infections
Parasitic Worms
Schistosomiasis (Bilharzia) and Other Parasitic Worms
African Trypanosomiasis (Sleeping Sickness)
American Trypanosomiasis (Chagas Disease)
Malaria
Toxoplasmosis
HIV
Acknowledgements
Further Reading
10 Nerve, Anterior Horn Cell and Muscle Disease. Peripheral Nerve Anatomy
Inherited Neuropathies
Charcot–Marie–Tooth Disease(s) and Related Disorders
Inflammatory and Acquired Neuropathies
Guillain–Barré Syndrome and GBS Variants
Chronic Inflammatory Demyelinating Polyradiculoneuropathy and CIPD Variants
Focal and Compressive Neuropathies
Upper Limb
Median Nerve Compression – Carpal Tunnel Syndrome (CTS)
Ulnar Nerve Compression
Lower Limb
Common Peroneal Nerve
Chronic Neuropathies with Paraproteinaemias
Vasculitic Neuropathies
Small Fibre Neuropathies (SFNs)
Plexopathies
Acute Brachial Neuritis a.k.a. Neuralgic Amyotrophy
Anterior Horn Cell Diseases
Motor Neurone Disease
Amyotrophic Lateral Sclerosis
Progressive Bulbar Palsy
Primary Lateral Sclerosis
Progressive Muscular Atrophy and MND Variants
Aetiology and Genetics
Management
Facial Onset Sensory Motor Neuronopathy
Monomelic Amyotrophy (Hirayama’s Disease)
Spinal Muscular Atrophies (SMAs)
Kennedy’s Disease (X‐Linked Spinobulbar Muscular Atrophy)
Investigation: SMA and Kennedy’s
Neuromuscular Junction Disorders. Myasthenia Gravis
Management
Lambert–Eaton Myasthenic Syndrome (LEMS)
Muscle Diseases
Assessment and Investigation
Genetic Muscle Diseases
Muscular Dystrophies
Dystrophinopathies: Xp21 Dystrophies – Duchenne and Becker
Myotonic Dystrophies (Dystrophia myotonica, DM1 and DM2)
Skeletal Muscle Channelopathies: Periodic Paralyses and Myotonias
Myotonia Congenita
Paramyotonia congenita and Sodium Channel Myotonias (SCMs)
Mitochondrial Respiratory Chain Diseases
Glycogen and Lipid Storage Myopathies
Inflammatory Myopathies, Rarities, Drugs and Rhabdomyolysis. Dermatomyositis
Polymyositis
Inclusion Body Myositis
Management
Rarities
Macrophagic Myofasciitis
Malignancy and Endocrine Disorders
Necrotising Autoimmune Myopathy
Drugs and Myopathies
Rhabdomyolysis
Acknowledgements
Further Reading and Websites
11 Multiple Sclerosis, Neuromyelitis Optica (Devic’s) and Other Demyelinating Diseases. Multiple Sclerosis
Aetiology, Migration, Genetics and Environment
Transmissible Agents
Vitamin D, Sunlight, Tobacco, Diet, Drugs, Infections, Immunisation
Pathology
Proposed Autoimmune Pathogenesis
Types of MS: Clinical Course
Relapsing‐Remitting MS (RRMS)
Secondary Progressive Multiple Sclerosis (SPMS)
Primary Progressive Multiple Sclerosis (PPMS)
Progressive Relapsing Multiple Sclerosis
MS Classifications and Criteria
Radiological MS and Clinically Isolated Syndrome (CIS)
Benign, Aggressive Forms and Early‐Onset MS
Natural History, Prognosis and Mortality
Clinical Features
Paroxysmal Symptoms and Pain
Diagnosis and Investigations
Differential Diagnosis
Management
Relapse Management
Disease‐Modifying Therapy
Interferon β
Glatiramer Acetate
Prescribing Guidelines and Other Therapies
Symptomatic Treatments and Rehabilitation
Neuromyelitis Optica, Devic's Disease
Epidemiology and Pathophysiology
Clinical Features and Investigations
Course, Natural History and Management
Acute Parainfectious Inflammatory Encephalopathies
Acute Disseminated Encephalomyelitis
ADEM: Features and Management
Acute Haemorrhagic Leukoencephalitis
Acknowledgements
Further Reading, References and Websites
12 Headache
Classification and Anatomical Concepts
Primary Headaches. Migraine
Management
Tension‐type Headache
Trigeminal Autonomic Cephalalgias (TACS)
Cluster Headache
Paroxysmal Hemicrania and Hemicrania Continua
Short‐lasting Unilateral Neuralgiform Attacks (SUNA and SUNCT)
Other Primary Headaches with Secondary Variants. Primary and Secondary Cough Headache
Primary and Secondary Exercise Headache
Primary (Benign) and Secondary Sex Headache
Primary and Secondary Thunderclap Headache
Other Primary Headaches
New Daily Persistent Headache and Chronic Daily Headache
Secondary Headaches. Medication Overuse Headache (MOH) and Withdrawal Headache
Headache and Vascular Disorders
Subarachnoid Haemorrhage
Carotid and Vertebral Artery Dissection
Giant Cell Arteritis
Intracranial Mass Lesion
Headache with High or Low Intracranial Pressure. Idiopathic (Benign) Intracranial Hypertension
Secondary Intracranial Hypertension Resembling IIH
Low‐pressure Headaches (Intracranial Hypotension)
Headaches and Head Trauma
Infection and Other Disorders
Acknowledgements
Further Reading, References and Websites
13 Cranial Nerve Disorders
I. Olfactory Nerve. Functional Anatomy
Examination
Olfactory Disorders
V. Trigeminal Nerve
Peripheral V Nerve Lesions
Superior Orbital Fissure Syndrome
Cavernous Sinus Syndrome
Nuclear V lesions
Trigeminal Neuralgia
Aetiology, Pathogenesis and Management
Trigeminal Idiopathic Sensory Neuropathy and Somatisation
Herpes Zoster Ophthalmicus (HZO)
Atypical Facial Pain
VII. Facial Nerve
Examination
Cerebellopontine Angle (CPA) Syndrome
A Canal Lesion or Distal to Stylomastoid Foramen
Bell’s Palsy
Melkersson–Rosenthal Syndrome
Bilateral Facial Weakness
Hemifacial Spasm
Other Involuntary Facial Movements
Progressive Hemifacial Atrophy
Lower Four Cranial Nerves: IX, X, XI and XII. IX. Glossopharyngeal Nerve
X. Vagus Nerve
Investigation and Causes
XI. Accessory Nerve
Examination and Localisation
XI Nerve Damage
XII. Hypoglossal Nerve
XII: Examination and Pathology
Jugular Foramen Syndrome
Bulbar and Pseudobulbar Palsy
Multiple Cranial Neuropathies
Cranial Epidural Abscess
Acknowledgements
Further Reading and Information
14 Neuro‐Ophthalmology
Visual Pathways
Retina and Optic Nerve
Photoreceptors, Bipolars, Amacrine and Ganglion Cells
Rods, Cones, Ganglion Cell Axons, Fovea and Foveola
Optic Chiasm, Optic Tract and Radiation
Occipital Cortex
Visual Association Cortex and V1–V5
‘What’
‘Where’
Cortical Eye Fields
Gaze Centres in the Brainstem
The Light Reflex: Pupil Constriction
III, IV and VI Nerves and Nuclei
Oculomotor Nucleus and the III Nerve
Trochlear Nucleus and the IV Nerve
Abducens Nucleus and the VI Nerve
Ocular Muscle Motor Units and Sensory Connections
The Near Response
The Far Response
Sympathetic Pathway to the Eye and Face
Examination
Visual Loss: Uni‐ and Bilateral Visual Failure
Optic Nerve Disease
Optic Neuritis with MS
Optic Neuritis with Neuromyelitis Optica (NMO‐ON, a.k.a. Devic’s Disease)
Optic Neuritis: Chronic Relapsing Inflammatory Optic Neuropathy
Infections and Other Causes – Optic Neuritis/Perineuritis
Neuroretinitis – the Macular Star
Optic Neuropathy – the Wider Classification
Retina and Optic Nerve Vascular Anatomy
Central and Branch Retinal Artery Occlusion
Central and Branch Retinal Vein Occlusion
Arteritic Anterior Ischaemic Optic Neuropathy: Giant Cell Arteritis (GCA)
Non‐Arteritic Anterior Ischaemic Optic Neuropathy
Posterior Ischaemic Optic Neuropathy
Chronic Ischaemia and Slow Flow Retinopathy
Tumours, Compressive and Infiltrative Optic Neuropathy
Meningioma
Optic and Opto‐Chiasmal Glioma
Hereditary – AD Optic Atrophy and Leber's
Toxic, Nutritional, Radiation‐Induced Optic Neuropathies and Trauma
Swollen Disc(s) – Papilloedema
Idiopathic Intracranial Hypertension
Uveo‐Meningitic Syndromes
Phakomatoses
Diplopia and Eye Movement Abnormalities
Orbital Conditions and Ophthalmoplegia
Orbital Inflammatory Syndromes
Orbital Infection, Mass Lesions and Infiltration
Cavernous Sinus Thrombosis
Caroticocavernous Fistulae
Ocular Myopathies
Palsies of III, IV and VI Nerves. Oculomotor Nerve (III Nerve Palsy)
Abducens (VI) and Trochlear (IV) Nerve Palsies
Gaze and Central Eye Movements
Saccades, Gaze Palsies and Oculogyric Crises
Internuclear Ophthalmoplegia (INO)
Skew Deviation, Ocular Tilt Reaction and Nystagmus
Chiasmal and Retrochiasmal Visual Pathways
Homonymous Hemianopia
Visual Cortex and Visual Association Areas
Functional (Non‐Organic) Visual Disorders
Pupil Abnormalities
Complete and Relative Afferent Pupillary Defect
Argyll Robertson Pupil and Parinaud's
Efferent Light Reflex Defects
Holmes–Adie Syndrome
Horner's – A Pupillary Sympathetic Defect
Acknowledgements
Further Reading and Information
15 Neuro‐Otology: Disorders of Balance and Hearing. Essential Anatomy
Afferents and Efferents
Dizziness and Vertigo Assessment
Nystagmus Varieties. Jerk, Normal, Deliberate and Pendular Nystagmus
Gaze‐Evoked, Gaze‐Paretic Jerk and Caloric Nystagmus
Nystagmus with Central Lesions. Torsional Central Nystagmus and Central Vestibular Horizontal Nystagmus
Downbeat, Upbeat, See‐Saw Nystagmus and Oculopalatal Tremor
Oculomasticatory Myorhythmia, Alternating and Convergence‐Retraction Nystagmus
Nystagmus in Childhood
General Medical Problems
Vestibular Investigations
Caloric Testing
Vestibular Disorders. Benign Paroxysmal Positional Vertigo
Posterior Semicircular Canal BPPV and Others
Vestibular Neuritis
Vestibular Migraine
Motion Sickness
Ménière's Disease
Bilateral Vestibular Failure
Vestibular Paroxysmia and Episodic Ataxia
Chronic and Persistent Postural‐Perceptual Dizziness (PPPD)
Management: Drugs and Physical Manoeuvres
Particle Repositioning Procedures and CBT
Ménière's, Central Vestibular Dysfunction and Migraine
Hearing Disorders
Auditory Anatomy and Investigation
Conductive Loss: Middle Ear and Tympanic Membrane
Age Related and Genetic Hearing Loss
Environmental, Trauma, Drug Related and Syndromic Hearing Loss. Acoustic Trauma
Drugs
Metabolic Disease and Autoimmune Disorders
Retro‐Cochlear Hearing Disorders
Infections
Extrinsic and Intrinsic Tumours
MS, Sarcoid and Superficial Siderosis
Vascular Disease
Temporal Lobe Disease
Auditory Agnosia and Corpus Callosum Surgery
Auditory Processing Disorder (APD)
Hearing Loss: Management
Conductive Hearing Loss
Sudden and Chronic Progressive Sensorineural Hearing Loss
Auditory Training and Strategies
Acknowledgements
Further Reading and Information
16 Spinal Cord and Spinal Column Disorders
Emergencies
Cervical Spine Degeneration and Pathology
Lateral and Central Cervical Disc Protrusion
Management of a Painful Cervical Root Lesion
Cervical and Thoracic Spinal Surgery
Lumbar and Sacrococcygeal Spine
Lumbar Canal Stenosis
Low Back Pain: Management
Lumbar Disc Surgery and Fusion
Paraparesis/Paraplegia, Myelitis, Transverse Myelitis and Myelopathy
Metabolic Conditions
Cord Vascular Disease. Cord Infarction and Spinal Haemorrhage
Cord Arteriovenous Malformations
Cord Vascular Neoplasms
Hereditary Spastic Paraparesis
Spinal Infection
Spine and Spinal Cord Major Trauma
Acute Spinal Injury Management and Rehabilitation
Whiplash
Craniocervical Junction – Basilar Invagination and Atlantoaxial Disease
Chiari Malformations and Os Odontoideum
Craniocervical Junction Disorders
Syringomyelia and Syringobulbia
Rheumatological and Bone Disorders. Rheumatoid Disease
Spondyloarthropathies
Paget's Disease of Bone
Osteopenic Disorders
Superficial Siderosis and Arachnoiditis
Spinal Dysraphism, Scoliosis and Kyphosis
Other Rare Causes of Spinal Deformity. Osteochondrodysplasias and Dysostoses
Metabolic Storage Disorders
Acknowledgements
Further Reading and Information
17 Ataxias, Cerebellar Disorders and Related Conditions
Acquired Ataxia Syndromes. The Posterior Fossa Mass Lesion
Acute Cerebellar Ataxia
Progressive Ataxia with a Chronic or Subacute Course
MS, Vascular and Inflammatory Disease
Alcohol, Solvent Abuse and Acquired Metabolic Disorders
Drugs, Heavy Metals and Physical Agents
Others
Late‐Onset Cerebellar Degenerations
Inherited Ataxia Syndromes. Autosomal Recessive Ataxias
Friedreich’s Ataxia
Ataxias with Defective DNA Repair
Ataxias with Oculomotor Apraxia
Ataxia: Metabolic Causes. Ataxia and Vitamin E Deficiency
Episodic Metabolic Ataxias
Progressive Metabolic Ataxias
Autosomal Dominant Cerebellar Ataxias
Episodic Ataxia: Genetic Forms
Episodic Ataxia Type 1
Episodic Ataxia Type 2 and Others
X‐Linked Ataxias and Mitochondrial Ataxia Syndromes
Congenital Ataxias
Acknowledgement
Further Reading and Information
18 Restorative Neurology, Rehabilitation and Brain Injury
Key Aspects
Restorative and Compensatory Approaches, Skill Learning and Task‐Related Training
Motor Disorders: Therapeutic Interventions. Bobath and Motor Relearning Programmes
Sensory Facilitation and Other Techniques
Balance and Posture
Treadmills, Functional Stimulation, Cueing, Fitness and Mental Imagery
Speech and Language Therapy and Communication Aids
Hemianopic Visual Loss and Restorative Therapies
Cognition, Environment and Compensatory Strategies
Restorative Approaches, Executive Function and Retraining
Specific Problems. Spasticity and Ataxia
Pain
Bladder, Bowel and Sexual Dysfunction
Fatigue
Dysphagia
Neuropsychiatric and Endocrine Problems
Single Incident Brain Injury
Stroke
Traumatic Brain Injury
Clinical Scales
Glasgow Coma Scale and Post‐Traumatic Amnesia Duration
‘Mayo’ Classification
Annegers Post‐Traumatic Epilepsy Data
Role of Imaging
Overall Outcome Assessment of TBI
Dementia
Service Delivery
Goal Setting
Outcome Measurement
Vocational Rehabilitation
Acknowledgements
Further Reading, References and Websites
19 Toxins, Physical Insults, Nutritional and Metabolic Disorders, Unregulated Drugs
Heavy Metals, Chemicals and Natural Toxins. Heavy Metals
Lead: Neuropathy and Encephalopathy
Mercury: Tremor and Encephalopathy
Arsenic: Polyneuropathy and Encephalopathy
Manganese and Aluminium: Encephalopathy
Thallium: Polyneuropathy and Encephalopathy
Tin and Bismuth Encephalopathy
Solvents and Chemicals
Toluene
Trichloroethylene, Tetrachlorethylene, Ethylene Oxide, Xylene, Styrene, Acrylamide, Acrylonitrile, Methyl Bromide, Allyl Chloride and Methyl Chloride
Cyanide
Methyl Alcohol (Methanol)
Nitrous Oxide
Organophosphates
Carbon Monoxide
Natural Toxins. Marine Toxins
Ciguatera
Tetrodotoxin
Scombroid
Shellfish, Contact and Envenomation Toxins
Snake Venoms
Spider Venoms
Scorpion Venoms
Tick Paralysis
Fungal Poisons
Lathyrism
Konzo
Hemlock
Subacute Myelo‐Optico Neuropathy and Tropical Myeloneuropathy
Radiation
Lightning and Electrical Shocks
Lightning
High‐Voltage (>1000 V) and Low‐Voltage Injuries
Injury from Lightning and Electric Shock
Heat, Cold, Diving and High Altitude. Heat Stroke
Hypothermia, Frostbite and Non‐Freezing Cold Injury
Diving and Decompression Sickness (DCS)
High Altitude: Acute Mountain Sickness, Cerebral and Pulmonary Oedema
Space Travel
Neurobiological Weapons
Vitamin Deficiencies and Copper Deficiency
Ethyl Alcohol
Alcohol Withdrawal
Alcoholic Cirrhosis and Encephalopathy, Wernicke and Korsakoff
Cerebellar Alcoholic Ataxia, Dementia and TBI
Neuropathies, Amblyopia, Strachan’s, Myopathy, Marchiafava–Bignami and Foetal Alcohol
Malignant Hyperthermia (MH)
Neuroleptic Malignant Syndrome (NMS)
Serotonin Syndrome and Tyramine Cheese Reaction
Porphyrias
Inherited Metabolic Disorders
Unregulated/Illegal Drugs and Drug Abuse
Stimulants
Stroke and Other Complications of Stimulants
Sedatives, Analgaesics and Tranquillisers
MPPP, MPTP and Parkinsonism
Hallucinogens
Solvents, Fuels and Gases
Acknowledgements, Further Reading and Personal References
20 Consciousness, Coma, Intensive Care and Sleep
Definitions of Consciousness and Coma
States of Impaired Consciousness
Stupor and Coma: A Practical Approach
Detailed Assessment
Level of Consciousness: Glasgow Coma Scale
Neurology
Eyelids in Coma
Pupils in Coma
Eye Movements in Coma
Other Cranial Nerves
Motor Responses
Tone and Reflexes
Involuntary Movements and Respiration
Other Coma‐Like States. Locked‐In Syndrome
Vegetative State a.k.a. Unresponsive Wakefulness
Minimally Conscious State
Akinetic Mutism
Psychogenic Unresponsiveness
Brain Death
Neurological Intensive Care
Severe Traumatic Brain Injury (TBI): General Principles
Neurosurgical Aspects of Severe TBI
Neurological Sequelae of ITU Care
Critical Illness Polyneuropathy and Myopathies
Sleep and Its Disorders. Sequence of Normal Sleep
The Autonomic Nervous System and Sleep
Regulation of Wakefulness and Sleep
Sleep Disorders
Insomnia
Obstructive Sleep Apnoea/Hypopnoea
OSAHS Management
Hypersomnias of Central Origin. Narcolepsy
Cataplexy
Possible Pathophysiology
Investigations and Management
Hypnogogic/Hypnopompic Hallucinations, Sleep Paralysis and Automatic Behaviours
Primary (Idiopathic) Hypersomnia
Idiopathic Recurring Stupor
Recurrent Hypersomnia (Kleine–Levin Syndrome)
Circadian Rhythm Adjustment Disorders: Shift Work and Jet Lag
Delayed/Advanced Sleep Phase Syndrome
Parasomnias
Non‐REM Parasomnias
REM Sleep Parasomnias
Other Parasomnias
Sleep‐Related Movement Disorders. Restless Legs Syndrome and Periodic Limb Movements of Sleep
Transition Disorders – Normal Variants
Other Sleep‐Related Disorders. Epilepsy and Sleep
Traumatic Brain Injury and Sleep
Extrapyramidal Disease and Rhythmic Movement Disorder
Acknowledgements, Further Reading and Personal References
21 Neuro‐Oncology
Clinical Features
Headache
Seizures and Focal Deficits
Brainstem/Cerebellar, Cognitive and Behavioural Symptoms
Endocrine, Visual and Olfactory Symptoms
Imaging
Tumours: The Multidisciplinary Approach
Neurosurgery
Radiotherapy and Chemotherapy
Classification and Grades
Diffuse Astrocytomas and Oligodendroglial Tumours
Surgery, Radiotherapy and Chemotherapy
Other Astrocytic Tumours
Ependymal Tumours and Choroid Plexus Tumours
Neuronal and Mixed Neuronal‐Glial Tumours
Pineal Region Tumours
Embryonal Tumours
Cranial and Paraspinal Nerve Tumours
Meningiomas
Mesenchymal, Non‐Meningothelial Tumours
Lymphomas
Germ Cell Tumours
Tumours of the Sellar Region
Pituitary Apoplexy
Pituitary Tumour Management
Craniopharyngioma
Metastatic Brain and Spinal Tumours
Other Mass Lesions: Dermoids, Epidermoid Cysts, Colloid Cysts, Rathke’s Pouch Cysts, Neuro‐Enteric Cysts, Optic Pathway Glioma
Optic Pathway Glioma
Primary Spinal Cord Tumours and Spinal Metastases
Complications of Radiotherapy and Chemotherapy
Acute Radiation Toxicity and Early‐Delayed Toxicity
Late‐Delayed Toxicity, Radiation Necrosis, Optic Neuropathy and Other Effects
Chemotherapy
Neurological Complications of Cancer (NCCs)
Indirect and Secondary NCCs
Acknowledgements, Further Reading and Websites
22 Neuropsychiatry
Mental State
Appearance and Behaviour
Speech
Mood and Affect
Thought
Thought Content
Perceptions
Cognition and Insight
Formulation
Risk Management
Lack of Insight
Suicidal Ideation and Behaviour
Agitation and Aggression
Mental Health and Capacity Acts
Definitions and Diagnoses in Psychiatry
Personality Disorders
Obsessions and Compulsions
Anxiety
Mood
Depression
Mania and Hypomania
Psychosis
Catatonia
Benign Sleep and Waking Phenomena
Functional Neurological Disorders
Functional: Terminology and Background
Diagnosis of FNDs
Functional Seizures
Functional Fixed Dystonia
Other Diagnoses
Management: FNDs
Dissociative Disorders
Dissociative Amnesia and Fugues
Depersonalisation/Derealisation Disorder
Neurology and Psychiatry
Epilepsy and Psychiatry. Pre‐ictal, Ictal and post‐ictal Disorders
Inter‐ictal Disorders
Forced Normalisation in Epilepsy
Personality Changes in Epilepsy
Psychotropic Effects of Anti‐Epileptics
Movement Disorders and Psychiatry
Parkinson’s Disease
Depression in Parkinson’s
Anxiety in Parkinson’s
Apathy in Parkinson’s
Psychosis in Parkinson’s
Cognitive Impairment in Parkinson’s
Impulse Control Disorder in Parkinson’s
Psychiatry and Other Movement Disorders
Electrical Stimulation Effects
Neuropsychiatry and Other Conditions
Psychiatry and MS
Minor Head Injury
Acknowledgement and Further Reading
Further Reading
23 Pain
Definitions
Neuropathic Pain Mechanisms – Sodium and Calcium Channel Expression
Sensitisation and Wind‐up
Excitation
Inhibition
Inflammation, Immune System and Pain
Supraspinal Influences
Central Pain
Pain in CNS Diseases. MS, Non‐paroxysmal, Paroxysmal and Nociceptive Pain
Parkinson’s Disease, Dystonia/Dyskinesia Pain, PAF and MSA
Central Post‐Stroke Pain
Spinal Cord Injury, Syringomyelia and MND
Phantom Pain
Painful Legs and Moving Toes
Epilepsy
Fibromyalgia
Peripheral Pain
Painful Peripheral Neuropathies
Small Fibre Neuropathies
Guillain–Barré and Neuralgic Amyotrophy
Painful Inherited Neuropathies
Other Painful Conditions. Shingles and Post‐Herpetic Neuralgia (PHN)
Erythromelalgia
Complex Regional Pain Syndrome
Viscerosomatic Disorders: Burning Mouth, Vulvodynia and Visceral Pain
Paroxysmal Extreme Pain Disorder
Plexopathies
Orthopaedic Conditions: Glomus Tumour and Osteoid Osteoma
Pain Management
Drug Treatments
Antidepressants
Antiepileptics
Local Anaesthetics and Topical Agents
NMDA Receptor Antagonists and Cannabinoids
Botulinum Toxin
Intrathecal Drugs
Neuroablative Procedures
Neurostimulation Procedures
Transcutaneous Nerve Stimulation
Peripheral Nerve Stimulation
Spinal Cord Stimulation
Deep Brain Stimulation
Motor Cortex Stimulation
Other Physical Treatments
Acupuncture
Psychological Approaches
The Placebo Phenomenon
Diminished Sensitivity to Pain
Congenital Insensitivity to Pain
Transient Indifference to Pain
Acknowledgement and Further Reading
24 Autonomic Aspects of Neurology. Anatomy and Neurotransmission
Localised Autonomic Disorders
Primary and Secondary Autonomic Disorders
Syncope
Orthostatic (Postural) Hypotension
Orthostatic Intolerance with Posturally Induced Tachycardia (PoTS)
Examination and Investigation
Regional Autonomic Dysfunction
Sudomotor: Anhidrosis and Hyperhidrosis
Gut
Kidneys and Urinary Tract
Sexual Dysfunction
Respiratory
Hypertension and Cardiac Changes
Parkinson’s Disease, Multiple System Atrophy (MSA) and Pure Autonomic Failure (PAF)
Rare Autonomic Disorders
Acknowledgements and Further Reading
25 Uro‐Neurology and Sexual Dysfunction
Lower Urinary Tract: Neurological Control
Bladder Dysfunction and Neurological Disease
Frontal Disease and Stroke
Parkinson’s Disease
Multiple System Atrophy
Spinal Cord Disease
MS and Peripheral Lesions
Urinary Retention
Urinary Retention in Women: Fowler’s Syndrome
Management
Storage Dysfunction: Simple Measures
Investigations
Other Measures and Drugs
Neuromodulation
Voiding Dysfunction
Clean Intermittent Self‐Catheterisation
Sexual Function
Sexual Dysfunction and Neurological Disease
Traumatic Brain Injury and Stroke
Epilepsy
Parkinson’s and Multi‐System Atrophy
Spinal Cord Injury, MS and other Spinal Lesions
Multiple Sclerosis
Sympathetic Thoracolumbar Outflow T10–L2 Lesions
Conus, Cauda Equina and Other Lesions
Sexual Dysfunction: Management
Type 5 Phosphodiesterase Inhibitors
Acknowledgememts, References and Further Reading
26 Systemic Conditions and Neurology
Cardiac and Aortic Disease
Cerebral Ischaemia and Aortic Disease
Spinal Cord Ischaemia and Aortic Disease
Cardiac Surgery: Neurological Complications
Cardiac Embolism
Endocrine Disease. Thyroid Disorders
Hyperthyroidism
Hypothyroidism
Hashimoto’s Encephalopathy
Diabetes Mellitus
Acute Metabolic Disturbances
Diabetic Neuropathies
Other Endocrine Disorders
Adrenal Disorders. Cushing’s
Addison’s
Phaeochromocytoma
Electrolyte Disturbances
Potassium, Calcium and Magnesium
Blood Disorders. Anaemias
Leukaemias
Plasma Cell Dyscrasias
Lymphomas
Langerhans Cell Histiocytosis
Polycythaemia and Thrombocythaemia
Thrombotic Thrombocytopenic Purpura and Bleeding Disorders
Coagulation Disorders
Primary Immunodeficiency
Hepatic Encephalopathy
Renal Disease
Uraemic Encephalopathy
Dialysis Encephalopathy
Dialysis Disequilibrium Syndrome
Neuropathy Associated with Renal Disease
Malabsorption
Coeliac Disease
Inflammatory Bowel Disease
Neurology and Transplantation
Some Specific Complications
Systemic Vasculitides and Related Disorders
Pathological Mechanisms
Polyarteritis Nodosa (PAN) and Microscopic Polyangiitis (MPA)
Granulomatosis with Polyangiitis (GPA)
Giant Cell Arteritis (GCA)
Isolated Cerebral Angiitis (ICA)
Rheumatoid Arthritis (RA)
SLE and Mixed Connective Tissue Disease (MCTD)
Antiphospholipid Syndrome
Sjögren’s Syndrome (SS)
Other Cerebral Arteriopathies. CADASIL
CARASIL
Fabry’s Disease
Susac’s Syndrome
Sneddon’s Syndrome
Degos’ Disease
Hereditary Angiopathy with Neuropathy, Aneurysms and Cramps (HANAC)
Reversible Cerebral Vasoconstriction Syndrome (RCVS) and Posterior Reversible Encephalopathy Syndrome (PRES)
Sarcoidosis
Clinical Features
Cranial Neuropathy
Peripheral Neuromuscular Sarcoid
Meningeal and Parenchymatous NS
Diagnosis and Prognosis
Behçet’s Syndrome
Pathergy Test
Treatment
IgG4‐Related Disease and CLIPPERS
Neurocutaneous Syndromes
Neurofibromatosis Types 1 and 2
Xeroderma Pigmentosum (XP)
Tuberous Sclerosis (TS)
Von Hippel–Lindau Disease (VHL)
Ataxia Telangiectasia
Sturge–Weber Syndrome
Neurological Aspects of Pregnancy. Epilepsy and Pregnancy
Reducing Risks to Mother and Child
Teratogenicity and Antiepileptic Drugs
Major Malformations and Antiepileptic drugs
Other Abnormalities and Neurodevelopmental Delay
Antiepileptic Drugs During Pregnancy
Foetal Malformations: Screening
Folic Acid
Pregnancy, Labour and Delivery
Eclampsia and Pre‐Eclampsia
Vitamin K and Antiepileptic Drugs
Post‐Partum Period
Breastfeeding
Maternal Epilepsy and an Infant
Cerebral Ischaemia, Haemorrhage and Emboli
Pregnancy and Other Neurological Conditions. Pituitary Disorders
Headache
Neuromuscular Disorders
Multiple Sclerosis
Chorea Gravidarum
Tumours
Idiopathic Intracranial Hypertension
Regional Obstetric Anaesthesia
Acknowledgements, References and Further Reading
Index. a
b
c
d
e
f
g
h
i
j
k
l
m
n
o
p
q
r
s
t
u
v
w
x
y
z
WILEY END USER LICENSE AGREEMENT
