Cover
Contents
Editorial Team
     Principal Editors
     International Editors
     Specialist Advisory Editors
     Authors
Beginnings
Foreword to the First Edition
Foreword to the Second Edition
Preface
Acknowledgements
1: Neurology Worldwide: The Epidemiology and Burden of Neurological Disease
     Epidemiology of neurological disease
          Frequency and distribution of neurological disease
          Causation
          Mortality
          Other measures and rates
     Burden of illness
          Definitions
          Cost of illness studies
          WHO burden of illness studies
          The personal burden of neurological disease – stigma
          Relative costs – developing countries
          Treatment gap
     References
     Further reading
2: Nervous System Structure and Function
     BASIC NEUROSCIENCE
          Introduction
               The functional unit: the neurone
               Amyloid and tau in Alzheimer’s disease
          Neurotransmission
               Electrical synapses
               Chemical synapses
               Types of CNS synapse
          Peripheral nervous system synapses
               Neuromuscular junction
          Neurotransmitters
               Transmitter release
               Transmitter‐gated ion channels and G‐protein‐coupled receptors
          Glia
               Astrocytes
               Oligodendrocytes (oligodendroglia)
               Microglia
               Ependyma
               Schwann cells
          Peripheral nerve fibre types
          Myelin and saltatory conduction
               Composition of the myelin sheath
                    Glycoproteins
                    Basic proteins
                    Other proteins
               Myelination and axon–Schwann cell interactions
          Sensory nerve endings
               Muscle spindle motor supply, annulospiral and flower spray endings
               Golgi tendon organs
     THE WORKING BRAIN
          Introduction
          Mechanisms of movement
               Three central systems of motor control
                    Corticospinal (pyramidal) system
                    Afferents to the primary motor cortex
                    Basal ganglia and ‘extrapyramidal’ movement disorders
                    Basic circuits within the basal ganglia
                    Other basal ganglia circuits
                    Cerebellum
          Sensation and sensory pathways
               Conscious and non‐conscious sensation
               Somatic sensory pathways in the cord and brain
                    Dorsal root ganglia – first order neurones
                    Termination of dorsal root afferents
                    Posterior column
                    medial lemniscus pathway
                    Spinothalamic pathway
                    Other ascending sensory pathways
          The brainstem
               Brainstem functions
               Motor fibres
                    Motor fibres to cranial nerve nuclei
               Sensory pathways within the brainstem
                    Spinothalamic tracts
                    Posterior column
                    medial lemniscus
                    Vth nerve central pathways
                    Clinical correlates
               Cranial nerve nuclear columns
          Reticular formation
               Essential anatomy
                    Respiratory control
                    Sleep, wakefulness and mood
                    Cardiovascular control
                    Patterns of primitive movements
                    Micturition control
                    Gate control: sensory modulation
          Limbic system, hippocampus and related structures
               Afferent hippocampal connections
               Efferent hippocampal connections
                    Declarative memory and long‐term potentiation
               Insula, cingulate cortex and parahippocampal gyrus
                    Amygdala
                    Nucleus accumbens
                    Septal region
                    Basal forebrain
          The thalamus
               Cortical connections
                    Specific (relay) thalamic nuclei
                    Association thalamic nuclei
                    Non‐specific thalamic nuclei
          Hypothalamus and pituitary gland
               Arterial and capillary supply
               Neuroendocrine cells
                    Anterior pituitary axis
               Posterior pituitary axis
               Circumventricular organs
               Sympathetic and parasympathetic hypothalamic activity
                    Temperature regulation
                    Water intake and thirst
                    Appetite and satiety
                    Mood, sexual arousal, wakefulness and memory
          Cranial nerves
               Olfactory nerve and its cortical connections
                    Olfactory epithelium
                    Olfactory bulb and tract
                    Cortical connections
                    Neurotransmission in the olfactory system
               Optic nerve and visual system
                    Retinal structure
                    Optic nerve, chiasm and optic tract
                    Optic radiation
                    Occipital cortex
                    Visual association cortex and V1–V5 terminology
                    Cortical eye fields
                    Gaze centres in the brainstem
                    The light reflex: pupil constriction
               III, IV and VI: third, fourth and sixth nerve nuclei and nerves
                    Oculomotor nucleus and IIIrd nerve
                    Trochlear nucleus and IVth nerve
                    Abducens nucleus and VIth nerve
                    The near response
                    The far response
                    Sympathetic pathway to the eye and face
               V: trigeminal nerve, sensory and motor nuclei
                    Motor nucleus of V and supratrigeminal nuclei
                    Sensory Vth nuclei
                    Mesencephalic Vth nucleus
                    Principal (pontine) Vth nucleus
                    Spinal Vth nucleus
                    Trigeminothalamic tract, lemniscus, cortical projection and reticular formation
                    VII: facial nerve
                    Nervus intermedius, greater petrosal nerve and chorda tympani
               VIII: vestibulocochlear nerve
                    Vestibular system
                    Auditory system
                    Cochlear nerve and central connections
               IX, X, XI and XII: glossopharyngeal, vagus, accessory and hypoglossal nerves
               XI: spinal accessory nerve
                    Cranial accessory XI, glossopharyngeal IX and vagus X – and their nuclei
               Glossopharyngeal nerve IX
               Vagus nerve X
               XII: hypoglossal nerve
          Autonomic nervous system
               Sympathetic system
               Parasympathetic system
                    Cranial parasympathetic III, VII, IX and X fibres and subsequent ganglia
                    Sacral parasympathetic fibres and ganglia
               Neurotransmission within the autonomic system
                    Neurotransmission at sympathetic and parasympathetic ganglia
                    Neuro‐effector junction transmission at target tissues
                    Junctional receptors at target tissues
                    Other autonomic neurotransmitter systems
     Acknowledgements
     Further reading
3: Mechanisms of Neurological Disease: Genetics, Autoimmunity and Ion Channels
     Genetics
          Modes of inheritance
               Autosomal dominant inheritance
               Autosomal recessive inheritance
               Sex‐linked inheritance
               Mitochondrial disorders
               Expanded repeat disorders
          How heritable are neurological conditions? Lessons from twins
          Mutation versus polymorphism
          The discovery process
          Practical considerations
          Ethical considerations: predictive versus diagnostic
          Incidental findings in diagnostic exome and genome sequencing
     Autoimmunity
          Fundamentals
          Components of the immune system
               Innate immune system
               Adaptive immune system
          Protection of neural tissues – the blood–brain and blood–nerve barriers
               Intrathecal antibody synthesis and the blood–brain barrier
               Analysis of CSF solutes
          Immune diseases of the nervous system
               Antibody‐mediated neurological diseases
               T‐cell mediated neurological disease
               Cytokine‐driven processes
          Interfering with the immune system as a treatment for disease
     Inherited mutations of ion channels
          Channelopathies
               Migraine
               Epilepsy
               Movement disorders and ataxia
               Disorders of peripheral nerve and autonomic function
               Muscle disease
               Psychiatric and cognitive disorders
          Disease causation in channelopathies
               Potassium channels
               Transient receptor potential channels
               Sodium channels
               Calcium channels
               Chloride channels
               Ligand‐gated ion channels
               Acquired versus inherited channelopathies
     References
     Further reading
          Genetics
          Immunology
          Channelopathies
4: The Language of Neurology: Symptoms, Signs and Basic Investigations
     Elements of diagnosis
          History
          Nature of symptoms
     Neurological examination
          Preliminary assessment
     Brief neurological examination
     Detailed neurological examination
          Cognition and mental state
          Skull, scalp and spine
          Cranial nerves
               I: olfaction
               II: vision, pupils and fundi
               III, IV and VI: eye movements
               V: trigeminal nerve – sensory and motor
               VII: facial nerve
               VIII: auditory nerve
               VIII: vestibular nerve
               IX and X: glossopharyngeal and vagus nerves
               XI: accessory nerve
               XII: hypoglossal nerve
          Gait and disorders of movement
          Motor system
               Posture of outstretched upper limbs
               Tone
               Power, muscle bulk and consistency
               Coordination – cerebellar signs
               Tendon reflexes
               Lower and upper motor neurone lesions
          Sensory system
          Formulation and diagnosis
               Difficulties with the history and examination
     Diagnostic tests in clinical neurology
          Imaging
               Conventional radiography (plain X‐rays)
               Computerised tomography
               Magnetic resonance imaging
          Clinical neurophysiology
               Electroencephalography
               Magneto‐encephalography and transcranial magnetic brain stimulation
               Clinical neurophysiological studies of nerve and muscle
               Electromyography
               Peripheral nerve conduction studies
               Neuromuscular transmission studies
               Cerebral‐evoked potentials
          Specialised blood and urine tests
          Cerebrospinal fluid examination
               Indications for LP and CSF examination
               LP technique
          Biopsy of brain, nerve and muscle
          Neuropsychological testing
               Intellectual function overall
               Formulation and conclusions
     Terminology, or vocabulary of clinical neurology
          Focal cortical disorders
               Language (and speech) disorders
               Temporal lobe lesions
               Frontal lobe lesions
               Occipital lobe lesions
               Parietal lobe lesions
          Motor abnormalities: brain and spinal cord
               Hemiparesis
               Cerebellar syndromes
               Disorders of movement
               Paraparesis
               Brainstem syndromes
               Anterior horn cell disease
          Sensory abnormalities: patterns at different levels
               Peripheral nerve lesions
               Sensory root and root entry zone lesions
               Spinal cord lesions
               Sensory changes in brainstem lesions
               Sensory changes following thalamic lesions
               Sensory changes in parietal lobe lesions
          Mononeuropathy, polyneuropathy, root lesions
               Mononeuropathy
               Multiple mononeuropathy
               Polyneuropathy
               Neurogenic muscle wasting
               Root lesions
               Cauda equina syndrome
               Myopathy
               Subacute paralytic conditions
               Unexplained symptoms, abnormal illness behaviour and somatoform disorder
     References
     Further reading
          General neurology, examination, classic works
          Nerve conduction and electromyography
          Electroencephalography
          CSF examination
          Neuropsychiatry
          Neuropsychology screening tests
          Imaging
5: Stroke and Cerebrovascular Diseases
     Epidemiology
     Clinical approach to stroke
     Ischaemic stroke
          Important vascular anatomy
               Heart and great vessels
               Extracranial and intracranial arteries
               Venous anatomy
          Pathophysiology of ischaemic stroke
               Thrombosis, embolism and hypoperfusion
               Microscopic and metabolic changes
               Ischaemic penumbra
               Pressure changes
          Risk factors and causes of ischaemic stroke
               Age, hypertension, smoking, lipids and drug misuse
               Cardiac disease
               Stroke and the blood
               Antiphospholipid antibodies and syndrome
          Clinical syndromes of cerebral ischaemia
               Transient ischaemic attacks
               Alternative diagnoses
               Lacunar stroke
               Rarer causes of lacunar syndromes
               Large vessel occlusion
               Internal carotid artery disease
               Middle cerebral artery occlusion
               Anterior cerebral artery occlusion
               Anterior choroidal artery occlusion
               Posterior cerebral artery
               Vertebral artery
               Basilar artery
               Border‐zone syndromes and ischaemic encephalopathy
               Vascular dementia
     Intracranial haemorrhage
          Risk factors
          Clinical syndromes of intracranial haemorrhage
               Deep haemorrhage
               Lobar haemorrhage
               Infratentorial haemorrhage
               Intraventricular haemorrhage
          Specific issues in intracerebral haemorrhage
          Prognosis of intracranial haemorrhage
     Subarachnoid haemorrhage
          Risk factors
          Clinical features
          Investigation
          Initial management
          Aneurysm treatment
          Management of complications
          Outcome
     Arteriovenous malformations
          Presentation
          Natural history
          Management
     Cavernous malformations
          Dural fistulae
     Investigation of stroke and TIAs
          Basic investigations for all: simple tests
          Imaging
          Guided investigations following basic profile
               Cardiac investigations
               Special investigations
     Management of acute stroke
          Organised care in a stroke unit
     Specific treatments for acute ischaemic stroke
          Thrombolysis
          Mechanical recanalisation
          Antiplatelet therapy in acute stroke
          Anticoagulation in thrombo‐embolic stroke
          Neuroprotection
          Maintenance of homeostasis
          Treatment of cerebral oedema
          Management of progressive stroke
          Common medical complications of stroke
     Secondary prevention
          Secondary prevention after TIA and stroke
          Lifestyle modification
          Lowering blood pressure
          Diabetes mellitus
          Lowering cholesterol
          Anticoagulation
          Antiplatelet therapy
          Management of carotid stenosis
               Symptomatic carotid stenosis
               Asymptomatic carotid stenosis
               Carotid stenting
               Vertebral stenosis
               Intracranial artery stenosis
     Non‐atherosclerotic vascular disease and other rarer causes of stroke
          Carotid and vertebral artery dissection
          Vasculitis
               Infective vasculitis
               Systemic vasculitides
               Collagen vascular disease
               Other vasculitis
               Isolated angiitis of the central nervous system
          Thrombotic thrombocytopenic purpura
          Behçet’s disease
          Susac’s syndrome
          Sneddon’s syndrome
          Mitochondrial disease
          Fabry disease
          CADASIL and CARASIL
          Hypertensive encephalopathy
          Migraine and stroke
          Moyamoya angiopathy
     Cerebral venous thrombosis
     Reversible cerebral vasoconstriction syndrome
     Vascular disease of the spinal cord
     Stroke: overall conclusions
     References
     Further reading
          Anticoagulation
          Arteriovenous malformations
          Atrial fibrillation
          CADASIL
          Carotid stenosis
          Causes of stroke
          Cavernous angiomas
          Cerebral amyloid angiopathy
          Dissection
          Fabry’s disease
          Endovascular treatment of hyperacute stroke
          Fibromuscular dysplasia
          Haematological disorders
          Hypertension
          Incidence, prevalence and prognosis
          Inflammatory vascular disorders
          Mitochondrial disease and MELAS
          Moyamoya syndrome
          Patent foramen ovale
          Radiology
          Secondary prevention
          Stroke definition
          Stroke units and organisation
          Subarachnoid haemorrhage
          Surgical treatment
          Thrombolytic therapy
          Transient ischaemic attack
          Venous thrombosis
6: Movement Disorders
     Parkinsonian (akinetic‐rigid) syndromes
          Cardinal motor features of parkinsonism
          Parkinson’s disease
               Premotor features of PD
               Typical’ motor presentation of PD
               Non‐motor features of PD
               Ancillary investigations
               Treatment of PD
               Levodopa
               Monoamine oxidase B inhibitors
               Catechol‐
               methyl transferase inhibitors
               Dopamine agonists
               Anticholinergics
               Amantadine
               Surgery for PD
          Dementia in association with Lewy body pathology
          Multiple system atrophy
          Progressive supranuclear palsy
          Corticobasal degeneration
          Ancillary investigations to distinguish between PD, MSA, PSP and CBD and other conditions
          Vascular parkinsonism
          Ethnic or region‐specific parkinsonism
          Other causes of parkinsonism
     Tremor
          Benign essential tremor
          Dystonic tremor
          Neuropathic tremor
          Fragile X tremor ataxia syndrome
          Cerebellar (pathway) tremor
               Intention tremor
               Holmes tremor
               Palatal tremor
               Orthostatic tremor
               Drug and toxin‐induced tremor
               Psychogenic tremor
     Dystonia
          Epidemiology
          Classifying dystonia
          Primary dystonia
          Dystonia‐plus syndromes
          Dopa‐responsive dystonia
          Myoclonus dystonia
          Symptomatic dystonia
          Heredodegenerative dystonias
          Wilson’s disease
               Clinical presentations
               Diagnosis
               Treatment
          Paroxysmal dyskinesias
          Investigation of dystonia
          Treatment of dystonia
          Surgery for dystonia
     Chorea
          Assessment of chorea
          Huntington’s disease
               Clinical features
               Juvenile Huntington’s disease
               Differential diagnosis of Huntington’s disease
          Neuro‐acanthocytosis
          Post‐streptococcal autoimmune disorders
          Benign hereditary chorea
          Drug‐induced chorea
          Drug management of chorea
     Tics
          Gilles de la Tourette syndrome
               Epidemiology, history and prevalence
               Psychopathology and associated co‐morbidity
               Assessment
               Current aetiological theories
               Multiple phenotypes of GTS
          Other forms of tic disorder
          Other diseases that can cause tics
          Investigation of tics
          Management of GTS and tics
     Myoclonus
          Physiological myoclonus
          Essential myoclonus
          Epileptic myoclonus
          Familial cortical tremor (also called benign autosomal dominant familial myoclonic epilepsy)
          Epilepsia partialis continua
          Secondary myoclonus
          Non‐progressive myoclonic encephalopathies
          Post‐anoxic action myoclonus (Lance–Adams syndrome)
          Opsoclonus myoclonus
          Myoclonus in neurodegenerative disorders
          Subcortical myoclonus
          Startle syndromes
          Palatal myoclonus
          Spinal myoclonus
          Peripheral myoclonus
          Psychogenic myoclonus
          Drug‐induced myoclonus
          Treatment of myoclonus
     Other movement disorders
          Psychogenic (functional) movement disorders
          Movement disorders associated with dopamine receptor blockade or dopamine depletion
          Restless legs syndrome
          Painful legs and moving toes
          Stiff person syndrome, stiff limb syndrome and encephalomyelitis with rigidity
     Neurophysiological assessment of movement disorders
          Neurophysiological assessment of tremor
          Neurophysiological assessment of dystonia
          Neurophysiological assessment of myoclonus
               Cortical myoclonus
               Reticular, or brainstem, myoclonus
               Spinal myoclonus
     Further reading
          Parkinson’s disease and related disorders
          Chorea
          Wilson’s disease
          Dystonias
          Tics
          Tremor
          Myoclonus
          Psychogenic movement disorders
          Other movement disorders
7: Epilepsy and Related Disorders
     Definitions
     Epidemiology
     ILAE classification of seizure type
          Partial (focal) seizures
               Simple partial seizures
               Complex partial seizures
          Generalised seizures
               Typical absence seizure (petit mal seizure)
               Atypical absence seizure
               Myoclonic seizure
               Clonic seizure
               Tonic seizure
               Tonic–clonic seizure (grand mal seizure)
               Atonic seizure
     ILAE classification of the epilepsies and epilepsy syndromes
          Idiopathic generalised epilepsy
               Childhood absence epilepsy
               Juvenile myoclonic epilepsy
               Epilepsy with grand mal seizures on awakening
          Benign partial epilepsy syndromes
               Benign partial epilepsy with centrotemporal spikes
               Early onset benign occipital epilepsy (
               Panayiotopoulos syndrome)
          West syndrome
          Lennox–Gastaut syndrome
          Febrile seizures
     Causes of epilepsy
          Idiopathic epilepsy
               Pure epilepsies resulting from single gene disorder
               Pure epilepsies with complex inheritance
          Symptomatic epilepsy
               Inborn errors of metabolism
               Progressive myoclonic epilepsy
               Epilepsies in neurocutaneous syndromes
               Cortical dysplasia
               Hippocampal sclerosis
               Cerebral palsy, perinatal and prenatal injury
               Post‐vaccination encephalopathy
               Cerebral tumour
               Cerebrovascular disease
               Arteriovenous malformation
               Cavernous haemangioma (cavernoma)
               Other vascular lesions
               Dementia and degenerative disorders
               Post‐traumatic epilepsy
               Epilepsy after neurosurgery
               Bacterial or viral meningitis and encephalitis
               Parasitic diseases
               Inflammatory and immunological disorders
     Differential diagnosis of epilepsy
          Loss of awareness and collapse
               Epilepsy
               Syncope
               Cardiac syncope
               Dissociative seizures
               Panic attacks
               Hypoglycaemia
               Other neurological disorders
          Generalised convulsive movements
               Epilepsy
               Syncope with myoclonic jerking movements
               Primary cardiac or respiratory abnormalities presenting with secondary anoxic seizures
               Involuntary movement disorders and other neurological conditions
               Hyperekplexia
               Dissociative seizure
          Focal convulsive movements
               Focal motor seizures
               Tics
               Transient cerebral ischaemia
               Tonic spasms of multiple sclerosis
               Paroxysmal movement disorders
               Transient facial muscle and eye movements
               Partial seizures
               Movement disorders
               Other neurological disorders
          Drop attacks
               Epilepsy
               Cardiovascular causes
               Movement disorders
               Brainstem, spinal or lower limb abnormalities
               Cataplexy
               Metabolic disorders
               Idiopathic drop attacks
               Vertebrobasilar ischaemia
          Transient focal sensory symptoms
          Transient vestibular symptoms
          Transient visual symptoms
          Transient psychic experiences
               Epilepsy
               Migraine
               Panic attacks
               Drug‐induced flashbacks
               Hallucinations or illusions caused by loss of a primary sense
               Psychotic hallucinations and delusions
               Dissociative seizures
          Aggressive outbursts
          Episodic phenomena in sleep
               Normal physiological movements
               Frontal lobe epilepsy
               Other epilepsies
               Pathological fragmentary myoclonus
               Restless leg syndrome
               Non‐REM parasomnia
               REM parasomnia
               Sleep apnoea
               Other movements in sleep
          Prolonged confusional or fugue states
               Acute encephalopathy
               Non‐convulsive status epilepticus
               Intermittent psychosis
               Transient global amnesia
               Hysterical fugue
     Investigation of epilepsy
          Routine tests to carry out in all cases
          Biochemical, haematological and immunological tests
          Electroencephalography
               EEG in the diagnosis of epilepsy
               Epileptiform phenomena
               EEG in the classification of epileptic seizures and syndromes
               EEG and prediction of seizure recurrence
               EEG and withdrawal of antiepileptic medication
               EEG and antiepileptic drugs
               Long‐term EEG monitoring
               Neurophysiology for assessing patients for epilepsy surgery
               EEG and tonic–clonic status epilepticus
               Non‐convulsive status epilepticus
               EEG in the intensive care setting
               EEG and cognitive deterioration
          Imaging in epilepsy
               X‐ray computed tomography
               Magnetic resonance imaging
               Functional magnetic resonance imaging
               Tractography
               Single‐photon emission computed tomography
               Positron emission tomography
     Medical treatment
          Principles of treatment of newly diagnosed patients
               Diagnosis
               Risk of recurrence of seizure
               Type, timing and frequency of seizure
          A protocol for initial treatment
          Treatment protocol for patients with chronic epilepsy
               Assessment
               Treatment plan
               Limits of therapy
               Choice of drugs and details of antiepileptic drugs
               Monotherapy versus combination therapy
          Role of antiepileptic drug level measurements
          Patient information
          Treatment of patients with epilepsy in remission
               Discontinuation of drug therapy
               How to withdraw therapy – the importance of slow reduction
          Management of epilepsy in learning disability
               Definition
               Prevalence of epilepsy in people with learning disability
               Assessment
               Diagnostic difficulties
               Treatment
          Management in the elderly
               Causes of epilepsy
               Diagnosis and investigations
               Medical treatment
     Antiepileptic drug treatment
          Acetazolamide
          Benzodiazepines
          Carbamazepine
          Eslicarbazepine acetate
          Ethosuximide
          Felbamate
          Gabapentin
          Lacosamide
          Lamotrigine
          Levetiracetam
          Oxcarbazepine
          Perampanel
          Phenobarbital
          Phenytoin
          Piracetam
          Pregabalin
          Primidone
          Retigabine
          Rufinamide
          Stiripentol
          Tiagabine
          Topiramate
          Valproate
          Vigabatrin
          Zonisamide
     Emergency drug treatment
          Prolonged convulsions or serial seizures
          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
          Who is suitable for presurgical assessment?
          Presurgical assessment
               Clinical history
               Psychiatric history
               Neuroimaging
               Electroencephalography and other neurophysiological investigations
               Neuropsychology
               Counselling
          Surgery
               Curative resective surgery
               Palliative procedures
               Follow‐up and prognosis
     Driving regulations in the UK
          Group 1 licensing
          Group 2 licensing
          For both types of licence
          Role of the doctor vis‐à‐vis the driving regulations
          Other types of vehicle
          Acknowledgement
     References
     Further reading
          Useful websites with epilepsy information for patients
          Books
          Journal articles
8: Dementia and Cognitive Impairment
     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
     The dementias
          Alzheimer’s disease
               Clinical features and assessment
               Investigations
               Management
          Frontotemporal dementia
               Behavioural variant frontotemporal dementia
               Semantic dementia
               Progressive non‐fluent aphasia
               Investigations
               Genetic pathological correlations
               Prognosis
               Management
          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
          Dementia in young adults
          Potentially reversible causes of dementia
               Neoplasms and other space‐occupying lesions
               Epilepsy and dementia
               Limbic encephalitis
               Infective, metabolic, toxic and other causes of dementia
          Controversial entities
     Management of dementia
          Risk factor management
          Co‐morbidity
          Behavioural management
          Safety
          Caring for the carer
          Planning for the future and end of life issues
     References
     Further reading
          Epidemiology
          Cognitive function and clinical syndromes
          Investigations
          Alzheimer’s disease
          Frontotemporal lobar degeneration
          Vascular cognitive impairment
          Parkinson’s disease dementia and dementia with Lewy bodies
          Dementia in young adults: general
          Others
          Management
               Treatment of Alzheimer’s disease
9: Infection in the Nervous System
     Bacterial meningitis
          Epidemiology
          Pathogenesis
          Clinical presentation
          Investigation
          Management
          Specific causes of bacterial meningitis
               meningococcal meningitis)
               pneumococcal meningitis)
               type b
               Group B streptococcus
               Gram‐negative meningitis
               Gram‐positive meningitis (non‐pneumococcal, non‐group B)
               Nosocomial meningitis
     Focal CNS infection
          Cerebral abscess
               Clinical features
               Investigations
               Treatment
               Surgical intervention
               Prognosis
          Subdural empyema
               Pathophysiology
               Causative agents
          Intracranial epidural abscess
          Spinal epidural abscess
          Spinal subdural abscess
          Spinal cord intramedullary abscess
     Infective endocarditis
     Granulomatous infections of the nervous system
          CNS tuberculosis
               Tuberculous meningitis
               Parenchymal CNS tuberculosis
               Tuberculous abscess
               Spinal tuberculosis (tuberculous spondylitis)
          Leprosy (Hansen’s disease)
               Clinical features
               Peripheral nerve involvement
               Borderline leprosy
               Primary neuritic leprosy
               Diagnosis
               Management
          Brucellosis (undulant fever)
     Spirochete CNS infections
          Syphilis
               Late neurosyphilis
               Intracerebral gumma
               Diagnosis
               Treatment
     Zoonoses
          Lyme neuroborreliosis
               Clinical features of early neuroborreliosis
               Early disseminated infection
               Cranial neuropathy
               Acute radiculoneuropathy
               Other syndromes
               Clinical features of late neuroborreliosis
               Post Lyme syndrome
               Diagnosis
               Treatment
          Leptospirosis
          Anthrax
          Psittacosis
          Cat scratch disease
     Infections of the nervous system associated with toxin production
          Diphtheria
               Management
          Botulism
               Forms of botulism
               Clinical presentation
               Diagnosis
               Neurophysiology
               Management
          Tetanus
               Epidemiology
               Clinical features
               Diagnosis
               Differential diagnosis
               Treatment
     Miscellaneous infections of the nervous system
          Mycoplasma
          Whipple’s disease
          Melioidosis
          Tick‐borne disease
               Ehrlichiosis
               Tularaemia
               Rickettsial disease
     Viral infections of the nervous system
          Viral meningitis
               Aetiology
               Clinical features
               Diagnosis
               Viral isolation
               Management and prognosis
          Chronic and recurrent meningitis
               Mollaret’s meningitis
          Encephalitis
               Pathophysiology
               Incidence
               Clinical features
               Diagnosis
               Herpes simplex encephalitis
               Other causes of encephalitis
               Arboviruses (arthropod‐borne viruses)
          Viral haemorrhagic fevers
          Polio and the post‐polio syndrome
               Differential diagnosis
               Prevention
               Post‐polio syndrome
          Subacute sclerosing panencephalitis
          Rabies
               Clinical features
               Diagnosis
               Management
          HTLV‐1
               HTLV‐1 associated myelopathy (tropical spastic paraparesis)
               Other neurological manifestions of HTLV‐1 infection
          HTLV‐2
     Fungal infections
          Risk factors
          True yeasts
               Histoplasmosis
               Coccidiomycosis
          Pseudohyphae
               species
          True hyphae (moulds)
               Mucormycosis
     Parasitic disease of the nervous system
          Neurocysticercosis
               Clinical features
               Diagnosis
               Management
          Trematodes
               Schistosomiasis (bilharzia)
     Protozoa
          American trypanosomiasis (Chagas disease)
          African trypanosomiasis (sleeping sickness)
          Malaria
               Diagnosis
               Acute management
               Prevention
     Neurological disorders resulting from HIV
          Basic principles of neuroAIDS
          Opportunistic infections in HIV
               Tuberculous meningitis and brain abscess
               Cryptococcal meningitis
               Cytomegalovirus infection
               Herpes simplex virus
               Varicella‐zoster virus
          Progressive multifocal leukoencephalopathy
          Primary CNS lymphoma
          Neurological complications resulting directly from HIV
               HIV‐associated neurocognitive disorders
               HIV‐related vacuolar myelopathy
               HIV‐related neuropathy
               Diffuse inflammatory lymphocytosis syndrome
               Toxic neuropathy for antiretroviral drugs
          HIV‐associated myopathy
          Motor neurone disease
          Immune reconstitution inflammatory syndrome
     Conclusions
     Acknowledgement
     References
     Further reading
          Bacterial meningitis
          Tuberculosis
          Syphilis and leprosy
          Toxins
          Zoonoses
          Viral infections
          Tropical diseases
          HIV‐related
10: Nerve and Muscle Disease
     Peripheral nerve disorders
          Macro‐anatomy of the peripheral nerve
               Upper limbs
               Lower limbs
          Micro‐anatomy of the peripheral nerve
               Peripheral nerve compartments
               Immunology
          Pathophysiology of the peripheral nerve
     Diseases of the peripheral nerve
          General approach to peripheral nerve disease
               History
               Examination
               Scores
               Neurophysiology
               Nerve biopsy
          Inherited neuropathies
               Charcot–Marie–Tooth disease and related disorders
               An approach to the diagnosis of CMT and related disorders
               Charcot–Marie–Tooth disease
               Hereditary sensory neuropathy
               Distal hereditary motor neuropathy
               Hereditary neuralgic amyotrophy
               Importance of a genetic diagnosis in CMT and related disorders
               Familial amyloid polyneuropathy
               Transthyretin‐related familial amyloid polyneuropathy
               Apolipoprotein A‐1 related FAP
               Gelsolin‐related FAP
               Beta‐2 microglobulin FAP
          Acquired neuropathies
               Inflammatory neuropathies
               Acute neuromuscular weakness and the inflammatory neuropathies
               Guillain–Barré syndrome and its variants
               Chronic inflammatory neuropathies
               Chronic inflammatory demyelinating polyradiculoneuropathy
               CIDP variants
               Paraproteinaemic neuropathies
               Vasculitic neuropathies
          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
               Common peroneal neuropathies
               Plexopathies
               Acute brachial neuritis
     Anterior horn cell diseases
          Motor neurone disease
               Amyotrophic lateral sclerosis
               Progressive bulbar palsy
               Flail arm/leg syndrome
               Primary lateral sclerosis
               Progressive muscular atrophy
               Cognitive involvement
               Other forms
               Aetiology
               Diagnosis of MND
               Investigations
               Incidence and prognosis
               Management
          Spinal muscular atrophy
               Genetics and aetiology
               Clinical features
               Investigation
               Management
     Disorders of the neuromuscular junction
          Myasthenia gravis
          Antibody negative’ myasthenia gravis – Anti MuSK antibodies
          Clinical features
          Diagnostic tests
          Management
          Ocular myasthenia gravis
          Pregnancy and myasthenia
          Myasthenic crisis
          Cholinergic crisis
          Anaesthesia and peri‐operative care
          Other causes of abnormal neuromuscular transmission
          Lambert–Eaton myasthenic syndrome
               Clinical features
               Aetiology
               Investigations
               Treatment
          Congenital myasthenia
               Clinical features
               Investigation
               Management
     Muscle diseases
          Basic muscle biology
          Clinical assessment of the patient with muscle disease
          History
          Examination
          Investigation of muscle diseases
               Creatine kinase
               Neurophysiology
               Muscle biopsy
               Metabolic testing
               Genetic testing
          Genetic muscle diseases
               Muscular dystrophies
               Congenital myopathies
               Skeletal muscle channelopathies (Chapter 3)
          Metabolic muscle disease
               Mitochondrial respiratory chain diseases
               Glycogenoses and lipid storage disorders (Chapter 19)
          Acquired muscle diseases
               Inflammatory myopathies
               Idiopathic inflammatory myopathies
               Dermatomyositis
               Polymyositis
               Inclusion body myositis
               Investigation of inflammatory myopathy
               Extended investigation of inflammatory myopathies
               Treatment of idiopathic inflammatory myopathies
               Other rare inflammatory myopathies
               Necrotising autoimmune myopathy
               Drugs and myopathy
               Rhabdomyolysis
     References
     Further reading
          Hereditary neuropathy
          Amyloidosis
          Acquired neuropathies
          Motor neurone disease and spinal muscular atrophy
          Myasthenia gravis
          Muscular dystrophy and congenital myopathies
          Myotonic dystrophy
          Muscle channelopathies
          Mitochondrial disease
          Metabolic muscle disease
          Inflammatory myopathy
11: Multiple Sclerosis and Demyelinating Diseases
     Epidemiology
          Migration studies
          Health economics
     Aetiology
          Genetic susceptibility
          Environmental factors
          Transmissible agents
               Vitamin D and sunlight exposure
               Smoking
     Pathophysiology
          Pathology
          Autoimmune pathogenesis
     Clinical course
          Types of multiple sclerosis
               Relapsing remitting multiple sclerosis
               Secondary progressive multiple sclerosis
               Primary progressive multiple sclerosis
               Progressive relapsing multiple sclerosis
          Natural history and prognosis
               Clinically isolated syndrome
               Established multiple sclerosis
               Benign multiple sclerosis
               Aggressive multiple sclerosis
               Early onset multiple sclerosis
               Mortality
          Factors affecting relapse activity
               Infections
               Pregnancy
               Stress
               Vaccines
     Clinical features
     Diagnosis
          Diagnostic investigations
               Magnetic resonance imaging
               Cerebrospinal fluid
               Evoked potentials
               Autoantibodies
          Diagnostic criteria
               Schumacher criteria
               Poser criteria
               McDonald 2001 criteria
               McDonald 2005 criteria
               McDonald 2010 criteria
               Diagnostic criteria for primary progressive multiple sclerosis
               Radiologically isolated syndrome
          Differential diagnosis
          The diagnostic process
     Management
          Education and support
          Management of acute relapses
               Assessment
               Treatment
          Disease‐modifying therapy
               Currently available therapies
               Interferon β and glatiramer acetate
               Interferon β‐1b
               Intramuscular interferon
               Intramuscular interferon β‐1a
               Subcutaneous interferon β‐1a
               PEGylated interferon β
               Glatiramer acetate
               Natalizumab
               Fingolimod
               Teriflunomide
               Alemtuzumab
               Dimethyl fumarate
               Initiation of treatment
               Discontinuation of treatment
               Emerging therapies
               Other therapies
               Future immunomodulatory therapies
               Neuroprotection and remyelination
               Disease‐modifying therapy in primary progressive multiple sclerosis
          Symptomatic treatment
               Fatigue
               Spasticity
               Weakness
               Ataxia
               Bladder and bowel dysfunction
               Sexual dysfunction
               Pain and paroxysmal symptoms
               Cognitive and psychiatric dysfunction
               Visual dysfunction
               Vertigo
               Bulbar and respiratory dysfunction
               Temperature sensitivity
               Neurological rehabilitation
               Vocational rehabilitation
               Palliative care
               Complementary and alternative medicine
     Neuromyelitis optica
          Epidemiology
          Pathophysiology
          Clinical features
          Investigations
          Diagnostic criteria
          Course and natural history
          Management
     Acute para‐infectious inflammatory encephalopathies
          Acute disseminated encephalomyelitis
               Clinical features
               Pathophysiology
               Differential diagnosis
               Investigations
               Clinical course and prognosis
               Management
          Acute haemorrhagic leukoencephalitis
               Clinical features
               Investigations
               Prognosis
               Management
     References
     Further reading
          Epidemiology and aetiology
          Pathophysiology
          Clinical course
          Diagnosis
          Management
          Disease‐modifying therapy
          Symptomatic treatment and neurological rehabilitation
          Neuromyelitis optica
          Acute disseminated encephalomyelitis
12: Headache
     Evaluation of the headache patient
          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
               Subarachnoid haemorrhage
               Carotid and vertebral artery dissection
               Giant cell arteritis
          Headache caused by disturbance of intracranial pressure
               Intracranial hypertension
               Low pressure headaches (intracranial hypotension)
     Primary headaches
          Anatomy and physiology of headache
          Migraine
               Epidemiology
               Clinical features
               Assessment and investigations
               Management
          Tension‐type headache
               Epidemiology
               Clinical features
               Investigations
               Management
     Trigeminal autonomic cephalalgias
          Cluster headache
               Epidemiology
               Clinical features
               Investigations
               Management
               Paroxysmal hemicrania
               Short‐lasting unilateral neuralgiform headache attacks
               Hemicrania continua
          Other primary headaches
               Primary cough headache
               Primary exercise headache
               Primary sex headache
               Primary thunderclap headache
               Primary stabbing headache
               Nummular headache
               Hypnic headache
               New daily persistent headache
     Chronic daily headache
     References
     Further reading
          Background reading
          Classification
          Epidemiology and clinical features
          Investigation
          Specific and unusual headache syndromes
          Treatments
13: Cranial Nerve Disorders
     I. Olfactory nerve
          Functional anatomy
          Symptoms
          Examination
          Causes of anosmia
               Ageing
               Upper respiratory infections, nasal and paranasal sinus disease
               Trauma and surgery
               Neurodegenerative disorders
               Other causes of olfactory dysfunction
     V. Trigeminal nerve
          Examination
          Peripheral Vth nerve lesions
               Numb chin syndrome
               Superior orbital fissure syndrome
               Cavernous sinus syndrome
          Nuclear Vth nerve lesions
          Trigeminal neuralgia
               Clinical features
               Aetiology and pathogenesis
               Treatment
          Trigeminal sensory neuropathy
          Herpes zoster ophthalmicus
          Atypical facial pain
     VII. Facial nerve
          Functional anatomy
          Examination
          Supranuclear facial weakness
          Nuclear VIIth lesions
          Cerebellopontine angle syndrome
          Facial canal syndrome
          Lesions at and distal to the stylomastoid foramen
               Bell’s palsy
               Recurrent facial palsy
               Bilateral facial weakness
               Hemifacial spasm
               Other involuntary facial movements
     Lower four cranial nerves: IX, X, XI and XII
     IX. Glossopharyngeal nerve
          Functional anatomy
          Examination
          IXth nerve lesions, peripheral and central
               Glossopharyngeal neuralgia
     X. Vagus nerve
          Functional anatomy
          Clinical features
          Causes and localisation of lesions
          Investigation
     XI. Accessory nerve
          Functional anatomy
          Examination and localisation of lesions
          XIth nerve lesions
     XII. Hypoglossal nerve
          Functional anatomy
          Localisation of lesions
          Causes of XIIth nerve lesions
          Investigation
          Cranial nerve injury following carotid endarterectomy or carotid angioplasty with stenting
          Jugular foramen syndrome
     Bulbar and pseudobular palsy
          Normal swallowing
          Bulbar palsy
          Pseudobulbar palsy
          Dropped head syndrome
     Multiple cranial neuropathies
          Other causes of MCNs
          Intracranial epidural abscess
     Acknowledgement
     References
     Further reading
          Trigeminal nerve (V)
          Facial nerve (VII)
          Bulbar and pseudobulbar palsy
          Glossopharyngeal nerve (IX)
          Accessory nerve (XI)
          Hypoglossal nerve (XII)
          Multiple cranial nerve palsies
14: Neuro‐Ophthalmology
     Unilateral visual failure
          History
               Mode of onset
               Positive symptoms
               Effect of light level
               Direct questions
          Examination
               Visual acuity
               Colour vision
               Amsler’s test
               Visual field testing
               Fundus examination
               Associated features
     Bilateral visual failure
     Special investigations in neuroophthalmology
          Optical coherence tomography
          Ultrasound
          Clinical electrophysiology of the eye
          Visual field testing
          Fundus fluorescein angiography
     Optic nerve disease
          Optic neuropathy
               Inflammatory optic neuropathies (optic neuritis)
               Optic neuritis associated with multiple sclerosis
               Optic neuritis associated with neuromyelitis optica (Devic’s syndrome)
               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
               Nosology 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
               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
               Uveomeningitic syndromes
               Neoplasia
     The phakomatoses in neuro‐ophthalmology
          Neurofibromatosis types 1 and 2 (Chapters 16 and 26)
          Von Hippel–Lindau disease (Chapter 26)
          Tuberous sclerosis
          Sturge–Weber syndrome (encephalo‐trigeminal angiomatosis)
     Abnormalities of eye movements
          Diplopia
          Orbital disease
               Thyroid ophthalmopathy
          Orbital inflammatory syndromes
          Cavernous sinus thrombosis
          Carotico‐cavernous 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
               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
     Nystagmus
          Horizontal nystagmus
          Nystagmus in normal subjects
          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
               Downbeat nystagmus
               Upbeat nystagmus
               Nystagmus in oculopalatal tremor
               See‐saw nystagmus
               Oculomasticatory myorhythmia
               Periodic alternating nystagmus
               Convergence–retraction nystagmus in Parinaud’s syndrome
               Voluntary nystagmus
               Eye lid 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 stato‐kinetic 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
               Pulfrich’s phenomenon
               Hemifield slide
               Tilt
               Visual synaesthesia
               Visual agnosia
               Prosopagnosia
               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
               Horner’s syndrome
     References
     Further reading
          General texts
          Unilateral and bilateral visual failure
          Optic nerve disease
          Ocular involvement in other neurological diseases
          Diplopia
          Central disorders of eye movements
          Nystagmus
          Chiasmal and retrochiasmal disorders
          Disorders of higher visual function
          Pupils
     Dizziness and vertigo: introduction
     Epidemiology
15: Neuro‐Otology: Problems of Dizziness, Balance and Hearing
     Basic concepts
          Three‐dimensional spatial orientation
               Vestibulo‐ocular reflexes
          Encoding of head movements in space
     Neuro‐otological assessment
          Clinical examination
               What to examine
               When to examine what – the clinical presentation of balance disorders
          What to examine
               Brief neurological examination
               Gait and posture
               Nystagmus, eye movements, positional testing
               Cover test
               Spontaneous nystagmus
               Gaze evoked nystagmus
               Congenital nystagmus
          Smooth pursuit eye movements
          Saccadic eye movements
               Optokinetic nystagmus
               Vestibulo‐ocular reflexes
               Doll’s head eye manoeuvre
               Dynamic visual acuity
               Head impulse test
               Vestibulo‐ocular reflex suppression
               Positional manoeuvres, Dix–Hallpike and roll manoeuvres
               Orthostatic blood pressure
          When to examine what – the clinical presentation of balance disorders
               Acute vertigo
               Recurrent vertigo
               Chronic dizziness and/or unsteadiness
     Commonly used vestibular investigations
          Electronystagmograph
               Clinical relevance of ENG
               Rotary chair testing
               Types of rotary chair stimuli
               Clinical relevance of rotary chair testing
          Video‐oculography
          Caloric testing
               Principles of caloric testing
               Quantitative analysis
               Closed‐circuit and air caloric testing
               Clinical value of caloric testing
          Posturography
               Vestibular evoked myogenic potentials
     Clinical disorders
          Vestibular neuritis
               Aetiology
               Clinical assessment
               Neuro‐otological investigations
               Course
               Differential diagnosis of vestibular neuritis
          Benign paroxysmal positional vertigo
               Incidence of BPPV
               Posterior semicircular canal BPPV
               Horizontal canal BPPV – geometric type
               Apogeotropic horizontal canal BPPV
               Anterior canal BPPV
          Migraine‐related dizziness
               Definitions
               Basilar migraine
               Vestibular migraine
               Benign recurrent vertigo
          Ménière’s disease
          Bilateral vestibular failure
          Vestibular paroxysmia
          Motion sickness
     Management of vestibular disorders
          Drug treatment
               Symptomatic treatment of acute vestibular symptoms
               Specific treatment of vestibular disorders
               Migraine
               Episodic ataxia
               Central vestibular dysfunction
          Treatment of chronic peripheral vertigo
               Vestibular rehabilitation physiotherapy
               Particle repositioning procedures
               Psychological treatment
               Surgical management of vertigo
     Hearing disorders
          Anatomy and physiology
          Definitions and introduction
          Basic concepts
               Conductive hearing loss
               Sensorineural hearing loss
               Auditory neuropathy
               Brainstem auditory dysfunction
               Auditory processing disorder
     Clinical examination of the ear and hearing
          Otoscopy
               Auricle (pinna)
               External auditory meatus
               Tympanic membrane and middle ear
               Tuning fork tests
     Audiological investigations
          Baseline audiometric tests
               Pure‐tone audiometry
               Acoustic impedance measurements
               Speech audiometry
          Electro‐acoustic and electrophysiological tests
               Oto‐acoustic emissions
               Cochlear microphonics and electro‐cochleography
               Acoustic brainstem evoked responses
               Middle latency response
               Cortical‐evoked auditory responses
     Aetiology of hearing loss
          Conductive hearing loss
               Disorders of the tympanic membrane and middle ear
          Sensorineural hearing loss
               Genetic hearing loss
               Metabolic disease
               Drugs
               Acoustic trauma
               Autoimmune disorders (Table 15.12)
          Retro‐cochlear hearing disorders
               Charcot–Marie–Tooth disease
               Neurofibromatosis type 2
               Friedreich’s ataxia
               Refsum’s disease
               Mitochondrial disorders
               Inherited muscle disorders
               Acquired retro‐cochlear hearing disorders (Table 15.14)
               Extrinsic and intrinsic tumours of the cerebellopontine angle
               Multiple sclerosis
               Sarcoidosis
               Vascular disease
               Superficial siderosis
     Auditory processing disorders
          Aetiology of APD
               Cortical hearing impairment
               Auditory agnosia
               Interhemispheric lesions
     Management of auditory disorders
          Hearing aids
          Conductive hearing loss
          Sensorineural hearing loss
          VIIIth nerve disorders
               Amplification and rehabilitation strategies
          Auditory processing disorders
               Environmental modifications
               Signal enhancement strategies
               Relatives and carers: speaker‐based adaptations
               Auditory training programmes and compensatory strategies
     References
     Further reading
16: Spinal Column and Spinal Cord Disorders
     Spinal embryology, anatomy and physiology
          Embryology of the spine
          Genetic control of spinal development
          Identifying genetic mutations involved in spinal anomaly
     Anatomy and physiology of spinal maldevelopment
          Disorders of axonal guidance
               Congenital mirror movements
          Human vertebral segmentation defects
     Diagnosis of spinal column and spinal cord disorders
          Clinical assessment of spinal disorders
               Craniocervical junction
               Syringobulbia and syringomyelia
               Cervical spine
               Thoracic spine
               Lumbar and sacrococcygeal spine
               Other clinical features of spinal disease
               Diagnosis of spinal tumours
               Clinical features of vascular disorders of the spine
               Diagnosis of spinal vascular disease
          Radiological assessment of spinal disorders
     Specific diseases of the spinal column and spinal cord and their management
          Skeletal disorders affecting the spine
               Idiopathic scoliosis
               Congenital scoliosis
               Neuromuscular scoliosis
               Kyphosis and lordosis
               Miscellaneous causes of spinal deformity
               Management of spinal deformity
               Surgical correction of spinal deformity
               Cranio‐cervical junction anomalies
               Down’s syndrome
               Chiari malformations
               Congenital basilar invagination
               Os odontoideum
               Management of craniocervical junction anomalies
               Syringomyelia
               Spinal dysraphsim
               Klippel–Feil syndrome
               Rheumatological disorders affecting the spine and spinal cord
               Indications for surgical management of the rheumatoid spine
               Spondyloarthropathies
               Spinal trauma
               Acute management of spinal injury
               Spinal tumours
               Degenerative disease of the spine
               Low back pain and degenerative disc disease
               Spinal infections
               Spinal cord inflammation
               Vascular disorders of the spine
               Management of spinal vascular disease
               Metabolic diseases of the spinal cord
               Hereditary spastic paraplegia
               Miscellaneous conditions affecting the spine and spinal cord
     Further reading
          Physiology
          Spinal cord development and repair
          Spinal cord trauma
          Spinal tumours
          Rheumatoid diseases
          Infections
          Other medical conditions affecting the spinal cord
          Scoliosis
          Other surgical
          Degenerative spine disease
17: Cerebellar Ataxias and Related Conditions
     Approach to the patient with ataxia
          Symptoms
          Physical signs
               Additional signs
     The ataxic disorders
     Inherited ataxia syndromes
          Congenital ataxias
          Autosomal recessive cerebellar ataxias
               Friedreich’s ataxia
               Ataxic disorders associated with defective DNA repair
               Ataxias associated with oculomotor apraxia
               Ataxia caused by vitamin E deficiency
               Autosomal recessive spastic ataxia of Charlevoix‐Saguenay
          Other metabolic causes of ataxia
               Intermittent metabolic ataxias
               Progressive metabolic ataxias
               Autosomal dominant cerebellar ataxias
               Dentato‐rubro‐pallido‐luysian atrophy
          Investigations
          Treatment
          Genetic forms of episodic ataxia
               Episodic ataxia type 1
               Episodic ataxia type 2
               Other episodic ataxias
          X‐linked ataxia syndromes
          Mitochondrial ataxia syndromes
          Next generation gene sequencing
     Acquired ataxia syndromes
          Infective disease
               Acute or subacute onset
               Progressive ataxia with a chronic or subacute course
          Inflammatory disease
          Vascular disease
               Vascular anomalies
          Acquired metabolic disorders
          Toxins and physical agents
               Ethyl alcohol (ethanol)
               Drugs
               Solvents and solvent abuse
               Heavy metals
               Physical agents
     Paraneoplastic cerebellar degeneration
     Late onset cerebellar degenerations
          Multiple system atrophy
          Ataxia and sensitivity to gluten
          Idiopathic late onset ataxia
     Conclusions
     References
     Further reading
18: Restorative Neurology, Rehabilitation and Brain Injury
     The nature of disability and its optimal management
     Key aspects of multi‐dimensional rehabilitation
     Natural histories of neurological damage
          Disease/Health condition (organ disorder/pathology) (ICD-10) Subjective quality of life and well-being
          Impairments
          Activities
          Participation
     Resources, integrated care pathways, effectiveness and evidence base
     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
               Bobath approach and Motor Relearning Programme
               Impairment based treatment techniques
               Constraint induced movement therapy
               Balance and posture
               Treadmill training, functional electrical stimulation, cueing, fitness and mental imagery
               Robotics and virtual reality
          Language disorders: speech and language therapy and communication aids
          Visual loss and restorative therapies
          Cognitive impairments
               Environmental manipulation and compensatory strategies
               Restorative approaches
               Executive function, retraining and behavioural modification
     Telerehabilitation
     Vocational rehabilitation
          Medical treatments for specific problems
               Spasticity
               Ataxia
               Pain
               Bladder, bowel and sexual dysfunction
               Fatigue
               Dysphagia
               Neuropsychiatric problems
     Single incident brain injury
          Stroke
          Traumatic brain injury
     Service delivery
          Organisational behaviours and interdisciplinary assessment
               Assessment
               Goal‐setting
     Outcome measurement
          Indices of outcome
          Service quality
     Conclusions
     Further reading
          General texts
          Epidemiology
          Guidelines
          Restorative neurology
          Skill learning and task‐related training
          Physical therapeutic interventions for motor disorders
          Language disorders
          Visual loss
          Cognitive impairments
          Telerehabilitation
          Medical treatments
          Single incident brain injury Stroke
          Traumatic brain injury
          Organisational behaviours
19: Toxic, Metabolic and Physical Insults to the Nervous System and Inherited Disorders of Metabolism
     Neurological disorders associated with exposure to toxic substances
          Heavy metals
               Lead
               Mercury
               Arsenic
               Manganese
               Aluminium
               Thallium
               Tin
               Bismuth
          Solvents and toxins
               Toluene
               Trichloroethylene and tetrachlorethylene
               Ethylene oxide
               Hexacarbon solvents
               Xylene and styrene
               Carbon disulfide
               Cyanide
               Acrylamide and acylonitrile
               Allyl chloride
               Methyl bromide
               Methyl chloride
               Methyl alcohol (Methanol)
               Nitrous oxide
               Organophosphates
               Carbon monoxide
          Marine toxins
               Ingested toxins
          Other biological toxins
               Snake venom
               Spider toxins
               Scorpion toxin
               Ticks
               Fungal poisons
               Lathyrism
               Konzo
               Subacute myelo‐optico neuropathy
               Tropical myelo‐neuropathy
     Radiation‐induced neurological disease
          Therapeutic radiation
          Early‐delayed radiation encephalopathy
          Late‐delayed radiation encephalopathy
          Transient radiotherapy myelopathy
          Radiation plexopathy
     Lightning and electrical damage to the nervous system
          Mechanisms of lightning and other electrical damage
               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
               Cardiac
               Skin and muscle damage
               Management
     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 pulmonary oedema
     Neurobiological weapons
          Modes of release
          Nerve agents
               Organophosphates
               Other toxins and poisons
     Vitamin deficiencies and toxicity
          Vitamin A
          Vitamin B
          thiamine)
          Vitamin B
          niacin, nicotinic acid)
          Vitamin B
          pyridoxine)
          Vitamin B
          deficiency (see Chapter 16)
               Subacute combined degeneration of the cord
          Folate deficiency
          Vitamin D
          Vitamin E
     Alcohol abuse
          Metabolism of alcohol
          Effects of acute intoxication
          Effects of alcohol substitutes
               Methyl alcohol (methanol)
               Ethylene glycol
          Withdrawal syndromes
               Withdrawal seizures (‘rum fits’)
               Management of alcohol withdrawal
          Chronic disorders associated with prolonged alcohol abuse
               Wernicke’s encephalopathy
               Korsakoff’s syndrome
               Cerebellar ataxia
               Confusional state and dementia
               Alcoholic peripheral neuropathy
               Alcoholic myopathy
          Other neurological complications of alcohol abuse
               Marchiafava–Bignami syndrome
               Fetal alcohol syndrome
               Psychiatric sequelae
               Traumatic injury
               Compressive neuropathies (see Chapter 10)
               Amblyopia (see Chapter 14)
               Alcoholic cirrhosis
               Strachan’s syndrome
     Other deficiency states associated with neurological manifestations
          Copper deficiency
          Magnesium deficiency
     Drugs of abuse
          Epidemiology
          Stimulants
               Amphetamines
               Designer drugs
               Methylphenidate (Ritalin)
               Methcathinone analogues – khat
               MPTP
               Cocaine
               Conditions caused by stimulant abuse
          Sedatives
               Opiates
               Barbiturates
               Benzodiazepines
          Hallucinogens
               Lysergic acid diethylamide
               Marijuana
               Ketamine
               Phencyclidine (angel dust)
               GABA hydroxybutyrate
               Anticholinergics
          Solvents
          Athletic performance‐enhancing drugs
          Investigation of suspected substance abuse
     Adverse reactions to drugs
          Seizures
          Headache
          Confusional states
          Encephalopathy
          Memory disturbance
          Neuropsychiatric effects
          Coma
          Sleep disorders
          Toxic leukoencephalopthy
          Cerebrovascular disease
          Impairment of taste and small
          Drug‐induced movement disorders (Chapter 6)
          Ototoxicity
          Cerebellar disorders
          Visual disorders
          Autonomic effects
          Neuromuscular drug effects
               Peripheral neuropathy
               Drugs interfering with neuromuscular transmission
               Myalgia, stiffness and cramp
          Malignant hyperthermia
          Neuroleptic malignant syndrome
          Serotonin syndrome
          Tyramine cheese reaction
          Anticholinergic syndrome caused by medication toxicity
     Inherited disorders of metabolism
     Porphyria
          Acute intermittent porphyria
               Diagnosis
               Management
          Disorders of amino acid metabolism
               Phenylketonuria
          Organic acidemias
               Maple syrup urine disease
               Isovaleric acidemia
               Propionic acidemia and methylmalonic acidemia
          Disorders of homocysteine metabolism
               Transsulfuration defects
          Remethylation Trans-sulfuration
               Defects in intracellular cobalamin metabolism
          Urea cycle disorders
               Diagnosis
               Management
               Arginase deficiency
          Disorders of carbohydrate metabolism
               Glycogen storage diseases (the GSDs)
               Hepatic involvement in glycogen storage diseases
               Skeletal/cardiac muscle involvement in glycogen storage diseases
               Central nervous system involvement in glycogen storage diseases
               Galactosaemia
          Fatty acid oxidation defects
               Diagnosis
               Management
          Neurotransmitter disorders
               Clinical presentations
               Laboratory diagnosis
               Management
          Lysosomal storage disorders
               Glycosphingolipidoses
     Adult onset inherited leukodystrophies
          X‐linked adrenoleukodystrophy
          Krabbe disease
          Cerebrotendinous xanthomatosis
          Metachromatic leukodystrophy
          Hereditary diffuse leukoencephalopathy with neuroaxonal spheroids
          Alexander’s disease
          Vanishing white matter disease
          Pelizaeus–Merzbacher disease and Pelizaeus– Merzbacher‐like disease
          Peroxisomal disorders
               Refsum disease
          Disorders of phospholipid and glycosphingolipid biosynthesis
               Disorders of phospholipid synthesis
               Disorders of glycosphingolipid synthesis
          Acute neurological emergencies caused by inherited metabolic disease
     References
     Further reading
          Toxins
          Metabolic disorders
          Physical insults
          Drugs of abuse
          Inborn errors of metabolism
20: Disorders of Consciousness, Intensive Care Neurology and Sleep
     Consciousness
          States of impaired consciousness
          Causes of coma
          Initial assessment and management of coma
               Medical assessment
               Examination
               Level of consciousness
               Glasgow Coma Scale
               Assessment of neurological function
               Distinction of metabolic and toxic coma from structural coma
               Psychogenic unresponsiveness
          Outcome from coma
               Aetiology
               Depth of coma
               Duration of coma
               Presence of seizures
               Co‐morbidity
               Investigations
          Locked‐in syndrome
          Vegetative state
          Minimally conscious state
     Determining brain death
          Repetition of testing
     Neurological intensive care
          Indications for intensive care management of neurological patients
          Ventilatory failure associated with neurological disease
          Neurological indications for tracheal intubation and mechanical ventilation
               Mechanical ventilation
               Weaning
     Conditions requiring neurointensive care support
          Raised intracranial pressure
               Measurement of intracranial pressure
               Indications for intracranial pressure monitoring
               Management
          Cerebral herniation
               Herniation of the temporal lobe through the tentorium (uncal herniation)
               Central herniation of the brainstem
               Subfalcine herniation
               Upward transtentorial herniation
               Tonsillar herniation
          Traumatic brain injury
               Skull fractures
               Extradural haematoma
               Subdural haematoma
               Traumatic subarachnoid haemorrhage
               Haemorrhagic contusions and lacerations
               Intracerebral haematoma
               Diffuse axonal injury
               Cerebral ischaemia
               Intensive care management of TBI
          Hydrocephalus and shunts
               Investigations
               Management
          Stroke (Chapter 5)
               Middle cerebral artery occlusion
               Acute basilar occlusion
               Artery of Percheron
               Cerebellar infarcts
               Subarachnoid haemorrhage
               Supratentorial intracerebral haemorrhage (basal ganglia or lobar)
               Infratentorial intracranial haemorrhage (cerebellar or brainstem)
               Cerebral venous thrombosis
          Seizures (Chapter 7)
               Status epilepticus (SE)
          Acute bacterial meningitis
          Herpes simplex encephalitis
          Autoimmune encephalopathy
               Limbic encephalitis associated with neuronal surface antigens
               Morvan’s syndrome
               Encephalitis due to NMDA receptor antibodies
               Steroid responsive encephalopathy
               Other antibody‐mediated encephalomyelopathies
          Metabolic encephalopathy
               Septic encephalopathy
               Uraemic encephalopathy
               Hepatic encephalopathy
               Hyperpyrexia and hyperthermia
               Hypernatraemia
               Hyponatraemia
               Cerebral salt wasting
               Central pontine myelinolysis
          Posterior reversible encephalopathy syndrome
          Hypoxic–ischaemic brain injury
               Prognostic factors following cardiac arrest
               Seizures
               Longer‐term complications of HIBI
          Movement disorder emergencies
               Stiff person syndromes and progressive encephalopathy with rigidity and myoclonus
               Acute dystonia
               Stridor
          Cervical cord disorders (Chapter 16)
          Anterior horn cell disease
          Neuromuscular disease
               Neuropathies (Chapter 10)
               Neuromuscular junction disease
               Muscle disease
          Tetanus (Chapter 9)
          Rabies (Chapter 9)
     General medical care on the NICU
          Nosocomial infection and infection surveillance
          Anticoagulation
          Delirium
          Patient comfort
               Pain
               Communication
               Sleep
               Communication with the family
          End of life issues on intensive care
     Neurology of general critical care
          Failure to awaken/depressed conscious state
          Weakness and failure to wean from mechanical ventilation
     Sleep and its disorders
          Structure of normal sleep
          REM 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
          Hypersomnias of central origin
               Primary (idiopathic) hypersomnia
               Recurrent hypersomnia
               Idiopathic recurring stupor
               Excessive daytime somnolence in neurological disease
          Narcolepsy
               Cataplexy
               Hypnogogic/hypnopompic hallucinations
               Sleep paralysis and automatic behaviours
               Pathophysiology of narcolepsy
               Investigations
               Management of narcolepsy
          Circadian rhythm disorders
               Delayed or advanced sleep phase syndrome
               Shift work and jet lag
          Parasomnias
               Non‐REM parasomnias
               REM sleep disorders
               Other forms of parasomnia
          Sleep‐related movement disorders
               Restless legs syndrome
               Periodic limb movements of sleep
               Rhythmic movement disorders
               Isolated symptoms, apparently normal variants
               Other sleep disturbances in extrapyramidal disease
          Epilepsy syndromes associated with sleep
          Traumatic brain injury and sleep
     Acknowledgement
     References
     Further reading
          States of impaired consciousness
          Brain death
          Neurological intensive care
          Encephalopathy
          Neuromuscular
          Sleep
21: Neuro‐Oncology
     Epidemiology of common primary intracranial tumours
          Incidence
          Survival
          Risk factors
     Clinical features
          Headache
          Seizures
          Focal deficits
          Brainstem symptoms
          Cognitive and behavioural symptoms
          Endocrine symptoms
          Rare presentations
     Histogenesis of brain cancer
          Molecular mechanisms involved in tumour formation
          Genetic components in oncogenesis
          Epigenetic events in oncogenesis
     WHO classification of CNS tumours
          Neuroepithelial tumours
     Imaging of brain tumours
          Structural imaging
          Physiological imaging
               Diffusion‐weighted imaging
               Perfusion‐weighted imaging
               Permeability imaging (Ktrans)
               MR spectroscopy
               Functional MRI
          Applications of physiological imaging
               Distinguishing between astrocytomas and oligodendrogliomas
               Distinguishing between low‐grade and high‐grade gliomas
               Imaging of peri‐tumoural tissue
          Monitoring tumour growth and response to treatment
          Pseudoprogression and pseudoresponse
          Imaging the effects of radiotherapy
               Radiation‐induced leukoencephalopathy
               Radiation necrosis
               Optic neuropathy
               Radiation‐induced tumours
          Imaging complications of chemotherapy
     Multidisciplinary management of brain tumours
          Improving outcome and quality of life
          Surgical management
               General principles
               Surgical instrumentation and methods
          Radiotherapy
               Radiotherapy planning
               Radiation neurotoxicity
     High‐grade gliomas
          Pathology
               Anaplastic astrocytoma
               Glioblastoma
          Imaging
               Anaplastic astrocytoma
               Glioblastoma
          Surgery
          Radiotherapy
          Chemoradiation
          Chemotherapy
               Relapsed high‐grade gliomas
               Anaplastic oligodendrogliomas
     Low‐grade gliomas
          Pathology
               Pilocytic astrocytoma
               Diffuse astrocytoma
               Oligodendroglioma
               Oligoastrocytoma
          Imaging
               Pilocytic astrocytoma
               Diffuse astrocytoma
               Oligodendroglioma
          Surgery for low‐grade gliomas
               Pilocytic astrocytoma
               Diffuse astrocytoma and oligodendroglioma
          Radiotherapy for low‐grade gliomas
          Chemotherapy for low‐grade gliomas
     Other low‐grade tumours in childhood
          Tumours of predominantly neuronal cell origin
               Gangioglioma and gangliocytoma
               Central neurocytoma
               Dysembryoplastic neuroepithelial tumour
          Choroid plexus tumours
     Meningiomas
          Pathology
          Imaging
          Surgery
          Radiotherapy
               Chemotherapy
     Brain metastases
          Pathology
          Imaging
          Surgery and/or radiotherapy
          Chemotherapy
     Primary spinal cord tumours
          Surgery
          Chemotherapy for intrinsic cord tumours
          Spinal ependymomas
          Spinal astrocytomas
          Spinal meningiomas and schwannomas
          Metastatic spinal cord compression
     Skull base tumours
          Chordoma
               Pathology
               Imaging
               Treatment
          Chondrosarcoma
     Neurofibromatosis and schwannomas
          Pathology
          Molecular genetics
          Clinical features
     Skull base meningiomas
     Pituitary tumours
          Biological behaviour
          Size
          Histology
          Functional criteria
          Clinical presentation
               Pituitary apoplexy
          Drug therapy and surgery
          Radiotherapy
     Craniopharyngioma
     Primary CNS lymphomas
          Imaging
          Pathology
          Clinical presentation
          Diagnosis
          Treatment
     Primitive neuroepithelial tumours and medulloblastomas
          Pathology
          Molecular genetics and histogenesis of medulloblastoma
          Imaging
          Clinical features
          Treatment
     Pineal region tumours
          Introduction
          Pathology
          Clinical features
          Treatment
          Germ cell tumours
          Optic pathway glioma
               Treatment of optic pathway glioma
          Ependymomas
               Pathology
               Imaging
               Treatment
          Dermoids and epidermoid cysts
               Imaging
          Haemangioblastomas
          Colloid cysts, Rathke’s pouch tumours and neuro‐enteric cysts
     Neurological complications of cancer
          Direct effects and infiltration
          Metastases
          Malignant meningitis
               Clinical features
               Diagnosis
               Treatment
          Indirect effects of cancer
               Toxic and metabolic encephalopathy
               Vascular disorders
               Non‐bacterial thrombotic endocarditis
          CNS infections in cancer patients
     Paraneoplastic neurological disorders
          Incidence and prevalence
          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 neurone syndromes
               Paraneoplastic sensory neuronopathy
               Paraneoplastic neuropathies
               Neuromyotonia
               Lambert–Eaton myasthenic syndrome
               Myasthenia gravis
               Polymyositis and dermatomyositis
               Acute necrotising myopathy
          Diagnosis of PND
               Paraneoplastic antineuronal autoantibodies
               Imaging
          Treatment for PND
          Neurological complications of chemotherapy
               Polyneuropathy
               Encephalopathy
     Further reading
          Epidemiology
          Clinical features
          Pathology
          Imaging
          Surgery, radiotherapy and chemotherapy
          Quality of life
          Paraneoplastic neurological syndromes
          Malignant meningitis
          Brain tumour charities and support services
22: Neuropsychiatry
     The mental state examination
          Appearance and behaviour
          Speech
          Mood and affect
          Thoughts
               Formal thought disorder
               Thought content
          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 change
          Obsessions and compulsions
          Anxiety
          Mood
          Pseudodementia
          Psychosis
          Catatonia
     Non‐organic disorders in neurology
          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
     Psychiatric disorders of epilepsy
          Peri‐ictal and ictal psychiatric symptoms
          Inter‐ictal psychiatric disorders
          Forced normalisation
          Personality changes in epilepsy
          Psychotropic effects of anticonvulsants
     Neuropsychiatric aspects of movement disorders
          Parkinson’s disease
               Depression
               Anxiety
               Apathy
               Psychosis
               Cognitive impairment
               Iatrogenic neuropsychiatric problems
          Other movement disorders
     Neuropsychiatric aspects of white matter disorders
     Minor head injury and post‐concussion syndrome
     Acknowledgement
     Further reading
          General
          Functional neurological symptoms
          Epilepsy
          Movement disorders
23: Pain
     Definitions
     Mechanisms of neuropathic pain
          Peripheral neuropathy
               Abnormal ion channel expression
          Spinal cord
               Central sensitisation and ‘wind‐up’
               Excitatory mechanisms
               Inhibitory mechanisms
               Role of inflammation and the immune system
          Supraspinal influences
     Assessment of pain in the patient with neurological disease
          The pain history
          Treatment history
          Physical examination
     Central spinal pain: some anatomical and pathological considerations
     Pain in individual disorders of the central nervous system
          Multiple sclerosis
               Neuropathic paroxysmal pain
               Neuropathic non‐paroxysmal pain
               Nociceptive pain
          Parkinson’s disease
               Neuropathic (central) pain
               Dystonia/dyskinesia pain
          Central post‐stroke pain
          Spinal cord injury
          Syringomyelia
          Phantom pain
          Painful legs and moving toes syndrome
          Epilepsy
          Fibromyalgia
     Peripheral pain: anatomical and pathological considerations (Chapter 2)
     Pain in individual disorders of the peripheral nervous system
          Painful peripheral neuropathies
          Burning feet, and the small‐diameter fibre neuropathies
          Erythromelalgia
               Treatment
          Neuropathy in diabetes mellitus (Chapter 10)
          Shingles and post‐herpetic neuralgia: shingles
          Post‐herpetic neuralgia
          Guillain–Barré syndrome
          Neuralgic amyotrophy (brachial neuritis)
          Painful inherited neuropathies
          Latrogenic painful peripheral nerve lesions
          Complex regional pain syndrome
     Pain associated with benign orthopaedic conditions
          Glomus tumour
          Osteoid osteoma
     Motor neurone disease
     Painful peripheral viscero‐somatic disorders
          Burning mouth syndrome
          Vulvodynia
          Visceral pain
     Plexopathies
          Traumatic brachial plexus lesions
          Malignant and radiation‐induced plexopathies
     Management
          Management of neuropathic pain
               Systemic drug treatments
               Opioids and other analgesics
               Antidepressants
               Antiepileptics
               Local anaesthetics and related compounds
               NMDA receptor antagonists
               Cannabinoids
               Topical agents
               Botulinum toxin
          Intrathecal drugs
          Neuro‐ablative and neuro‐stimulation procedures
               Neuro‐ablative procedures
               Neuro‐stimulation procedures
          Other physical methods of treatment
               Acupuncture
          Psychological approaches to management of chronic pain
               The placebo phenomenon
          Insensitivity to pain
               Congenital insensitivity to pain
               Transient indifference to pain
     Conclusions
     Acknowledgement
     References
     Further reading
          General
          Classic papers on pain
          Clinical assessment
          Specific Conditions
          Treatment and the placebo response
24: Autonomic Aspects of Neurology
     Classification of autonomic dysfunction
     Clinical features
          Cardiovascular system
               Orthostatic hypotension
               Syncope without orthostatic hypotension
               Orthostatic intolerance with posturally induced tachycardia
               Hypertension
               Heart rate disturbances
               Facial and peripheral vascular changes
          Sudomotor system
          Alimentary system
          Kidneys and urinary tract
          Sexual function
          Eyes and lacrimal glands
          Respiratory system
          Additional features of neurological conditions: MSA and Parkinson’s disease
          Psychological and psychiatric disturbances
     Clinical examination
     Investigations
     Management
          Cardiovascular system
               Orthostatic hypotension
               Supine hypertension
               Autonomic mediated syncope
               Postural tachycardia syndrome
               Hypertension
          Sudomotor disorders
               Anhidrosis
               Hyperhidrosis
          Alimentary system
          Urinary tract
          Sexual function and the reproductive system
          Respiratory system
          Eye and lacrimal glands
     Treatment in MSA and Parkinson’s disease
     References
     Further reading
25: Uroneurology
     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
          Brainstem lesions
          Spinal cord disease
               Multiple sclerosis
               Other (non‐traumatic) spinal cord diseases
          Sacral and infrasacral lesions
               Conus or cauda equina lesions
               Diabetic neuropathy
               Other neuropathies
               Pelvic nerve injury
               Myotonic dystrophy
          Urinary retention
               Urinary retention in women
     Management of lower urinary tract dysfunction
          Management of storage dysfunction
               Antimuscarinics (anticholinergics)
               Desmopressin
               Botulinum toxin
               Neuromodulation
          Management of voiding dysfunction
     Sexual functions and its neurological control
          Physiology
          Sexual dysfunction following neurological disease
               Traumatic brain injury
               Stroke
               Epilepsy
               Parkinson’s disease
               Multiple system atrophy
               Spinal cord injury
               Multiple sclerosis
               Lesions of the sympathetic thoraco‐lumbar outflow
               Conus or cauda equina lesions
               Peripheral neuropathies
          Management of sexual dysfunction
     References
     Further reading
26: Systemic Conditions and Neurology
     Aortic and cardiac disorders
          Aortic pathology
               Anatomy
               Blood supply
               Cerebral ischaemia resulting from aortic disease
               Spinal cord ischaemia resulting from aortic disease
          Neurological complications of cardiac surgery
          Neurological complications of acquired cardiac disease
               Cardiac embolism
               Rhythm disturbances
               Cardiomyopathies
               Valve disease
               Atrial myxoma
     Endocrine conditions
          Thyroid disorders
               Hyperthyroidism
               Hypothyroidism
               Neurological aspects of Hashimoto’s thyroiditis
          Diabetes mellitus
               Acute metabolic disturbances
               Diabetic neuropathies
          Pituitary disorders
               Inappropriate antidiuretic hormone secretion and cerebral salt wasting
          Parathyroid disorderss
               Adrenal disorders
     Electrolyte disturbances
          Sodium
          Potassium
          Calcium and magnesium
     Haematological disorders
          Anaemias
          Proliferative conditions
               Leukaemias
               Plasma cell dyscrasias
               Lymphomas
               Langerhans cell histiocytosis
          Polycythaemia
          Thrombocythaemia
          Bleeding disorders
               Thrombotic thrombocytopenic purpura
               Coagulation disorders
               Primary immunodeficiency
     Gastrointestinal disorders
          Hepatic encephalopathy
          Vitamin deficiencies causing neurological disorders
               Vitamin B
               deficiency
               Vitamine B
               niacin) deficiency (Pellagra)
               Vitamin D deficiency
               Vitamin E deficiency
          Malabsorption
               Coeliac disease
          Inflammatory bowel disease
     Renal disease
          Conditions affecting both renal and neurological function
          Neurological consequences of renal disease and its treatment
               Uraemic encephalopathy
               Dialysis encephalopathy
               Dialysis disequilibrium syndrome
               Neuropathy associated with renal disease
     Neurological aspects of organ transplantation
          CNS infections
               Viral infection
               Bacterial infection
               Fungal infection
               Parasitic infection
          Neurological sequelae of transplantation
               Seizures
               Encephalopathy
               Stroke
               Medication
               CNS malignancy
               Neuro‐ophthalmological problems
               Movement disorders
               Neuromuscular problems
          Complications related to specific allograft transplantation
               Renal transplantation
               Liver transplantation
               Cardiac transplantation
               Lung transplantation
               Bone marrow transplantation
     Neurological involvement in systemic vasculitides and related disorders
          Pathological mechanisms
          Diagnosis and treatment of vasculitides involving the nervous system
               Polyarteritis nodosa and related conditions
               Granulomatosis with polyangiitis (Wegener’s granulomatosis)
               Giant cell arteritis
               Isolated cerebral angiitis
               Rheumatoid arthritis
               Systemic lupus erythematosus
               Antiphospholipid syndrome
     Sjögren’s syndrome
     Miscellaneous cerebral arteriopathies
          CADASIL
          CARASIL
          Fabry’s disease
          Susac’s syndrome
          Sneddon’s syndrome
          Degos’ disease
          HANAC
          Reversible cerebral vasoconstriction syndrome
     Sarcoidosis
          Clinical features and investigation
          Cranial neuropathy
          Meningeal and parenchymatous sarcoid
          Sarcoid encephalopathy
          Peripheral neuromuscular sarcoid
          Diagnosis
          Prognosis
     Behçet’s syndrome
          Epidemiology and pathology
          Patterns of nervous system involvement
          CNS involvement: parenchymal
          Cerebral venous sinus thrombosis
          Investigation
               Pathergy test in Behçet’s disease
          Treatment
     IgG4‐related disease
     CLIPPERS
     Neurocutaneous syndromes
          Neurofibromatosis type 1 (von Recklinghausen’s disease)
          Neurofibromatosis type 2
          Xeroderma pigmentosa
          Tuberous sclerosis
          Von Hippel–Lindau disease
          Ataxia telangiectasia
          Sturge–Weber syndrome
     Neurological aspects of pregnancy
          Epilepsy and women of childbearing age
               Fertility
               Pregnancy
               Epilepsy and the fetus
               Teratogenicity of antiepileptic drugs
               Puerperium
               Reducing risks of pregnancy to mother and child
               Cerebrovascular disorders in pregnancy
          Pregnancy and other neurological diseases
               Pituitary disorders
               Headache
               Neuromuscular disorders
               Multiple sclerosis
               Chorea gravidarum
               Tumours
               Idiopathic intracranial hypertension
          Complications of obstetric anaesthesia
               Postpartum compression neuropathies
          Other conditions causing acute neurological symptoms during pregnancy
     Palliative and end of life care in neurology
          The dying patient
     References
     Further reading
          Cardiovascular disorders
          Endocrine disorders
          Haematological disorders
          Gastro‐intestinal disorders
          Renal disease
          Vasculitis
          Neurosarcoidosis, Sjögren
          Behçet’s syndrome
          Neurocutaneous disorders
          Pregnancy
          Palliative care
Index