700 Essential Neurology Checklists

Description

700 Essential Neurology Checklists is a collection of essential checklists which provide handy, practical, comprehensive, and evidence-based information on every aspect of neurology. The checklists cover all aspects of neurology and its allied specialties, and they were developed to minimise error and boost clinical safety in the care of neurological patients. The lists included in the book apply to all aspects of neurology, a specialty noted for its size, diversity, and complexity, and they place emphasis on all aspects of neurological practice, from history and clinical examination to investigations and treatment. They also contain details of aetiology, epidemiology, genetics, and pathology. The information is primarily sourced from widely regarded neurology journals such as Neurology, Brain, the JNNP, Practical Neurology, and Journal of Neurology, and the emphasis is on evidence-based guidelines, review articles, ground-breaking studies, and relevant case reports. The checklists in the book will be of benefit not only to neurologists and neurology trainees, but to all medical professionals including psychiatrists, neurosurgeons, paediatricians, general physicians, obstetricians, ophthalmologists, and specialist nurses. Purchasers of the book will also be eligible for a 12-month complimentary access to more than 3,500 online neurology checklists at www.neurochecklists.com Cover Half Title Title Page Copyright Page Table of Contents Preface Acknowledgements and Dedication Introduction Chapter 1 Disorders of Cognition and Consciousness Cognitive Symptoms and Signs Cortical Release Phenomena Physiological Types Pathological Types Causes Clinical Significance Confabulation Clinical Features Types Psychiatric Causes Neurological Causes Physiological Causes Assessment Tools Pathological Laughter Clinical Manifestations Neoplastic Causes Neurological Causes Treatment Aphasia: Classification Broca’s Aphasia Wernicke’s Aphasia Transcortical Aphasia Subcortical Aphasia Conduction Aphasia: Lesion Locations Conduction Aphasia: Features Aphemia Pure Word Deafness Other Aphasia Types Akinetic Mutism Pathology Vascular Causes: Stroke Vascular Causes: Others Neoplastic Causes Traumatic Causes Infective Causes Toxic and Drug-Induced Other Neurological Causes Treatment Delirium (Acute Confusional State) Delirium: Risk Factors Individual Risk Factors Medical Risk Factors Drug-induced Risk Factors Delirium: Clinical Features Types of Delirium Cognitive and Psychiatric Features Neurological Features Delirium: Differential Diagnosis Neurological Differentials Psychiatric Differentials Medical Differentials Toxic Differentials Delirium: Management Non-drug Treatments Drug Treatments Outcome Dementia Presentations Reversible Dementia Neurological Causes Infectious Causes Metabolic Causes Other Causes Rapidly Progressive Dementia Infective Causes Neurodegenerative Causes Autoimmune and Inflammatory Causes Metabolic and Toxic Causes Neoplastic Causes Other Causes Young-onset Dementia: Causes Neurodegenerative Infective Neuroinflammatory Metabolic Vascular Toxic Other Causes Subacute Encephalopathy: Causes Neurodegenerative Infectious Neuroinflammatory and Autoimmune Metabolic Malignancy-related Toxic and Drug-Induced Other Causes Alzheimer’s Disease (AD) Alzheimer’s Disease (AD): Risk Factors Non-modifiable Risk Factors Lifestyle Risk Factors Medical Risk Factors Proposed Microbial Risk Factors Risk Factors for Accelerated Cognitive Decline Unlikely Risk Factors Alzheimer’s Disease (AD): Clinical Features Pre-clinical Features Cognitive Features Non-cognitive Features Features of Young Onset AD Alzheimer’s Disease (AD): Preventative Measures Measures With Very Strong Evidence Measures With Weaker Evidence Measures of Uncertain Benefit Measures Not Recommended Alzheimer’s Disease (AD): Non-Drug Treatments Give Written Information On Clinical and Social Issues Discuss Advanced Directives Assess Functional Impairments Non-drug Interventions Alzheimer’s Disease (AD): Drug Treatments Acetylcholinesterase Inhibitors (ACheI): Indications Acetylcholinesterase Inhibitors (ACheI): Types Memantine Other Drug Treatments Investigational Drug Treatments Frontotemporal Dementia (FTD) Behavioural Variant Frontotemporal Dementia (BvFTLD): Clinical Features Features of Disinhibition Other Behavioural Abnormalities Motor Stereotypy and Repetitive Activities Features Associated With C9orf72 Gene Mutation Neuropsychiatric Features Other Features Poor Prognostic Factors Primary Progressive Aphasia (PPA): Non-Fluent Variant (NfvPPA) Diagnostic Criteria Language Features: Relevance Language Features: Manifestations Cognitive Features Behavioural and Psychiatric Features Motor Impairment Progression to Other Syndromes Synonym Primary Progressive Aphasia (PPA): Logopenic Variant (LvPPA) Pathological Sites Genetic Mutations Clinical Features Relatively Spared Speech Functions Diagnostic Criteria: Core Features Diagnostic Criteria: Supportive Features Differentiating Features From Other PPA Variants Other Differentials Synonyms Amnestic Syndromes Acute Amnestic Syndromes Transient Amnestic Syndromes Vascular Acute Amnestic Syndromes Other Acute Amnestic Syndromes Subacute and Chronic Amnestic Syndromes Neurological Causes Systemic Syndromes Transient Global Amnesia (TGA): Risk Factors and Triggers Risk Factors Triggers: Medical Procedures Triggers: Others Transient Global Amnesia (TGA): Clinical Features Clinical Features Associated Features Exclusion Criteria for TGA Possible Predictors of Recurrent TGA Magnetic Resonance Imaging (MRI) Features Transient Global Amnesia (TGA): Differential Diagnosis Neurological Drugs Psychiatric Encephalopathy Wernicke’s Encephalopathy: Clinical Features Nutritional Risk Factors Medical Risk Factors Metabolic Risk Factors Neurological Risk Factors Other Risk Factors Clinical Features Wernicke’s Encephalopathy: MRI Features Typical Location of Lesions Unusual Location of Lesions in Non-Alcoholics Diffusion Weighted Imaging (DWI) Changes: Locations Other MRI Features Korsakoff Syndrome Risk Factors Clinical Features Magnetic Resonance Imaging (MRI) Lesions: Locations Posterior Reversible Encephalopathy Syndrome (PRES): Clinical Features Typical PRES Triad Neurological Features Visual Features Blood Pressure in PRES Recurrent PRES Possible PRES Variants Poor Prognostic Features Posterior Reversible Encephalopathy Syndrome (PRES): Risk Factors Vascular Disorders Autoimmune Disorders Medical Disorders Immunosuppressant Therapy Other Medical Interventions Posterior Reversible Encephalopathy Syndrome (PRES): Differentials Encephalitic Vascular Demyelinating Other Differentials Posterior Reversible Encephalopathy Syndrome (PRES): Management Magnetic Resonance Imaging (MRI): FLAIR Hyperintensities Magnetic Resonance Imaging (MRI): Haemorrhage Types Magnetic Resonance Imaging (MRI): Other Features Cerebrospinal Fluid (CSF) Features Treatment Osmotic Demyelination Disorders (ODD): Causes Metabolic Causes Drug-withdrawal Gastrointestinal Causes Nutritional Causes Other Causes Osmotic Demyelination Disorders (ODD): Clinical Features Types of ODD Neurological Features Movement Disorders Psychiatric Features Osmotic Demyelination Disorders (ODD): Management Magnetic Resonance Imaging (MRI) Brain Treatment of Hyponatraemia Potentially Beneficial Treatments Transient Loss of Consciousness (TLOC) Transient Loss of Consciousness (TLOC): Causes Cardiac Causes Reflex Causes Other Causes Causes of Nocturnal TLOC Transient Loss of Consciousness (TLOC): Clinical Features Features Suggestive of Syncope Features Suggestive of Seizures Features Suggestive of Cardiac TLOC Non-discriminatory Features Transient Loss of Consciousness (TLOC): Differentials Neurological Differentials Medical Differentials Cardiovascular Differentials Chapter 2 Epilepsy Seizure Risk Factors Seizures: Medical Risk Factors Neurological Infections Human Herpes Virus (HHV) Vascular Neurological Disorders Traumatic Autoimmune Metabolic Seizures: Drug-Induced Antibiotics Antidepressants and Antipsychotics Anti-epileptic Drugs (AEDs) Chemotherapy Abuse Drugs Other Drugs Seizures: Risks for Recurrence Recurrence Risk After First Seizure Subject-related Risk Factors Seizure-related Risk Factors Pathology-related Risk Factors Environmental Risk Factors Treatment-related Risk Factors Risk Factors for Poor Seizure Control at 5 Years Seizures: Clinical Features Seizures: Typical Features Seizure Prodrome Seizure Markers Inter-ictal Non-Seizure Features Seizures: Differential Diagnosis Transient Neurological Events Sleep Disorders Psychiatric Disorders Medical Disorders Hyperkinetic Disorders Paediatric Differential Diagnosis Febrile Seizures (FS): Clinical Features Risk Factors Diagnostic Criteria Types Recurrence: Epidemiology Recurrence: Risk Factors Risk Factors for Progression to Epilepsy Transient Epileptic Amnesia (TEA) Epidemiology Amnesia: Features Accelerated Long Term Forgetting (ALF) Seizures: Types Compulsive Versifying Other Reported Features Post-ictal Psychosis of Epilepsy Demographic Features Onset and Course Psychiatric Features Neurological Features Electroencephalogram (EEG) Features Treatment Myoclonus Myoclonus: Classifications and Differentials Physiological Myoclonus Epileptic Myoclonus Pathological Myoclonus Classification By Site of Origin Classification By Spread Classification By Posture Differential Diagnosis Myoclonus: Neurological Causes Neurodegenerative Infectious Hereditary Late Onset Asymmetric Myoclonus Other Causes Myoclonus: Drug-Induced Antidepressants and Antipsychotics Anti-epileptic Drugs (AEDs) Antimicrobials Anti-Parkinsonian Drugs Chemotherapy Anti-arrhythmics Other Drugs Myoclonus: Systemic Causes Organ Failure Metabolic Abnormalities Mitochondrial Disorders Thyroid Disorders Miscellaneous Causes Acronyms Major Epilepsy Types Childhood Absence Epilepsy (CAE) Epidemiology Clinical Features of Absences Electroencephalography (EEG) Treatment Contraindicated Medications Eyelid Myoclonia With Absences (Jeavon’s Syndrome) Types Epidemiology Features of Eyelid Myoclonia Other Clinical Features Differential Diagnosis Electroencephalogram (EEG): Features Treatment Contraindicated Medications Juvenile Absence Epilepsy (JAE) Clinical Features Treatment Contraindicated Medications Idiopathic GENERALISED Epilepsy (IGE) Types Genetic Mutations Seizure Types Differential Diagnosis Electroencephalogram (EEG): Features Synonym Juvenile Myoclonic Epilepsy (JME): Clinical Features Epidemiology Myoclonic Features Reflex Features Other Seizure Types Triggers for Seizures Cognitive Features Adult Onset JME Risk Factors for Refractory JME GENERALISED Epilepsy With Febrile Seizures Plus (GEFS+) Genetic Transmission Genetic Mutations Genetic Subtypes Phenotypes Clinical Features Possible Associated Features Treatment Synonym Temporal Lobe Epilepsy With Hippocampal Sclerosis (TLE-HS): Features Motor Symptoms Visual Symptoms Auditory and Gustatory Symptoms Dyscognitive and Affective Symptoms Other Symptoms Frontal Lobe Epilepsy: Clinical Features Epidemiology Pathogenesis Typical Seizures Motor Movements Vocal Features Sleep Associated Features Prognosis Synonyms Occipital Lobe Epilepsy Causes Visual Hallucinations Illusionary Distortions Other Symptoms Differential Diagnosis Status Epilepticus Convulsive Status Epilepticus: Clinical Features Definitions Types Stages Motor Features Autonomic Features Predictors of Poor Prognosis Outcome Prediction Scales Convulsive Status Epilepticus: Management Initial Treatment Options Alternative Initial Treatment Options Second Line Treatment: Intravenous After 20 Minutes Third Line Treatment: After 40 Minutes Emerging Treatments Non-convulsive Status Epilepticus (NCSE): Clinical Features Impaired Higher Brain Function Impaired Speech and Language Psychiatric Features Movement Disorders Automatisms and Autonomic Features Other Neurological Features Refractory Status Epilepticus (RSE): Classification Refractory Status Epilepticus (RSE) Super Refractory Status Epilepticus (SRSE) New Onset Refractory Status Epilepticus (NORSE) Febrile Infection-Related Epilepsy Syndrome (FIRES) Super Refractory Status Epilepticus (SRSE) Definitions Complication Non-drug Treatment Options Drug Treatment Options Interventional Treatment Options General Care and Monitoring Sudden Unexpected Death in Epilepsy (SUDEP) Sudden Unexpected Death in Epilepsy (SUDEP): Subject-Related Risk Factors Individual Risk Factors Behavioural Risk Factors Possible Genetic Risk Mutations Sudden Unexpected Death in Epilepsy (SUDEP): Risk Factors Pathological Risk Factors Seizure-related Risk Factors Prolonged Post-Ictal Generalised EEG Suppression (PGES) Antiepileptic Drug (AED) Related Risk Factors Doubtful Risk Factors Sudden Unexpected Death in Epilepsy (SUDEP): Clinical Indicators Pre-ictal Respiratory Features Pre-ictal Cardiac Features Ictal Features Post-ictal (Terminal) Cardiorespiratory Features Differential Diagnosis Sudden Unexpected Death in Epilepsy (SUDEP): Management Improve Night-Time Conditions Optimise Seizure Control Consider Alternative Epilepsy Interventions Preventative Measures Proposed Preventative Measures SUDEP Discussion Points With Patient Chapter 3 Sleep Disorders Narcolepsy Narcolepsy: Clinical Features Demographic Features Excessive Sleepiness: Features Cataplexy Sleep Paralysis Hypnagogic Hallucinations Sleepiness-related Daytime Symptoms Complex Sleep-Onset Movements Associated Sleep Disorders Other Clinical Features Narcolepsy: Differential Diagnosis Causes of Secondary Narcolepsy Differential Diagnosis of Excessive Sleepiness Differential Diagnosis of Cataplexy Narcolepsy: Investigations HLA Associations Cerebrospinal Fluid (CSF) Hypocretin: Values Cerebrospinal Fluid (CSF) Hypocretin: Causes of Intermediate Low Levels Multiple Sleep Latency Test (MSLT) Aquaporin 4 Antibodies Narcolepsy: Treatment of Hypersomnia First Line Treatments Other Treatments Treatment of Disturbed Night Sleep Narcolepsy: Treatment of Cataplexy Sodium Oxybate Tricyclics Selective Serotonin Receptor Inhibitors (SSRIs) Serotonin Norepinephrine Reuptake Inhibitors (SNRIs) Histamine H3 Receptor Inverse Agonist Insomnia Insomnia: Causes Primary Insomnia: Types Medical Causes Drug-induced Insomnia: Clinical Features Diagnostic Criteria Sleep-related Impairments Daytime Impairments Complications Insomnia: Non-Drug Treatments Cognitive Behaviour Therapy (CBT) Sleep Hygiene: Helpful Habits Sleep Hygiene: Factors to Avoid at Bedtime Stimulus Control Therapy Paradoxical Intention Other Psychological Therapies Insomnia: Drug Treatments Indication for Drug Treatments Drug Treatments Indicated for Short-Term Use: =4 Weeks Benzodiazepine Receptor Agonists: Z Drugs Sedating Antidepressants: If There Is Co-Existing Mood Disorder Dual Orexin Receptor Antagonists (DORA) Other Drug Treatments Treatments Not Recommended Hypersomnia Central Hypersomnias: Classification Narcolepsy Recurrent Hypersomnia Medical Conditions Causing Hypersomnia and Narcolepsy Idiopathic Hypersomnia Central Hypersomnia: Drug Treatments Amphetamines and Derivatives Non-amphetamine CNS Stimulants Other Drugs Idiopathic Hypersomnia: Clinical Features Types Background Classical Features Associated Disorders Possible Triggers Possible Aggravating Factors Course Idiopathic Hypersomnia: Management HLA Studies Multiple Sleep Latency Test (MSLT) Polysomnography Cerebrospinal Fluid (CSF) Stimulant Therapy Investigational Treatments REM Sleep Parasomnias REM Sleep Behaviour Disorder (RBD): Risk Factors Synucleinopathies Tauopathies Other Neurodegenerative Diseases Drug-induced Other Causes Potential Risk Factors REM Sleep Behaviour Disorder (RBD): Clinical Features Epidemiology Physical Features Language Features Emotional Features Semi-purposeful Actions Dream-related Symptoms Associated Symptoms Complications REM Sleep Behaviour Disorder (RBD): Management Main Recommended Drug Treatments Drug Treatments Which May Also Worsen RBD Poorly Evidenced Drug Treatments Environmental Treatments Pressurised Bed Alarm Anti-IgLON5 Antibody Syndrome: Clinical Features Phenotypes Sleep Related Features Bulbar Features Respiratory Features Movement Disorders Dysautonomia Hyperexcitability Other Features Anti-IgLON5 Antibody Syndrome: Management Pathogenesis HLA Allele Risk Factors Pathology Investigations Treatment Predictors of Treatment Response Exploding Head Syndrome (EHS) Demographic Features Clinical Features Potential Triggers Differential Diagnosis Treatment Synonym Non-REM Sleep Parasomnias Confusional Arousals Defining Features Types Predisposing Factors Motor Features Associated Psychiatric Disorders Associated Sleep Disorders Treatment Sleep Walking (Somnambulism) Defining Features Clinical Features Associated Pain Disorders Predisposing Genetic Susceptibility Predisposing Medications Other Predisposing Factors Differential Diagnosis: Nocturnal Frontal Lobe Epilepsy Non-drug Treatments Drug Treatments Sleep Talking (Somniloquy) Epidemiology and Pathology Content of Speech Associated Features Associated Disorders Chapter 4 Movement Disorders Parkinsonism Parkinson’s Disease (PD): Neurological Risk Factors Strong Risk Factors Genetic Risk Factors Brain Structural Risk Factors Other Possible PD Risk Factors Parkinson’s Disease (PD): Systemic Risk Factors Strong Risk Factors Risk Occupations: Possibly Infections Dietary and Intestinal Other Reported PD Risk Factors Controversial Risk Factor: Appendectomy Controversial Risk Factor: Statin Use Parkinson’s Disease (PD) Genetics: Classification Autosomal Dominant Autosomal Recessive X-linked Unclassified Lysosomal Storage Disorders Genes Parkinson’s Disease (PD): Bradykinesia Manifestations of Bradykinesia Facial Bradykinesia Parkinson’s Disease (PD): Resting Tremor Clinical Types Clinical Features Functional MRI Features Differential Diagnosis Progression Other PD Tremor Types Parkinson’s Disease (PD): Freezing of Gait Types Clinical Patterns Clinical Features Clinical Assessment Strategies to Treat Freezing Drug-treatments Treatment of Co-Morbidities Other Treatments Parkinson’s Disease (PD): Differential Diagnosis Parkinsonian Differentials Tremor Differentials Structural Differentials Red Flags Against PD Diagnosis Tests to Differentiate PD From MSA: More Impaired in MSA Emerging Differentiating Tests of PD Parkinson’s Disease (PD): Treatment of Motor Features Treatments to Reduce Off-Time Treatment of Tremor Treatment of Postural Deformities Treatment of Freezing of Gait (FOG) Acute Alternatives to Oral Treatments Multiple System Atrophy (MSA): Clinical Features Motor Subtypes Non-motor Variants Premotor Symptoms Motor Features Dystonia Autonomic Features Sleep-related Features Other Features Multiple System Atrophy (MSA): Investigations Magnetic Resonance Imaging (MRI) Transcranial Ultrasound Other Brain Imaging Cardiac MIBG Scintigraphy Scan Sphincter Electromyogram (EMG) Bulbocavernosus Reflex (BCR) Optical Coherence Tomography (OCT): Features Other Investigations Progressive Supranuclear Palsy (PSP): Clinical Features Domains Clinically Predictive of PSP Facial Features Ophthalmic Features Postural and Gait Abnormalities Bulbar Features Applause Sign Other Features Poor Prognostic Markers Progressive Supranuclear Palsy (PSP): Differential Diagnosis Parkinson’s Disease (PD) Corticobasal Degeneration (CBD) Other Parkinsonian Disorders Cognitive Disorders Mokri Syndrome Other Differentials Dementia With Lewy Bodies (DLB): Clinical Features Essential and Core Clinical Features of DLB Autonomic Features Psychiatric Features Corticobasal Syndrome (CBS) Presentation Other Supportive Features Other Features Old Synonyms Dementia With Lewy Bodies (DLB): Investigations Indicative Biomarkers of DLB Magnetic Resonance Imaging (MRI) Brain: Atrophic Areas Magnetic Resonance Imaging (MRI) Brain: Loss of Swallow Tail Sign Diffusion Tensor Imaging (DTI): Increased Diffusion Areas Single Photon Emission and Positron Emission Tomography (FP-CIT SPECT) Electroencephalogram (EEG): Features Other Investigations Emerging Investigations Corticobasal Degeneration (CBD): Clinical Features CBD Phenotypes Parkinsonian Features Other Movement Disorders Cortical Features Behavioural Features Speech and Language Features Fulminant Or Rapidly Progressive CBD (RP-CBD) Other Features Corticobasal Degeneration (CBD): Diagnosis Atypical Presentations of CBD Differential Diagnoses Diagnostic Criteria for Probable CBD Diagnostic Criteria for Possible CBD Pathology Levodopa Responsiveness Dystonia DYT1: Early Onset Primary Dystonia Genetics Demographic Features Dystonia Phenotypes Dystonia Features Differential Diagnosis Treatment Synonyms DYT5: Dopa-Responsive Dystonia (DRD): Clinical Features Genetics Pathology Epidemiology Features of Limb Dystonia Features of Postural Tremor Other Dystonic Features Associated Clinical Features Synonym DYT8: Paroxysmal Non-Kinesigenic Dyskinesia 1 (PNKD1) Genetics and Epidemiology Major Features Characteristics of Dystonic Episodes Triggers for Episodes Associated Features Treatment Synonym DYT11: Myoclonus Dystonia: Clinical Features Clinical Patterns Demographic Features Features of Myoclonus Features of Dystonia Psychiatric Features Other Features Differential Diagnosis Synonym DYT12: Rapid Onset Dystonia-Parkinsonism (RDP) Genetics Other ATP1A3 Spectrum Disorders RPD Overlap Syndromes Onset Age Features Clinical Features Triggers Other Features Acronym Cervical Dystonia: Clinical Features General Features Postural Cervical Deformities Associated Dystonias Associated Features Geste Antagoniste (Alleviating Manouevres) Complications Differential Diagnosis Outcome Cervical Dystonia: Management Palliative Manoeuvres Oral Treatment Chemo-denervation Deep Brain Stimulation (DBS) Surgical Treatment Other Treatments Wilson’s Disease: Neurological Features Dystonic Features Associated Movement Disorders Associated Neurological Features Ophthalmic Features Psychiatric Features Clinical Features Related to Zinc Therapy Wilson’s Disease: Management Treatment Phases Penicillamine: Dosing Penicillamine: Complications Trientine Zinc Acetate Liver Transplantation: Indications Other Treatments Precautions in Pregnancy and Surgery Tremor Tremors: Medical Causes Dystonic Tremor: Causes Other Primary Tremor Disorders Tremors With Neurological Disorders Metabolic Tremors Toxin-induced Tremors Other Tremors Essential Tremor (ET): Tremor Features Demographic Features Postural Limb Tremor Features Head Tremor Features Alcohol Sensitivity Other Tremor Features Red Flags Against Essential Tremor Diagnosis Predictors of Fast Progression Essential Tremor (ET): Non-Tremor Features Non-motor Features Parkinsonian Features Magnetic Resonance Imaging (MRI) Brain: Features Essential Tremor (ET): Drug Treatment Level A: Established Effective Level B: Probably Effective Level C: Possibly Effective Level U: Insufficient Evidence Ineffective Absolute Contraindications to Beta Blockers Relative Contraindications to Beta Blockers Ataxia Friedreich’s Ataxia (FA): Clinical Features Genetics Sites of Pathology Onset Age and Types Neurological Features Ophthalmic Features Systemic Features Magnetic Resonance Imaging (MRI) Friedreich’s Ataxia (FA): Monitoring Annual Surveillance Scoliosis Screening Friedreich’s Ataxia Monitoring Scales Indications for Cardiology Referral Friedreich’s Ataxia (FA): Treatment Treatment of Spasticity Treatment of Neuropathic Pain Treatment of Square Wave Jerks and Ocular Flutter Treatment of Cardiomyopathy Management in Pregnancy Multidisciplinary Care Potential and Investigational Treatments Spinocerebellar Ataxia Type 1 (SCA 1) Genetics Central Features Dystonic Features Peripheral Features Pathology Magnetic Resonance Imaging (MRI) Brain Spinocerebellar Ataxia Type 2 (SCA2) Genetics Ataxic Features Other Movement Disorders Peripheral Nerve Features Magnetic Resonance Imaging (MRI) Brain Spinocerebellar Ataxia Type 3 (SCA3): Clinical Features Genetics Types Ophthalmic Features Central Neurological Features Peripheral Neurological Features Differential Diagnosis Synonym Spinocerebellar Ataxia Type 6 (SCA6) Genetics CACNA1A Mutation Associated Disorders Cerebellar Features Other Features Investigations Spinocerebellar Ataxia Type 7 (SCA7) Genetics Clinical Features Magnetic Resonance Imaging (MRI) Brain Episodic Ataxia Type 1 (EA1) Genetic Mutations Features of Ataxia Associated Features Triggers for Attacks Associated Disorders Treatment Episodic Ataxia Type 2 (EA2) Genetics and Clinical Features Triggers Associated Features in Attacks Associated Conditions Differential Diagnosis Investigations Treatment Acronyms Episodic Ataxia (EA): Differential Diagnosis Neurological Differentials Metabolic Differentials Sporadic Adult Onset Ataxia: Neurological Causes Chronic Infections Sporadic Degenerative Hereditary Synonyms for Idiopathic Types Sporadic Adult Onset Ataxia: Systemic Causes Heavy Metals Autoimmune Paraneoplastic Drug-induced Other Causes Synonyms for Idiopathic Types Acronym Chorea Chorea: Neurological Causes Genetic Infective Focal Brain Lesions Other Causes Chorea: Systemic Causes Genetic Autoimmune Diabetic Metabolic Drug-induced Other Causes Chorea: Management Investigations Good-evidenced Drug Treatments Neuroleptics Vesicular Monoamine Transporter 2 (VMAT2) Blockers Other Drug Treatments Huntington’s Disease (HD): Clinical Features Psychiatric Features Cognitive Features Movement Disorders Oculomotor Disorders Other Neurological Features Systemic Features Huntington’s Disease (HD): Differential Diagnosis Huntington’s Disease-Like 1 (HDL1) Huntington’s Disease-Like 2 (HDL2) Huntington’s Disease-Like 3 (HDL3) Huntington’s Disease-Like 4 (HDL4, SCA17) Spinocerebellar Ataxia (SCA) Other Neurodegenerative Causes of Chorea Other Causes of (HD) Phenotype Huntington’s Disease (HD): Treatment Treatments of Chorea: VMAT Blockers Treatment of Chorea: Neuroleptics Treatment of Chorea: Other Agents Treatments of Depression Treatments of Altered Sleep-Wake Cycle Mood Stabilisers Physical Exercise: Benefits Investigational Treatments Acronym Paroxysmal Kinesigenic Dyskinesia (PKD): Clinical Features Triggers for Attacks Aura Symptoms Dyskinesia Features Associated Movement Disorders Associated Neurological Disorders Distinctive Features of PRRT2 PKD Synonyms Dentatorubral Pallidolyusian Atrophy (DRPLA) Genetics Clinical Features Magnetic Resonance Imaging (MRI) Miscellaneous Movement Disorders Tic Disorders: Causes Neurodegenerative Diseases Other Neurological Diseases Drug-induced Infections Developmental Syndromes Other Causes Tourette Syndrome: Clinical Features Genetics Types Onset Age Clinical Features Diagnostic Criteria Co-morbidities Features of Adult Tourette Syndrome Pathological Features Tardive Dyskinesia: Clinical Features Defining Features Stereotypic Movements Akathisia (Inner Restlessness): Manifestations Other Movement Disorders Respiratory Dyskinesia Other Movements Serotonin Syndrome: Causes Antidepressants Monoamine Oxidase Inhibitors (MAOI) Anti-emetics and Antihistamines Abuse Drugs Opiates Other Drugs Serotonin Syndrome: Clinical Features Onset Features Features of Autonomic Hyperactivity Neuromuscular Features Hunter Diagnostic Criteria: Core Features Alternative Diagnostic Criteria Severity Restless Legs Syndrome (RLS): Risk Factors and Causes Demographic Risk Factors Peripheral Neurological Causes Central Neurological Causes Systemic Causes Exacerbating Drugs Restless Legs Syndrome (RLS): Drug Treatments Level A Evidenced Drug Treatment Level B Evidenced Drug Treatment Level C Evidenced Drug Treatment Optional Drug Treatments: Gabapentin Optional Drug Treatments: Others Guidelines for Iron Therapy Insufficient Evidenced Treatment Acronyms Neuroleptic Malignant Syndrome (NMS): Causes and Risk Factors Causes Neuroleptic-related Risk Factors Other Risk Factors Acronym Neuroleptic Malignant Syndrome (NMS): Clinical Features Onset Features Main Neurological Features Autonomic Features Other Neurological Features Differential Diagnosis Neuroleptic Malignant Syndrome (NMS): Management Investigations Treatment of Rigidity Treatment of Excessive Dopaminergic Block Non-drug Treatments Painful Legs Moving Toes (PLMT) Causes Types Clinical Features Variants Drug Treatments Non-drug Treatments Chapter 5 Neuroinflammatory and Autoimmune Disorders Multiple Sclerosis Multiple Sclerosis (MS): Non-Modifiable Risk Factors Genetic Mutations Ethnicity Age-related Risk Factors Month of Birth Chronic Cerebrospinal Venous Insufficiency (CCVI) Multiple Sclerosis (MS): Modifiable Risk Factors Dietary Cigarette Smoking Lifestyle Infections Environmental Other Risk Factors Non-risk Factors Multiple Sclerosis (MS): Classification Relapsing Remitting MS (RRMS) Primary Progressive MS (PPMS) Secondary Progressive MS (SPMS) Benign (non-Progressive) MS Spinal Onset MS Pure Spinal MS Myelocortical MS Cortically Dominant MS Oligoclonal Band (OCB) Negative MS Marburg Variant Other Forms and Variants of MS Multiple Sclerosis (MS): Typical Neurological Features Cerebellar and Brainstem Features Trigeminal Neuralgia Autonomic Features Cognitive Features Psychiatric Features Pyramidal Features Ophthalmic Features Fatigue: Types Multiple Sclerosis (MS): Other Neurological Features Cranial Nerve Dysfunction Movement Disorders Sleep-related Disorders Headache Disorders Neuromuscular Features Other MS Neurological Presentations Prodromal MS Symptoms Disorders Reported to Be Associated With MS Multiple Sclerosis (MS): Systemic Features Respiratory Dysfunction Cardiac Dysfunction Episodic Hypothermia Systemic Lupus Erythematosus (SLE) Hormonal Disorders Gastrointestinal Disorders Possible Cancer Risk Systemic MS Associations Multiple Sclerosis (MS): Differential Diagnosis Inflammatory Autoimmune Infective Myelopathic Ischaemic and Vascular Neoplastic Miscellaneous Differentials Acronym Clinically Isolated Syndromes (CIS): Predictors of Conversion to MS Clinical Predictors Radiological Predictors Cerebrospinal (CSF) Potential Predictors Serum Predictors Predictors of Conversion of Spinal Cord CIS to MS Predictors of Conversion of Optic Neuritis (ON) to MS CIS Conversion Rate to MS Radiologically Isolated Syndrome (RIS): Predictors of Conversion to MS Clinical Radiological Cerebrospinal Fluid (CSF) Predictors of Progression to Primary Progressive MS (PPMS) RIS Conversion and Progression Rate to MS Acronyms Multiple Sclerosis (MS): Symptomatic Treatments Fatigue: Drug Treatments Fatigue: Non-Drug Treatments Urinary Symptoms Spasticity Tremors Tonic Spasms Emotional Lability Oscillopsia Disease Modifying Treatments (DMTs): Types Interferons Monoclonal Antibodies Oral Agents Other Agents Conventional First Line DMTs for MS First Line DMTs for Aggressive Disease Second Line DMTs for Aggressive Disease Newer DMTs Neuromyelitis Optica (NMO) Neuromyelitis Optica (NMO): Central Neurological Features Demographic Features Cranial Nerve Features Cerebral Features Brainstem Features Spinal Cord Features Spinal Movement Disorders (SMDs) NMO Relapses Late Onset NMO Neuromyelitis Optica (NMO): Systemic Features Endocrine Paraneoplastic NMO and Pregnancy Autoimmune Associations Other Systemic NMO Associations Neuromyelitis Optica (NMO): Clinical Differentials and Prognosis Inflammatory Viral Other Infections Other Clinical Differentials Poor Prognostic Features Neuromyelitis Optica (NMO): Differentials of LETM Muscle Sclerosis (MS) Other Inflammatory Differentials Infections Neoplastic Metabolic Other Radiological Differentials Acronym Neuromyelitis Optica (NMO): Long-Term Immunosuppression Treatment Indications Rituximab: Use Rituximab: Side Effects Azathioprine Alternative Agents Emerging Agents Other Neuroinflammatory Disorders Anti-MOG Antibody Disorders: Phenotypes Acute Disseminated Encephalomyelitis (ADEM) Optic Neuritis (ON) Neuromyelitis Optica Spectrum Disorders (NMOSD) Multiple Sclerosis (MS) Multiphasic Disseminated Encephalomyelitis (MDEM) Leukodystrophy-like Phenotype Other Anti-MOG Antibody Disorder Phenotypes Acronym Neurosarcoidosis: Cranial Features Demographic Features Ophthalmological Features Cranial Nerve Features Cerebral Features Neurosarcoidosis Associated Myelopathy Clinical Features Magnetic Resonance Imaging (MRI): Patterns Magnetic Resonance Imaging (MRI): Other Features Magnetic Resonance Imaging (MRI): Enhancement Patterns Cerebrospinal Fluid (CSF) Features Neurosarcoidosis: MRI Features Typical Brain MRI Features Spinal MRI Features Venous Sinus Features Ischaemic Lesions Haemorrhagic Lesions Other Lesions Neurosarcoidosis: Treatment Steroids Alone Steroids With Other Immunosuppression Immunomodulators in Refractory Cases Other Treatments Progressive Multifocal Leukoencephalopathy (PML): Risk Factors JC Virus Infection Immunosuppressive Disorders Natalizumab: High Risk Features for PML Other Drugs Progressive Multifocal Leukoencephalopathy (PML): Clinical Features Demographic Features Neurological Features Poor Prognostic Features Factors That Do Not Affect Prognosis Causes of Death Progressive Multifocal Leukoencephalopathy (PML): Investigations Magnetic Resonance Imaging (MRI) Cerebrospinal Fluid (CSF) Diagnostic Criteria for PML Differential Diagnosis From PML-IRIS On MRI Acronyms Progressive Multifocal Leukoencephalopathy (PML): Management Pre-Natalizumab Assessments: JC Virus Antibody Pre-Natalizumab Assessments: Others PML Monitoring On Natalizumab Therapy Treatment Guidelines in Suspected PML Treatment of PML Re-starting Treatment in Suspected PML Investigational Treatments: Immune Checkpoint Inhibitors Acronym Autoimmune Encephalitis Anti-LGI1 VGKC Autoimmune Encephalitis: Clinical Features Epidemiology Possible Associated HLA Haplotypes Clinical Triad Psychiatric Features Cognitive Features Other Features Outcome Differential Diagnosis: Anti-CASPR2 Anti-LG1 VGKC Autoimmune Encephalitis: Faciobrachial Dystonic Seizures Presentation Clinical Features Triggers Implications Anti-CASPR2 VGKC Autoimmune Encephalitis: Clinical Features Causes of Anti CASPR2 Positivity Clinical Presentations Central Features Peripheral Features Clinical Outcome Anti-CASPR2 VGKC Autoimmune Encephalitis: Management Anti-VGKC Antibody Testing Initial Treatment Secondary Treatment for Refractory Cases Anti-NMDAR Autoimmune Encephalitis: Clinical Features Epidemiology Triggers Encephalitic Features Movement Disorders Sleep Disorders Psychiatric Features Systemic Features Anti-NMDAR Autoimmune Encephalitis: Investigations HLA Associations Genetics Associated Glial and Neuronal Surface Antibodies Serum Antibody Titers Cerebrospinal Fluid (CSF): Antibody Titers Cerebrospinal Fluid (CSF): Other Features Magnetic Resonance Imaging (MRI) Brain Positron Emission Tomography (PET) Electroencephalography (EEG) Features Anti-NMDAR Autoimmune Encephalitis: Treatment First Line Immunotherapy Second Line Immunotherapy Emerging Treatments Surgery Possible Markers of Treatment Response Anti-AMPAR Autoimmune Encephalitis Pathology Epidemiology Clinical Features Associated Tumours Investigations Treatment Acronym Peripheral Autoimmune Disorders Neuromyotonia: Clinical Features Peripheral Features Central Features Synonym Morvan’s Syndrome Aetiology Neurological Features Systemic Features Anti-GAD Syndromes: Phenotypes Classic Anti-GAD Syndromes Temporal Lobe Epilepsy Cerebellar Ataxia Eye Movement Disorders Other Anti-GAD Associated Neurological Disorders Other Anti-GAD Associated Medical Disorders Stiff Person Syndrome (SPS): Clinical Features Epidemiology Features of Rigidity Features of Spasms Other Features Associated Autoimmune Disorders Associated Cancers Differential Diagnoses Stiff Person Syndrome (SPS): Variants Stiff Limb Syndrome Stiff Person Syndrome (SPS) Plus: Types Stiff Person Syndrome (SPS) With Encephalomyelitis Other SPS Variants Stiff Person Syndrome (SPS): Treatment Spasticity Treatment: Benzodiazepines Spasticity Treatment: Others Treatment of Respiratory Crisis Immunosuppression Chapter 6 Infections Viral Infections Viral Encephalitis: Aetiological Indicators Recent Travel Skin Rash Contact With Animals Tremors (Basal Ganglia Involvement) Acute Flaccid Paralysis Immunocompromised State Other Indicative Features of Aetiology Viral Encephalitis: Management Investigations Treatment of Infection Treatment of Raised Intracranial Pressure Indications for Steroids in Viral Encephalitis HIV Associated Neurological Syndromes: Classification HIV Associated Central Neurological Syndromes HIV Associated Myopathies HIV Associated Neuropathy (HAN) Other HIV Associated Neurological Disorders HIV Associated Neurocognitive Disorders (HAND): Clinical Features Classification HIV-associated Dementia (HAD) Infective Differential Diagnoses Other Differential Diagnoses Neurocognitive Screening Tools Neurocognitive Testing HIV Associated Neuropathy (HAN) Sensory Neuropathy Acute and Chronic Inflammatory Demyelinating Polyradiculoneuropathy Opportunistic Vasculitic Neuropathy: Causes Herpes Zoster Associated Neuropathy: Types Diffuse Infiltrative Lymphocytosis Syndrome (DILS) Other HIV Associated Neuropathies Treatment Rabies Encephalitis: Clinical Features Types of Rabies Infection General Features Hydrophobia Peripheral Neurological Features Autonomic Features Sexual Features Differential Diagnosis Course and Outcome Rabies Encephalitis: Virology and Management Virology Transmitting Animals Investigations Post-exposure Management Pre-exposure Management Varicella Zoster Virus (VZV) Infection: Central Features Virology Risk Factors for Reactivation Cerebral Presentations Spinal Cord Presentations Herpes Zoster Ophthalmicus Cranial Nerve Palsies Ocular Features Acronym Varicella Zoster Virus (VZV) Infection: Peripheral Features Dermatological Features Herpes Zoster Plexopathy Zoster Mononeuropathies Zoster Paresis Other Peripheral Neurological Features Varicella Zoster (VZV) Vasculopathy Risk Factors Possible Pathogenetic Mechanisms Onset Features Vascular Features Non-vascular Features Varicella Zoster (VZV) Vasculopathy: Management Cerebrospinal Fluid (CSF) Analysis: Features Magnetic Resonance Imaging (MRI): Infarcts Magnetic Resonance Imaging (MRI): Vasculitis Treatment Dengue Virus Infection (DENV): Neurological Features Encephalopathy Meningoencephalitis Neuroinflammatory Syndromes Cerebellitis Vascular Features Cranial Nerve Palsies Spinal Cord Features Other Central Features Dengue Virus Infection (DENV): Ophthalmological Features Optic Nerve Features Retinal Features Vitreal Features Dengue Virus Infection (DENV): Systemic Features Dengue Haemorrhagic Fever Dengue Shock Syndrome: Features Hepatic Features Gastrointestinal Features Cardiac Features Haematological Features Metabolic Features Other Systemic Features West Nile Virus (WNV) Infection: Central Features Meningitis Movement Disorders Ophthalmic Features Psychiatric Features Other Central Features West Nile Virus (WNV) Infection: Peripheral and Systemic Features Muscle Weakness: Types Peripheral Neuropathy (PN): Types Neuromuscular Disorders Dermatological Features Systemic Features Risk Factors for Mortality Coronavirus (SARS-CoV-2) Infection: Systemic Features Cardiorespiratory Features Ophthalmic Features Dermatological Features Multisystem Inflammatory Syndrome in Children (MIS-C) Other Systemic Features Synonym Coronavirus (SARS-CoV-2) Infection: Central Vascular Features Ischaemic Stroke: Pathology Ischaemic Stroke: Presentation Ischaemic Stroke: Management Haemorrhagic Features Other Vascular Features Synonym Coronavirus (SARS-CoV-2) Infection: Central Non-Vascular Features COVID Encephalopathy Other Forms of Encephalopathy SARS-Cov-2 Related Encephalitis Other Forms of Encephalitis Inflammatory and Demyelinating Features Headache Other Central Presentations Unconfirmed Associations Synonym Coronavirus (SARS-CoV-2) Infection: Cranial Nerve Disorders Olfactory and Gustatory Features: Epidemiology Olfactory and Gustatory Features: Presentation Olfactory and Gustatory Features: Prognosis Other Cranial Neuropathies Synonym Coronavirus (SARS-CoV-2) Infection: Peripheral Features Guillain–Barre Syndrome (GBS) Neuromuscular Junction (NMJ) Features Muscle Features Peripheral Nerve Features Synonym Bacterial Infections Bacterial Meningitis: Clinical Features Commonest Causative Organisms Risk Factors Clinical Features Complications Predictor of Poor Outcome: the FOUR Score Differential Diagnosis: Crowned Dens Syndrome Bacterial Meningitis: Management Management Guidelines Indications for Head CT Before Lumbar Puncture (LP) Antibiotic Choice Duration of Antibiotic Treatment Contact Prophylaxis: for Meningococcal Meningitis Vaccination Tuberculous Meningitis (TBM): Clinical Features Risk Factors Prodrome Meningeal Features Cranial Nerve Palsies Neuroimaging Features Indicators of TBM V Cryptococcal Meningitis Tuberculous Meningitis (TBM): CSF Analysis Cell Count Protein Glucose Microscopy Culture Other CSF Tests Tuberculous Meningitis (TBM): Treatment Anti TB Drug Treatment Adjunctive Treatments Treatment of Hydrocephalus Lyme Neuroborreliosis: Clinical Features Onset Features Lymphocytic Meningitis Cranial Neuropathies Peripheral Nerve Features Other Features Late Lyme Neuroborreliosis (post-Lyme Syndrome): Clinical Features Late Lyme Neuroborreliosis (post-Lyme Syndrome): Causes of Persistent Symptoms Neurosyphilis: Clinical Features Clinical Phenotypes Tabes Dorsalis: Features General Paresis of the Insane (GPI): Features Meningovascular Syphilis: Types Syphilis in Association With HIV Other Presentations of Neurosyphilis Differential Diagnosis Neurosyphilis: Management Indications for Syphilis Screening Cerebrospinal Fluid (CSF) Analysis: Indications Cerebrospinal Fluid (CSF) Analysis: Features Brain Magnetic Resonance Imaging (MRI): Features Treatment Cerebrospinal Fluid (CSF) Monitoring Tetanus: Clinical Features Tetanus Syndromes Painful Spasms Rigidity: Manifestations Autonomic Dysfunction Other Features The Spatula Test Differential Diagnosis Magnetic Resonance Imaging (MRI) Brain: Features Poor Prognostic Features Tetanus: Treatment Main Antibiotics Alternative Antibiotics Treatment of Spasms Other Treatments Treatment of Complications Parasitic Infections Parasitic Infections of the Nervous System: Classification Cestode Infections Trematode Infections Nematode Infections Protozoan Infections Cerebral Malaria: Pathology and Clinical Features Pathology of Malaria Pathogenesis of Cerebral Malaria Brainstem Features Other Neurological Features Malarial Retinopathy Systemic Features Long-term Neurocognitive Features Cerebral Malaria: Investigations and Treatment Blood Investigations Emerging Biomarkers Computed Tomography (CT) Head: Features Magnetic Resonance Imaging (MRI) Brain: Features Other Investigations Emerging Ophthalmologic Investigations Main Treatments Follow Up Treatment: for Seven Days Ancillary Treatments Post Malaria Neurological Syndrome (PMNS) Pathology Systemic Features Cognitive Features Psychiatric Features Other Features Other Post Malaria Neurological Syndromes Course Magnetic Resonance Imaging (MRI) Brain: Features Other Investigations Treatment Neurocysticercosis: Parenchymal Type Biology Pathology Diagnostic Criteria Clinical Features Neurocysticercosis: Extraparenchymal (Racemose) Type Diagnostic Criteria Intracranial Features Fourth Ventricle Features (Brun’s Syndrome) Isolated Brainstem Features Orbital Features Spinal Cord Involvement Other Extraparenchymal Sites Neurocyticercosis: Management Serology Magnetic Resonance Imaging (MRI): Cyst Types Magnetic Resonance Imaging (MRI): Other Features Magnetic Resonance Imaging (MRI): Differentials Computed Tomography (CT) Cerebrospinal Fluid (CSF) Other Tests Treatment Toxoplasmosis: Clinical Features Biology Maturation Forms Risk Factors for Infection and Reactivation Encephalitic Features Other Neurological Features Ocular Toxoplasmosis Congenital Toxoplasmosis: Features Congenital Toxoplasmosis: Differentials (TORCH Complex) Other Features Toxoplasmosis: Management Parasite Identification Computed Tomography (CT) Scan Magnetic Resonance Imaging (MRI): Features Radiological Differentials: Cerebral Lymphoma Radiological Differentials: Others Prevention Drug Treatments Drug Prophylaxis in Immunosuppressed People Fungal Infections Fungal Infections of the Nervous System: Classification Major Nervous System Fungal Infections Dimorphic Fungi Non-aspergillus Moulds Other Nervous System Fungal Infections Cryptococcal Meningitis: Clinical Features Pathology Risk Factors: Cell-Mediated Immunodeficiency Syndromes Risk Factors: Others Neurological Features Ophthalmic Features Systemic Features Cryptococcal Immune Reconstitution Inflammatory Syndrome (IRIS) Cryptococcal Meningitis Associated Disorders Outcome Cryptococcal Meningitis: Management Magnetic Resonance Imaging (MRI) Brain: Features Cerebrospinal Fluid (CSF) Analysis: Routine Tests Cerebrospinal Fluid (CSF) Analysis: Fungal Tests Antifungal Treatment Management of Raised Intracranial Pressure Preventative Treatment Aseptic, Recurrent, and Chronic Meningitis Aseptic Meningitis: Causes Infectious Drug-induced Autoimmune Other Causes Recurrent Meningitis: Causes Tumours Chemical Other Causes Chronic Meningitis: Causes Infective Drug-induced Chemical Neoplastic Neuroinflammatory Uveomeningitis Other Causes Chapter 7 Headache Migraine Migraine: Non-Modifiable Risk Factors Individual Risk Factors Familial Hemiplegic Migraine (FMH) Gene Mutations High Homocysteine Gene Mutations Other Genetic Risk Factors Hypercoagulable Risk Factors Protective Factors Acronyms Migraine: Modifiable Risk Factors Individual Risk Factors Patent Foramen Ovale (PFO) Environmental Risk Factors Dietary and Endocrine Risk Factors Migraine: Triggers Physiological Stress Dietary Environmental Physical Exertion Radiotherapy Migraine Auras General Aura Features Classification of Auras By Duration Scintillating Scotomas Other Visual Aura Types Associated Visual Phenomena Differential Diagnoses Prophylactic Treatment Treatment of Migraine Aura Status Migraine: Headache Features Typical Headache Features Defining Accompanying Features Cranial Autonomic Features Unusual Migraine Features Migraine Postdrome Significant Differential Diagnoses Ophthalmoplegic Migraine Clinical Features Pathogenesis Differential Diagnosis Treatment Synonym Retinal Migraine Pathogenesis and Epidemiology Clinical Features Associated Visual Phenomena Differential Diagnosis Red Flags Against Migraine Acute Treatment Prophylaxis Contraindicated Drugs Synonyms Vestibular Migraine Diagnostic Criteria Genetic Risk Factor Features of Vertigo Features of Headache Associated Features Acute Treatment Prophylaxis Familial Hemiplegic Migraine (FHM) Genetic Types and Mutations Triggers Clinical Features Complications Differential Diagnosis: Sporadic Hemiplegic Migraine (SHM) Treatment Status Migrainosus Clinical Features Pathology Secondary Causes Drug Treatments Non-drug Treatments Migraine Acute Drugs: Analgesics and Anti-Emetics Non-steroidal Anti-Inflammatory Drugs (NSAIDs) Analgesics Anti-emetics: Prochlorperazine Anti-emetics: Others Acronyms Triptans: Types and Clinical Use Mode of Action and Indications Triptan Types and Doses Specific Indications Drug Interactions: Anti-Depressants Drug Interactions: Others Acronyms CGRP Receptor Antagonists (CGRP-RAs): General Aspects CGRP Gene Family Members CGRP Receptor Subunits CGRP Role in Migraine Pathogenesis Indications for CGRP-RAs Types Emerging CGRP-RAs Discontinued CGRP-RAs Synonym Lasmiditan Pharmacology Dosing Contraindications and Precautions Side Effects Migraine Non-Drug Treatments Lifestyle Modification Psychological Treatments Complementary Treatments Other Interventional Treatments Emerging Treatments Migraine Prophylactic Drugs: Classification Level A Evidenced Drugs Level B Evidenced Drugs Level C Evidenced Drugs Insufficient Evidenced Drugs Other Drugs Investigational Prophylactic Drugs Acronym Migraine Prophylaxis: CGRP Monoclonal Antibodies (CGRP MAbs) General Aspects Erenumab Fremanezumab Galcanezumab Eptinezumab Contraindications Acronym Trigeminal Autonomic Cephalalgias Cluster Headache (CH): Causes Familial Vascular Neoplastic Infectious Drug-induced Dental Developmental Other Causes Lifestyle Risk Factors Cluster Headache (CH): Clinical Features Episodic CH Chronic CH Premonitory Symptoms Headache Features Autonomic Features: Conjunctival Injection Autonomic Features: Others Associated Attack Features Associated Migrainous Symptoms Cluster Headache (CH): Acute Treatment Level A Evidence Level B Evidence Level C Evidence Galcanezumab Insufficient-evidence Vagus Nerve Stimulation (VNS) Investigational Acute Treatments Acronyms Cluster Headache (CH): Transitional Prophylaxis Prednisolone Suboccipital Steroid Injection Methysergide Ergotamine Tartrate Dihydroergotamine Cluster Headache (CH): Chronic Drug Prophylaxis Verapamil Civamide Nasal Spray Lithium Warfarin Melatonin Insufficient Evidenced Treatments (Level U) Probably Ineffective Treatments Other Reported Treatments Acronym Paroxysmal Hemicrania (PH) Pathology Clinical Features Associated Features Main Treatment Other Treatments Hemicrania Continua (HC) Clinical Features Associated Features Treatment: Indomethacin Other Treatments Long Lasting Autonomic Symptoms With Associated Hemicrania (LASH) Definition Triggers Clinical Features Autonomic Features Treatment SUNCT: Causes and Triggers Vascular Causes Neoplastic Causes Other Causes Triggers Acronym SUNCT: Clinical Features Demographic Features Typical Features Location of Headaches Associated Features Atypical Features Magnetic Resonance Imaging (MRI): Features Acronym SUNCT: Treatment Drug Treatments Interventional Treatments for Refractory Cases Acronym Intracranial Pressure Headaches Idiopathic Intracranial Hypertension (IIH): Typical Clinical Features Risk Factors Headache Features Neurological Features Ophthalmic Features Differential Diagnoses of Papilloedema Red Flags Against IIH Synonym Idiopathic Intracranial Hypertension (IIH): Variant Types IIH Without Papilloedema (IIHWOP) Fulminant IIH Late Onset IIH IIH With Normal CSF Opening Pressure IIH in Men Synonym Idiopathic Intracranial Hypertension (IIH): Medical Differentials Neurological Endocrine Respiratory Chromosomal Rheumatological Nutritional Other Medical Differentials Synonym Idiopathic Intracranial Hypertension (IIH): Drug Differentials Antibiotics Hormones Drug Withdrawal Other Drugs Synonym Idiopathic Intracranial Hypertension (IIH): MRI Features Sella Features Optic Features Posterior Fossa Features Venous Sinus Features Other Features Idiopathic Intracranial Hypertension (IIH): Medical Treatment Indications for Treatment Non-drug Treatments Acetazolamide: Benefits and Dosing Acetazolamide: Side Effects Non-evidenced Drug Treatments Lumbar Puncture (LP) Urgent Treatments Emerging Drug Treatments Idiopathic Intracranial Hypertension (IIH): Shunting Indications Benefits Protocol Complications Idiopathic Intracranial Hypertension (IIH): Other Surgical Treatments Dural Venous Sinus Stenting (DVSS) Optic Nerve Sheath Fenestration (ONSF) Bariatric Surgery Spontaneous Intracranial Hypotension (SIH): Clinical Features Types of Spinal CSF Leaks Epidemiology Typical Headache Features Atypical Headache Features Auditory Features Other Associated Features Unusual Presentations Spontaneous Intracranial Hypotension (SIH): MRI Features Fluid Collections: Locations Meningeal Features Engorgements Spinal Features Other Features MRI Differentials Post Dural Puncture Headache (PDPH): Clinical Features Risk Factors Factors Unrelated to Risk of PDPH Clinical Features Exacerbating Features Associated Features Synonym Post Dural Puncture Headache (PDPH): Management Prevention Ineffective Preventions Cerebrospinal Fluid (CSF): Features Magnetic Resonance Imaging (MRI) Features Established Treatments Emerging Treatment Treatments Based On Small Trials and Case Reports Synonym Other Headache Types Tension Type Headache (TTH): Clinical Features Triggers Clinical Features Differential Diagnosis Tension Type Headache (TTH): Treatment Analgesics Non-steroidal Anti-Inflammatory Drugs (NSAIDs) Prophylactic Treatments Non-drug Treatments Acronym Medication Overuse Headache (MOH): Clinical Features Diagnostic Criteria Individual Risk Factors Medical Risk Factors Clinical Features Differential Diagnosis Acronym Medication Overuse Headache (MOH): Treatment Non-drug Treatments Drug Treatments Treatment of Co-Morbidities Detoxification: Benefits Detoxification: Indications for In-Patient Care Relapse: Risk Factors Relapse: Prevention Patient Education Thunderclap Headache (TCH) Vascular Causes Other Neurological Causes Medical Causes Drug-induced Triggers Clinical Features Assessment Exertional Headache Benign Exertional Headache Symptomatic Exertional Headache: Causes Symptomatic Exertional Headache: Clinical Features Sexual Headache Causes Clinical Features Treatment New Persistent Daily Headache (NPDH) Clinical Features Differential Diagnosis Other Causes of Daily and Near Daily Headaches Acute Treatment Prophylaxis: Evidence From Small Case Series Chapter 8 Vascular Disorders Ischaemic Stroke Features Transient Ischaemic Attacks (TIA): Clinical Features Ophthalmic Features Focal Limb Deficits Focal Bulbar Deficits Cerebral Features Brainstem Features Vegetative Features Up-going Thumb Sign Transient Ischaemic Attacks (TIA): Investigations Magnetic Resonance Imaging (MRI) Brain Carotid Doppler Ultrasound Intracranial Vascular Investigations Cardiac Investigations Indications for Transoesophageal ECHO (TOE) Transient Ischaemic Attacks (TIA): Treatment Antiplatelets Other Treatments Ischaemic Stroke: Genetic Risk Factors Connective Tissue Diseases Prothrombotic Disorders Miscellaneous Genetic Disorders Genetics of Sporadic Stroke Acronyms Ischaemic Stroke: Medical Risk Factors Cardiovascular Risk Factors Contraceptives Migraine With Aura Medical Co-Morbidities Metabolic Risk Factors Infections Other Risk Factors Ischaemic Stroke: Social and Environmental Risk Factors Individual Risk Factors Smoking Dietary Stress Cocaine: Mechanisms Trauma Environmental Pollution Heavy Metal Exposure Possible Protective Environmental Factors Ischaemic Stroke: Differential Diagnosis Central Neurological Differentials Peripheral Neurological Differentials Systemic Differentials Radiological Differentials Ischaemic Stroke Complications: Classification Major Stroke Complications Neurological Complications Post-stroke Psychosis: Types Neuropsychiatric Complications: Others Cardiorespiratory Complications Systemic Complications Cryptogenic Stroke: Potential Causes Potential Cardiac Causes Potential Systemic Causes Predictors of Atrial Fibrillation HAVOC AF Prediction System HAVOC AF Risk Scoring Embolic Stroke: Risk Factors Major Risk Factors Minor and Uncertain Risk Factors Embolic Stroke of Undetermined Source (ESUS): Potential Causes Conventional Cardiac Causes Left Atrial Dysfunction Atrial Cardiopathy Carotid Artery Web Potential Systemic Causes Spinal Cord Infarction (SCI): Risk Factors and Causes Risk Factors Procedural Causes Aortic Causes Embolic Causes Vascular Causes Other Causes Synonym Posterior Circulation Stroke: Causes Basilar Artery Stenosis Intracranial Arterial Dolichoectasia (IADE) Other Vascular Causes Non-vascular Causes Posterior Circulation Stroke: Clinical Features Epidemiology Anatomical Supply of the Posterior Circulation Features of Impaired Consciousness Features of Impaired Vegetative Functions Features of Weakness Unusual Features Malignant Cerebellar Infarction Locked-in Syndrome Red Flag Presentations Difficulties With Clinical Diagnosis Stroke in the Young: Vascular Causes Vasculopathies Prothrombotic Conditions Acronyms Stroke in the Young: Systemic Causes Commonest Cardioembolic Causes Low Or Uncertain Cardioembolic Risk Infective Causes Autoimmune Causes Miscellaneous Causes Stroke Treatment Ischaemic Stroke: Acute Treatment Outline Antiplatelets Thrombolysis Mechanical Thrombectomy Anticoagulation Carotid Endarterectomy (CEA) Management of Malignant Cerebral Oedema (MBE) Acute Decompressive Hemicraniectomy: Indications Treatment of Silent Brain Infarcts Emerging Treatments for Stroke Thrombolysis: Clinical Use Timing Potentially Beneficial But Unconfirmed Indications Thrombolytic Agents Tenecteplase Versus Alteplase Thrombolysis and Large Strokes Thrombolysis and Seizures Predictors of Good Outcome Prediction Scoring Systems Thrombolysis: Contraindications Bleeding Related Non-bleeding Related Additional European Contraindications Thrombectomy: Clinical Use Key Thrombectomy Trials With 6-Hour Time Window Criteria for Thrombectomy Timing Complications Secondary Stroke Prevention Antiplatelet Therapy Dual Antiplatelet Therapy Blood Pressure Reduction Statin Therapy Smoking Cessation: Mechanisms Alcohol Reduction Exercise Dietary Control Treatment of Atrial Fibrillation Other Preventative Measures Investigational Preventative Measures Stroke Rehabilitation Rehabilitation Setting Goals Assessments Therapies Investigational Treatments Discharge Planning Post-discharge Haemorrhagic Stroke Intracerebral Haemorrhage (ICH): Causes and Risk Factors Vascular Causes Congenital Heart Diseases Oral Anticoagulants: Predictors Genetic Risk Factors Metabolic Risk Factors Other Risk Factors Intracerebral Haemorrhage (ICH): Complications Early Seizures Delayed Seizures: Risk Factors Delayed Seizures: CAVE Predictive Score Intraventricular Extension Hyperacute Injury Marker (HARM) Recurrent Haemorrhage: Risk Factors Other Neurological Complications Systemic Complications Intracerebral Haemorrhage (ICH): Acute Medical Treatment Reverse Anticoagulation Monitor Intracranial Pressure (ICP): Indications Intracranial Pressure (ICP): Management Blood Pressure Management Seizure Management Prevent Deep Vein Thrombosis (DVT) Other Treatments Precautions Investigational Treatments Subarachnoid Haemorrhage (SAH): Causes Vascular Malformations Vasculopathies Vascular Disorders Brain Lesions Systemic Causes Aspirin Subarachnoid Haemorrhage (SAH): Clinical Features Headache Loss of Consciousness Sudden Death: Predictors Terson’s Syndrome Other Clinical Features Subarachnoid Haemorrhage (SAH): Medical Treatment General Medical Treatment Blood Pressure Control Seizure Control Indications for Long-Term Antiepileptic Drug (AED) Treatment Nimodipine Other Treatments Vascular Malformations Cerebral Aneurysms: Risk Factors for Formation Acquired Risk Factors Familial Risk Factors Adult Polycystic Kidney Disease (APCKD) Other Connective Tissue Disorders Candidate Genes Risk Factors for Multiple Aneurysms Cerebral Aneurysms: Clinical Features Features of Familial Aneurysms Complications of Cerebral Aneurysms Cranial Nerve Impairment TIA and Stroke Prognosis of Ruptured Aneurysms Cerebral Aneurysms: Screening Indications for Aneurysm Screening Screening Frequency Counselling Points Screening After Aneurysm Surgery Cerebral Aneurysms: Treatments Coil Embolisation: Risks Coil Embolisation: Predictors for Acute Re-Rupture (Within 3 Days) Surgical Clipping Surgical Clipping Compared to Coiling Woven Endobridge (WEB) Device Preventative Measures Against Rupture Acronyms Arteriovenous Malformations (AVM): Clinical Features Epidemiology Risk Factors for De Novo (Acquired) AVMs Associated Disorders Presentations Risk Factors for Haemorrhage Risk Factors for Seizures AVM Rupture Risk Grading Systems Spinal Dural Arteriovenous Fistula (DAVF): Clinical Features Pathology Risk Factors Clinical Features Triggers of Symptoms Differential Diagnosis: Peripheral Neuropathy (PN) Differential Diagnosis: Other Spinal Vascular Malformations Differential Diagnosis: Others Spinal Dural Arteriovenous Fistula (DAVF): Management Magnetic Resonance Imaging (MRI): Features Spinal Catheter Angiography Endovascular Embolisation Therapy Microsurgical Occlusion Precaution Vasculopathies Cervical Artery Dissection (CAD): Causes and Risk Factors Physiological Risk Factors Metabolic Risk Factors Vascular Risk Factors Environmental Risk Factors Direct Causes Other Causes Familial CAD (FCAD): Genetic Variants Cervical Artery Dissection (CAD): Clinical Features Headache Neck Pain Stroke Global Orbital Infarction Syndrome Other Features Features of Familial CAD (FCAD) Differential Diagnosis of Headaches Preceding Stroke Recurrent Cervical Artery Dissection Prognosis Cerebral Amyloid Angiopathy (CAA): Clinical Features Pathology Hereditary CAA: Types Transient Focal Neurological Episodes (TFNE, Amyloid Spells): Types Transient Focal Neurological Episodes (TFNE, Amyloid Spells): Features Intracerebral Haemorrhage (ICH): Predictive Features Subarachnoid Haemorrhage (SAH) Cognitive Impairment Other Clinical Features Boston Criteria for Probable CAA Acronym Cerebral Amyloid Angiopathy (CAA): Radiological Features Microbleeds Haemorrhage Types Superficial Siderosis (SS) Ischaemic Features Other MRI Features Amyloid PET Scan Features Differential Diagnosis of Microhaemorrhages Reversible Cerebral Vasoconstriction Syndrome (RCVS): Causes Antidepressants Nasal Decongestants Migraine Drugs Cytotoxic Agents Other Drugs Drugs of Abuse Medical Causes Neurosurgical Causes Other Causes Reversible Cerebral Vasoconstriction Syndrome (RCVS): Clinical Features Demographic Features Headache Features Other Features RCVS2 Diagnostic Scoring System (-2 to +10) Complications Course Primary Angiitis of the Central Nervous System (PACNS): Clinical Features Classification Risk Factors Demographic Features Headache Stroke and Transient Ischaemic Attacks (TIAs) Other Clinical Features Primary Angiitis of the Central Nervous System (PACNS): Radiological Differentials Vascular Differentials Haemorrhagic Differentials Infectious Differentials Neoplastic Differentials Inflammatory Differentials Angiographic Differentials Acronym CADASIL: Clinical Features Stroke Migraine Cognitive Features Psychiatric Features Other Features Atypical Manifestations Acronym CADASIL: Management Magnetic Resonance Imaging (MRI): Features Cerebrospinal Fluid (CSF) Analysis Other Investigations Treatment Acronym Venous Disorders Cerebral Vein Thrombosis (CVT): Haematological Risk Factors Thrombophilia Blood Disorders Other Haematological Disorders Synonym Cerebral Vein Thrombosis (CVT): Non-Haematological Risk Factors Medical Conditions Immunological Conditions Procedures Drugs High Altitude Synonym Cerebral Vein Thrombosis (CVT): Clinical Features Distribution of CVT Usual Presentations Other Presentations Complications Risk Factors for Recurrent CVT Predictors of Good Outcome Synonym Cerebral Vein Thrombosis (CVT): Investigations Thrombophilia Tests: Indications Thrombophilia Tests: Screening D-dimer Magnetic Resonance Imaging (MRV): Features Computed Tomography Venogram (CTV): Benefits Computed Tomography Venogram (CTV): Diagnostic Signs Paramagnetic-sensitive MRI Sequences Other Imaging Techniques Radiological Differential Synonym Cerebral Vein Thrombosis (CVT): Anticoagulant Treatment Acute Anticoagulation Longer Term Anticoagulation Short-term Anticoagulation (3–6 Months): Indications Chronic Anticoagulation (6–12 Months): Indications Indefinite Anticoagulation: Indications Follow-up Imaging Synonym Cavernous Sinus Syndrome (CSS) Contents of the Cavernous Sinus Causes Clinical Features Chapter 9 Cranial Nerves Optic Nerve Optic Neuropathy: Medical Causes Autoimmune Connective Tissue Diseases Inflammatory Compressive Hereditary Ischaemic Miscellaneous Causes Optic Neuropathy: Infectious Causes Viral Causes Bacterial Causes Optic Neuropathy: Toxic and Drug-Induced Toxic Causes Drug-induced Optic Neuropathy: Clinical Features Major Features Uhthoff’s Phenomenon Fundoscopy Features Differential Diagnoses Acronym Optic Neuritis: Clinical Features Onset and Progression Features of Eye Pain Other Symptoms Relative Apparent Pupillary Defect (RAPD) Uhthoff’s Phenomenon Pulfrich Phenomenon Fundoscopy Other Features Optical Coherence Tomography (OCT) Optic Neuritis: Differential Diagnosis Differential Diagnosis of Optic Neuritis Differential Diagnosis of Optic Nerve Head Oedema (ONHE) Optic Atrophy: Genetic Causes Nutritional and Metabolic Mitochondrial Genetic Neuropathy Neurodegenerative Dystonic Miscellaneous Causes Acronyms Optic Atrophy: Non-Genetic Causes Neoplastic Infectious Autoimmune and Inflammatory Vascular Toxic and Drug-Induced Ocular Miscellaneous Causes Trigeminal Nerve Trigeminal Neuropathy: Causes Neoplastic Infective Drug-induced Neurological Medical Causes Trigeminal Neuralgia (TN): Clinical Features Triggers Trigger Zones Pain Features Other Features Trigeminal Neuralgia (TN): Management Head Imaging Trigeminal Nerve Evoked Potentials First Line Drug Treatments Second Line Or Add-On Drug Treatments Insufficient-evidenced Treatment Microvascular Decompression Gasserian Ganglion Neuroablative Treatments Treatments of Refractory Cases Investigational Treatments Facial Nerve Bell’s Palsy: Clinical Features Facial Weakness Associated Features Features of Aberrant Renervation House–Brackmann Grading System Other Grading Systems Poor Prognostic Factors Synonyms Bell’s Palsy: Differential Diagnosis Infectious Differentials Parry–Romberg Syndrome Stroke Foville Syndrome Millard-Gubler Syndrome Cerebellopontine Angle Syndrome Other Neurological Differentials Bell’s Palsy: Management Investigations Tests to Exclude Differentials Antiviral and Steroid Treatment Eye Protection Facial Re-Animation Treatment of Facial and Eyelid Weakness Treatment of Synkinesis Other Treatments Synonyms Ramsay Hunt Syndrome (RHS) Pathology Classic Triad Clinical Features: Others Differential Diagnosis: Bell’s Palsy Investigations Antiviral Treatment Other Treatments Other Ramsay Hunt Syndromes Synonym Post Herpetic Neuralgia (PHN) Definition Risk Factors Treatment Other Cranial Nerves Anosmia: Causes Rhinological Causes Parkinson’s Disease (PD) Other Neurodegenerative Diseases Other Neurological Causes Systemic Causes Drug-induced Nutritional Causes Oculomotor Nerve Palsy: Clinical Features Muscles Innervated By the Oculomotor Nerve Clinical Features Syndromes of Oculomotor Nucleus Palsy Features of Aberrant Regeneration of the Oculomotor Nerve Synonyms for Oculomotor Nerve Synonyms for Aberrant Regeneration Trochlear Nerve Palsy: Causes Neurological Causes Vascular Causes Neoplastic Causes Infective Causes Iatrogenic Causes Synonyms Abducens Nerve Palsy: Neurological Causes Traumatic and Iatrogenic Causes Intracranial Vascular Causes Inflammatory and Immune Causes Neoplastic Causes Other Neurological Causes Synonyms Abducens Nerve Palsy: Systemic Causes Vascular Risk Factors Viral Causes Bacterial Infections Fungal Infections Medical Causes Drug-induced Synonyms Abducens Nerve Palsy: Brainstem Syndromes Raymond’s Syndrome Millard-Gublar Syndrome Foville Syndrome Godtfredsen (Eye Twist and Tongue Twist) Syndrome Synonyms Vagus Nerve Palsy: Causes Intracranial Causes Neoplastic Causes Cardiovascular Causes Thoracic Causes Other Neurological Causes Systemic Causes Synonyms Vagus Nerve Palsy: Clinical Features Clinical Features Sensory Neuropathic Cough Synonyms Dysphonia: Causes Neurological Causes Occupational Causes Laryngeal Causes Head and Neck Causes Medical Causes Drug-induced Deafness: Genetic Causes Genetic Causes of Deafness Deafness: Acquired Causes Intracranial Infective Drug-induced Otologic Autoimmune Hypoglossal Nerve Palsy: Causes Vascular Causes Hypoglossal Nerve Tumours Other Neurological Causes Orthopaedic Causes Iatrogenic Causes Other Causes Synonyms Hypoglossal Nerve Palsy: Clinical Features Clinical Features Collet Sicard Syndrome Jugular Foramen Syndrome Schmidt Syndrome Villaret’s Syndrome Jackson Syndrome Opalski Syndrome Dejerine Syndrome Tapia Syndrome Synonyms Cranial Nerve Associated Disorders Painful Ophthalmoplegia: Causes Aneurysms Tumours Cavernous Sinus Lesions Inflammatory and Infective Causes Orbital Lesions Pituitary Lesions Other Causes Supranuclear Gaze Palsy: Causes Parkinsonian Causes Infective Causes Other Neurological Causes Iatrogenic Causes Chapter 10 Spinal Cord Disorders Myelopathy Acute Transverse Myelitis (ATM): Infectious and Inflammatory Causes Inflammatory Viral Bacterial Parasitic and Fungal Vaccinations Acute Transverse Myelitis (ATM): Other Causes Vascular and Ischaemic Autoimmune and Paraneoplastic Neoplastic Nutritional Drug-induced Other Causes Cervical Compressive Myelopathy: Clinical Features Causes Symptoms Clinical Signs Myelopathy Hand Magnetic Resonance Imaging (MRI) Spine: Features Predictors of Poor Progression Synonym Non-compressive Myelopathy: Neurological Causes Inflammatory and Vasculitic Causes Degenerative Causes Other Causes Myelopathy With Normal MRI Scan Neurodegenerative Causes Metabolic Causes Infective Causes Other Causes Misdiagnosis Spastic Paraparesis Spastic Paraparesis: Causes Neurodegenerative Inflammatory and Infective Nutritional and Metabolic Structural and Vascular Other Causes Acronym Spastic Paraparesis: Investigations Biochemistry Tests Microbiology Genetics Neurophysiology Other Tests Spinal Cord Tumours Spinal Cord Tumours: Classification Intramedullary Intradural Extramedullary Extradural Spinal Cord Tumours: Clinical Features and Management Symptoms Clinical Signs Causes of Papilloedema With Spinal Cord Tumours Differentials of Intramedullary Spinal Tumours Magnetic Resonance Imaging (MRI): Features Treatment Metastatic Cord Compression Commonest Primary Sites Clinical Features Surgical Treatment: Decompression Radiotherapy Bisphosphonates: Indications Other Treatments Poor Prognostic Factors Spinal Canal Stenosis Spinal Canal Stenosis: Clinical Features and Management Pain Characteristics Provoking Factors Relieving Factors Sensory and Autonomic Symptoms Stance and Gait Difficulty Clinical Assessments Magnetic Resonance Imaging (MRI) Treatment Spinal Canal Stenosis: Differential Diagnosis Vascular Claudication Lateral Disc Prolapse Other Differentials Chapter 11 Anterior Horn Cell Disorders Motor Neurone Disease Motor Neurone Disease (MND): Major Genetic Risk Factors SOD-1 Gene Mutations C9orf72 Gene Mutation Multisystem Proteinopathy Gene Mutations ALS Gene Mutations CHCHD10 Gene Mutations: Features TBK1 Gene Mutations Motor Neurone Disease (MND): Non-Genetic Risk Factors Individual Risk Factors Human Endogenous Retrovirus (HERV-K) Heavy Metals Possible Occupational Risk Factors Uncertain Risk Factor: Smoking Motor Neurone Disease (MND): Neuromuscular Features Onset Major MND Subtypes Signs of Muscle Weakness Bulbar and Pseudobulbar Features Split Signs Tongue Features Other Neuromuscular Features Motor Neurone Disease (MND): Other Features Sleep Disorders Cognitive Impairments Neuropsychiatric Features Autonomic Features Pain Movement Disorders Risk of Cancer Other Features Behavioural Assessment Tools Motor Neurone Disease (MND): Diagnostic Criteria Diagnostic Classification Systems Neurophysiological Requirements Clinical Requirements Definite ALS Probable ALS Probable ALS-Laboratory Supported Possible ALS Motor Neurone Disease (MND): Differential Diagnosis Peripheral Differentials: Multifocal Motor Neuropathy (MMN) Peripheral Differentials: Others Central Differentials SOD 1 Deficiency Non-neurological Differentials ALS Differential Diagnostic Index (ALSDI) Split Hand (SI) Differential Diagnostic Index Primary Lateral Sclerosis (PLS): Clinical Features Demographic Features Features of Spasticity Other Features Occasional Features Diagnostic Inclusion Criteria Diagnostic Exclusion Criteria Progression Predictors of Progression to ALS Progressive Muscular Atrophy (PMA) Demographic Features Pathology Clinical Features Differential Diagnosis Flail Arm Syndrome (FAS) Variant Motor Neurone Disease (MND) Demographic Features Genetics Onset Features Clinical Features Differential Diagnosis Investigations Pathology Synonym C9orf72 Variant Motor Neurone Disease (MND): Clinical Features Epidemiology Onset Features Cognitive Features Neurological Features Psychiatric Features Cancer Risk Predictors of Poor Prognosis C9orf72 Variant Motor Neurone Disease (MND): Investigations Genetics Pathology Magnetic Resonance Imaging (MRI) Brain: Features Pre-symptomatic MRI Markers Cerebrospinal Fluid (CSF): Features MicroRNAs Riluzole Pharmacology Benefits Dosing and Administration Monitoring Side Effects Acronyms Edaravone Pharmacology and Benefits Dosing Side Effects Motor Neurone Disease (MND): Neurological Symptomatic Treatments Spasticity Cramps Labile Emotions Pain Anxiety Fatigue Insomnia Motor Neurone Disease (MND): Systemic Symptomatic Treatments Sialorrhoea and Drooling Refractory Sialorrhoea and Drooling Dyspnoea Laryngospasm Constipation Anxiety Other Symptomatic Treatments Motor Neurone Disease (MND): Supportive Care Measures to Improve Swallow Other Measures to Support Eating and Swallowing Communication Aids Dietary Modifications Interventional Care for Secretions Nutritional Management Spinal Muscular Atrophy Spinal Muscular Atrophy (SMA): Classification Classical SMA Types Major SMA Variants Riboflavin Transporter Deficiencies (RTDs) Other SMA Variants Spinal Muscular Atrophy (SMA): Types I-IV SMA Type I (Werdnig-Hoffman Syndrome) SMA Type II SMA Type III (Kugelberg-Welander Syndrome) SMA Type IV Spinal Muscular Atrophy (SMA): General Treatments Respiratory Treatment Nutritional Management Measures to Improve Swallowing Measures to Improve Intestinal Function Orthopaedic Care Prevention of Contractures Other Treatment Considerations Spinal Muscular Atrophy (SMA): Gene Therapy Nusinersen Onasemnogene Abeparvovec Other Anterior Horn Cell Disorders Monomelic Amyotrophy: Pathology and Epidemiology Pathogenesis Pathology Epidemiology Synonyms Monomelic Amyotrophy: Clinical Features Weakness and Wasting Patterns of Weakness and Wasting Other Features Preserved Functions Differential Diagnosis: Major Types Progress and Outcome Synonyms Monomelic Amyotrophy: Management Magnetic Resonance Imaging (MRI) Spine: Features Magnetic Resonance Imaging (MRI) Spine: Dynamic Contrast Magnetic Resonance Imaging (MRI) Muscles Electromyogram (EMG) Blood Tests Treatments Synonyms Kennedy Disease (SBMA): Clinical Features Neuromuscular Features Central Features Endocrine Features Cardiac Features Other Features Differential Diagnosis Synonym Kennedy Disease (SBMA): Genetics and Management Genetics CAG Repeat Expansion Sizes Muscle Enzymes Elevated Magnetic Resonance Imaging (MRI) Positron Emission Tomography (PET) Scan Blood Tests Investigational Treatments Synonym Post-polio Syndrome (PPS): Clinical Features Potential Causes Pathology Risk Factors Clinical Features Complications Variant: Muscular Atrophy (PPMA): Features Variant: Muscular Dysfunction (PPMD): Features Post-polio Syndrome (PPS): Differentials and Management Differential Diagnosis Investigations Fatigue Treatment Other Treatments Chapter 12 Root and Plexus Disorders Radiculopathy Radiculopathy: Causes Degenerative Causes Vascular Causes Extra-spinal Causes Other Causes Cervical Radiculopathy: Clinical Features C2 Radiculopathy C3-C4 Radiculopathy C5 Radiculopathy C6 Radiculopathy C7 Radiculopathy C8-T1 Radiculopathy Provocative Tests Cervical Radiculopathy: Differential Diagnosis Orthopaedic and Rheumatological Differentials Neurological Differentials Lumbosacral Radiculopathy: Clinical Features Anatomy of L4-L5 Disc Prolapse Differentiating Features Red Flags for Sinister Causes Lumbosacral Radiculopathy: Differential Diagnosis Femoral Neuropathy Common Peroneal Neuropathy Hip Abnormalities Other Differential Diagnoses Lumbosacral Polyradiculopathy Compressive Causes Infiltrative Causes Infective Causes Other Causes Clinical Features Nerve Conduction Studies (NCS) Cauda Equina Syndrome (CES) Causes Clinical Features Treatment Elsberg Syndrome Pathology Causes Clinical Features Differential Diagnosis Magnetic Resonance Imaging (MRI) Spine: Features Cerebrospinal Fluid (CSF) Analysis Treatment Thoracic Outlet Syndrome (TOS): Causes and Risk Factors Risk Groups Causes Synonym Thoracic Outlet Syndrome (TOS): Clinical Features Sensory Symptoms Neck Pain Radiation Territories Other Features Clinical Signs Differential Diagnosis Synonym Thoracic Outlet Syndrome (TOS): Provocative Tests Roos Test Elvey’s Test Morley’s Sign Vascular Tests Other Provocative Tests Synonym Plexopathy Brachial Plexopathy: Causes Immune-mediated Surgical Causes Genetic Causes Neonatal Causes Vasculitic Causes Other Causes Brachial Neuralgia: Risk Factors Immune Diseases Other Risk Factors Synonym Brachial Neuralgia: Clinical Features Commonest Affected Nerves Nerve Involvement Outside the Plexus Pain Weakness Arm Positioning Diaphragmatic Paralysis Other Features Variant Presentations Synonym Lumbosacral Plexopathy: Causes Traumatic Causes Retroperitoneal Causes Gynaecological and Obstetric Causes Aortic Causes Neoplastic Causes Other Causes Lumbosacral Radiculoplexus Neuropathy Types Onset Features Features of Pain Features of Weakness Outcome Differential Diagnosis Electromyogram (EMG) Features Nerve Biopsy Features Treatment of Pain Other Treatments Chapter 13 Peripheral Nerve Disorders Neuropathy Causes Demyelinating Peripheral Neuropathy (PN): Causes Genetic Causes Inflammatory Causes Infective Causes Drug-induced Other Causes Hereditary Peripheral Neuropathy (PN): Causes Primary Hereditary Neuropathies Mitochondrial Neuropathies Other Hereditary Neuropathies Peripheral Neuropathy (PN) With Nerve Hypertrophy Commonest Causes Other Causes Usual Affected Nerves Investigations Peripheral Neuropathy (PN) With Spasticity Nutritional Causes Neurodegenerative Causes Systemic Causes Axonal Neuropathy Chronic Idiopathic Axonal Polyneuropathy (CIAP): Differential Diagnoses Metabolic Dysimmune Autoimmune Nutritional Miscellaneous Small Fiber Neuropathy: Causes Genetic Toxic Autoimmune Endocrine and Metabolic Infectious Other Causes Drug-induced Peripheral Neuropathy (PN): Causes Antimicrobials Cardiac Drugs Chemotherapy-induced PN (CIPN) Anti-programmed Death 1 (PD-1) Monoclonal Antibodies Other Drugs Acronym Systemic Vasculitic Peripheral Neuropathy (PN): Causes Primary Systemic Vasculitis Connective Tissue Diseases Inflammatory Conditions Infections Drug-induced Other Causes Sensory Neuronopathy: Causes Autoimmune Causes Platinum-based Chemotherapy Viral Infections Hereditary Causes Degenerative Causes Paraneoplastic Causes Other Causes Synonyms Acquired Demyelinating Neuropathy Guillain–Barre Syndrome (GBS): Non-Infective Triggers Vaccination Drug-induced and Toxic Autoimmune Neoplastic Miscellaneous Guillain–Barre Syndrome (GBS): Clinical Features Peripheral Features Pain Psychiatric Features Cranial Nerve Features Other Features Red Flags Against GBS Guillain–Barre Syndrome (GBS): Complications Neurological Complications Systemic Complications Guillain–Barre Syndrome (GBS): Differential Diagnoses Severe Neuropathies Infective Neuropathies Drug-induced Demyelinating Neuropathies Other Neuromuscular Disorders Other Differentials Guillain–Barre Syndrome (GBS): Treatment Monitoring for Complications Immune Treatments Supportive Treatment Cardiorespiratory Support Multidisciplinary Rehabilitation CIDP: Clinical Features Typical Clinical Features Nerve Hypertrophy Nerve Root Hypertrophy Pseudotumour Syndrome Unusual CIDP Presentations Acronym CIDP: Associated Disorders Paraproteinaemia Infections Inflammatory Conditions Diabetes Mellitus Drug Triggers Other Associations Acronyms CIDP: Investigations Nerve Conduction Studies (NCS): Features Cerebrospinal Fluid (CSF): Features Nerve Ultrasound Autoantibodies Other Investigations Emerging Investigation Acronym CIDP Treatment: IVIg Benefits Indications for First Line Use of IVIg: Ahead of Steroids Protocol Response Predictors of Poor Response Subcutaneous Immunoglobulins (SCIG) Acronym CIDP Treatment: Immunosuppressants Steroids: Benefits Steroids: Options Rituximab Treatments With Poor Evidence Investigational Treatments Scoring Systems Monitoring Acronym Multifocal Motor Neuropathy (MMN): Clinical Features Main Clinical Features Rarer Clinical Features Reported MMN Risk Factors Monofocal Motor Neuropathy Variant Differential Diagnosis: Motor Neurone Disease (MND) Differential Diagnosis: Others Hereditary Neuropathies Charcot–Marie–Tooth Disease 1A (CMT1A) Genetics Main Neurological Features Other Neurological Features Abnormal Gait Patterns Overlap Features Charcot–Marie–Tooth Disease 2A (CMT2A) Genetics and Epidemiology Onset Phenotypes Clinical Features Occasional Features Magnetic Resonance Imaging (MRI) Familial TTR Amyloid Polyneuropathy (FAP TTR): Clinical Features Genetics Peripheral Neuropathy (PN) Patterns Cardiomyopathy Ophthalmic Features Other Features Red Flag Indicators of FAP Mean Duration to Death Familial TTR Amyloid Polyneuropathy (FAP TTR): Treatment Liver Transplantation: Options Tafamidis Diflunisal Inotersen Patisiran Neuropathic Treatments Other Symptomatic Treatments Pre-symptomatic Management Investigational Treatments HNPP: Clinical Features Genetics Clinical Features Frequently Affected Peripheral Nerves Occasionally Affected Cranial Nerves HNPP Phenotypes Unusual HNPP Presentations Acronym Paraproteinaemic Neuropathy IgG and IgA MGUS Paraproteinaemic Neuropathy Criteria for MGUS MGUS Neuropathy Types Treatment: Limited Evidence Acronyms IgM Anti-MAG Paraproteinaemic Neuropathy: Clinical Features Epidemiology Risk Factors Clinical Presentation Typical Clinical Features Atypical Clinical Features Differential Diagnosis of IgM Without Anti MAG Antibody Nerve Conduction Studies (NCS) Outcome Acronyms CANOMAD Paraproteinaemic Neuropathy Demographics Clinical Features Blood Tests Magnetic Resonance Imaging (MRI) Nerve Conduction Studies (NCS) Nerve Biopsy Differential Diagnosis Treatment Acronym Paraproteinaemic Neuropathy: Management General Investigations Conditional General Investigations Neurological Investigations Radiological Investigations Treatment Acronym Mononeuropathies Carpal Tunnel Syndrome (CTS): Causes and Risk Factors Causes Individual Risk Factors Occupational Risk Factors Carpal Tunnel Syndrome (CTS): Clinical Features Pain and Paraesthesias Other Symptoms Clinical Signs Provocative Manoeuvres Differential Diagnosis Cubital Tunnel Syndrome: Causes and Risk Factors Occupational Risks Habitual Risk Factors Medical Causes Orthopaedic Causes Cubital Tunnel Syndrome: Clinical Features Sensory Features Weakness Froment’s Sign Ulnar Paradox Elbow Flexion Sign Other Clinical Signs Differential Diagnosis: C8 Radiculopathy Differential Diagnosis: Others Ulnar Neuropathy: Anomalous Anastomoses Martin-Gruber Anastomosis (MGA) The Riche-Cannieu Anastomosis (RCA) Marinacci Communication Berretini Anastomosis Sciatic Neuropathy: Causes Iatrogenic Posture-related Extrinsic Compression Abscesses Trauma Vascular Lesions Other Causes Sciatic Neuropathy: Clinical Features Sensory Features Motor Features Features of Severe Cases Investigations Predictors of Good Outcome Common Peroneal Neuropathy: Causes Traumatic Causes Orthopaedic Surgery Extrinsic Compression Posture-related Nerve Lesions Other Causes Synonym Common Peroneal Neuropathy: Clinical Features Weakness Sensory Loss: Distribution Preserved Functions Differential: L4/L5 Radiculopathy Synonym Long Thoracic Nerve Palsy: Causes Traumatic Surgical Neuromuscular Infections Drugs and Toxins Other Causes Long Thoracic Nerve Palsy: Occupational Risks Sporting Risks Work Related Risks Military Risks Scapula Winging: Causes Mononeuropathies Other Neurological Causes Soft Tissue and Orthopaedic Causes Scapula Winging: Clinical Features Spinal Accessory Nerve Winging Long Thoracic Nerve Winging Dorsal Scapular Nerve Winging Thoracodorsal Nerve Winging Differential Diagnosis (Mimics) of Scapula Winging Foot Drop: Causes Cranial Causes Spinal Causes Root and Plexus Causes Anterior Horn Cell Disorders Neuropathic Causes Neuromuscular Junction (NMJ) Disorders Muscle Causes Foot Drop: Localisation L5 Root Lesions Sciatic Nerve Lesions (L4–5, S1–3) Common Peroneal Nerve Lesions Superficial Peroneal Nerve Lesions Lesions Causing Inversion Weakness Lesions Causing Dorsiflexion Weakness Lesions Causing Eversion Weakness Lesions Causing Planar Flexion Weakness Diaphragmatic Paralysis: Neurological Causes Radiculopathies Spinal Cord Disorders Anterior Horn Cell (AHC) Disorders Neuropathic Causes Neuromuscular Junction (NMJ) Disorders Muscle Causes Diaphragmatic Paralysis: Systemic Causes Medical Causes Infective Causes Autoimmune Causes Drug-induced and Toxic Causes Diaphragmatic Paralysis: Clinical Features Respiratory Features Sleep-related Features Systemic Features Phrenic Nerve Palsy Neurological Causes Cardiothoracic Causes Infective Causes Syndromic Causes Other Causes Mononeuropathy Multiplex: Causes Vasculitic Causes Infective and Inflammatory Causes Malignant Causes Drug-induced Other Causes Synonym Chapter 14 Neuromuscular Junction Disorders Myasthenia Gravis: General Features Myasthenia Gravis (MG): Classification Early-onset MG With Acetylcholine Receptor Antibodies Late-onset MG With Acetylcholine Receptor Antibodies MUSK Associated MG LRP4 Associated MG Antibody Negative (Seronegative) MG Thymoma Associated MG Ocular MG Other MG Types Acronyms Myasthenia Gravis (MG): Drug Triggers Antibiotics Cardiac Drugs Anaesthetic Drugs Antiepileptic Drugs (AEDs) Anti-inflammatory Drugs Anti-malarial Drugs Other Drugs Myasthenia Gravis (MG): Non-Drug Triggers Environmental Physiological Medical Vaccinations Myasthenia Gravis (MG): Differential Diagnosis Lambert–Eaton Myasthenic Syndrome (LEMS) Congenital Myasthenic Syndromes (CMS) Ophthalmic Differentials Neuromuscular Differentials Other Differentials Monoclonal Antibody-Induced MG Other Drugs Myasthenia Gravis Types Ocular Myasthenia Gravis (MG) Ptosis Enhanced Ptosis Ophthalmoplegia Cogan’s Lid Twitch Other Signs Treatment Options Bulbopharyngeal Myasthenia Gravis (MG) Bulbar Weakness Facial and Neck Weakness Pharyngeal Weakness Generalised Myasthenia Gravis (MG) Limb Girdle Weakness Respiratory Muscle Weakness Chronic Fatigue Juvenile Myasthenia Gravis (MG) Classification Clinical Presentation Thymic Abnormalities Antibody Profile Prognosis Treatment Anti-MUSK Myasthenia Gravis (MG): Clinical Features Pathology Aetiology Genetics Facial and Bulbar Features Ocular Features Generalised Features Anti-MUSK MG and Pregnancy Variant Presentations Features Distinguishing Anti-MUSK From Anti-AChR MG Anti-LRP4 Myasthenia Gravis (MG) Pathology Clinical Features Electromyogram (EMG) Treatment Myasthenia Gravis (MG) With Thymoma Thymic Thymoma Extra-thymic Thymoma Other Thymic Pathologies Myasthenia Gravis With Thymoma Other Thymoma Neurological Manifestations Myasthenia Gravis: Complicated Types Refractory Myasthenia Gravis (MG) Defining Features Treatment: Plasma Exchange (PE) Treatment: Intravenous Immunoglobulins (IVIg) Treatment: Rituximab Treatment: Eculizumab Emerging Treatments Myasthenic Crisis: Risk Factors Disease-related Risk Factors MG Drugs-Related Risk Factors Other Drug Related Risk Factors Medical Risk Factors Others Risk Factors Myasthenic Crisis: Differential Diagnosis Infections and Toxins Peripheral Nerve Disorders Anterior Horn Cell (AHC) Disorders Neuromuscular Junction (NMJ) Disorders Myopathies Muscular Dystrophies Drugs Central Differentials Myasthenia Gravis (MG): Cholinergic Crisis Triggers Neurological Features Systemic Features Edrophonium Test: Benefit Edrophonium Test: Causes of False Positive Worsening Treatment Myasthenia Gravis Treatment Myasthenia Gravis (MG) Treatment: Pyridostigmine Dosing Regime Precautions Side Effects Treatment of Side Effects Myasthenia Gravis (MG) Treatment: Steroids Indications Regime for Ocular MG Regime for Generalised MG Steroid Withdrawal Long-term Steroid Therapy Predictors of Response to Steroids Steroid Dip Precautions On Steroids Myasthenia Gravis (MG): Non-Steroid Immunosuppression Indications for the Sole Use of Non-Steroid Immunosuppressants Indications for Combining Steroids With Non-Steroid Immunosuppressants Azathioprine Methotrexate Other Conventional Options Tacrolimus Lambert–Eaton Myasthenic Syndrome (LEMS) Lambert–Eaton Myasthenic Syndrome (LEMS): Clinical Features Limb Weakness Ptosis Other Neuromuscular Features Autonomic Features Associated Autoimmune Disorders Differential Diagnosis: Myasthenia Gravis (MG) Differential Diagnoses: Others Acronym Lambert–Eaton Myasthenic Syndrome (LEMS): Paraneoplastic Epidemiology Small Cell Cancer (SCLC) Other Associated Cancers DELTA-P Paraneoplastic Score: Benefit DELTA-P Paraneoplastic Score: Scoring Items Investigations for Cancer Lambert–Eaton Myasthenic Syndrome (LEMS): Antibodies P/Q Type VGCC Antibody N Type VGCC Antibodies Anti SOX1 Antibody Anti GRP78 Antibody Acronym Lambert–Eaton Myasthenic Syndrome (LEMS): Treatment 3,4 Diaminopyridine (3,4 DAP) Complementary Treatments to 3,4 DAP Second Line Treatments Other Immunosuppressants Drugs to Avoid Annual Surveillance Congenital Myasthenic Syndromes (CMS) Congenital Myasthenic Syndrome (CMS): Classification By Pathway Presynaptic Defects Synaptic Defects Endplate Development and Maintenance Defects Postsynaptic (Acetylcholine Receptor) Defects Glycosylation Pathway Defects Myasthenia Associated With Centronuclear Myopathies Other Myasthenic Syndromes Congenital Myasthenic Syndrome (CMS): General Features Demographic Features Electromyogram (EMG) Features Differential Diagnosis Congenital Myasthenic Syndrome (CMS): DOK7 Pathology Ophthalmic Features Facial and Bulbar Features Limb Weakness Skeletal Features Respiratory Features Treatment Congenital Myasthenic Syndrome (CMS): RAPSN Genetics and Pathology Onset Clinical Features Treatment Congenital Myasthenic Syndrome (CMS): Fast Channel Pathology Bulbar Symptoms Weakness Ophthalmic Features Foetal Features Electromyogram (EMG) Treatment Congenital Myasthenic Syndrome (CMS) Presenting in Adulthood Frequent Adult Forms Rare Adult Forms Demographic Features Clinical Features Differential Diagnosis Treatment Congenital Myasthenic Syndrome (CMS): Drug Treatment Pyridostigmine: Indications Pyridostigmine: Contraindications 3,4 Diaminopyridine (3,4 DAP): Indications 3,4 Diaminopyridine (3,4 DAP): Contraindications Treatment of Slow Channel Treatment of DOK7 Chapter 15 Muscle Disorders Muscle Symptoms and Signs Dropped Head Syndrome (DHS): Neuromuscular Causes Inflammatory Myopathies Metabolic Myopathies Endocrine Myopathies Other Myopathies Muscular Dystrophies Anterior Horn Cell (AHC) Disorders Neuromuscular Junction Disorders and Neuropathies Dropped Head Syndrome (DHS): Non-Neuromuscular Causes Cervical Diseases Neurodegenerative Causes Miscellaneous Causes Causes of Intermittent Head Drop Myopathy With Respiratory Failure: Causes Metabolic Myopathies Congenital Myopathies EMARDD: Features SMARD1 Other Muscle Diseases Acronym Rapidly Progressive Weakness: Causes Neuropathic Causes Myopathic Causes Other Neurological Causes Infective Causes Systemic Causes Muscle Hypertrophy: Causes Myopathic Causes Muscular Dystrophies Other Muscle Disorders Anterior Horn Cell (AHC) and Root Disorders Neuropathic Disorders Other Causes Fasciculations: Causes Physiological Causes Anterior Horn Cell (AHC) Disorders Nerve Disorders Cerebral Causes Hereditary Spastic Paraplegia (HSP) Systemic Causes Drug-induced Fasciculations Causes of Tongue Fasciculations Camptocormia: Causes Idiopathic Camptocormia Parkinsonian Causes Other Neurodegenerative Causes Neuromuscular Causes Drug-induced Miscellaneous Causes Synonym Inflammatory Myopathies Inflammatory Myopathy: Classification Primary Inflammatory Myopathies Secondary Inflammatory Myopathies Acronyms Dermatomyositis: Clinical Features Possible Risk Factors Major Dermatological Features Other Dermatological Features Neurological Features Oral Features Anti-MDA5 Features Associated Disorders Dermatomyositis: Management Autoantibody Tests Muscle Enzymes Electromyogram (EMG): Features Muscle Biopsy: Features Magnetic Resonance Imaging (MRI): Muscle Polymyositis Demographic Features Neurological Features Cardiac Features Neoplastic Features Differential Diagnosis Immune Mediated Necrotising Myopathy (IMNM): Causes Idiopathic Autoimmune Statins Paraneoplastic Other Causes Synonym Acronyms Immune Mediated Necrotising Myopathy (IMNM): Clinical Features Antibody Profile Clinical Features Features of Seronegative IMNM Muscle Biopsy Other Investigations Differential Diagnosis Prognostic Features Synonym Inclusion Body Myositis (IBM): Risk Factors Infections Anti-cytosolic 5’-Nucleotidase 1A (Anti NT5c1A) VCP-related Multisystem Proteinopathy (MSP): Components Possible IBM Risk Associations Inclusion Body Myositis (IBM): Clinical Features Quadriceps Weakness Long Finger Flexion Weakness: Difficulties Other Weakness Features Peripheral Neuropathy (PN) Spared Muscles and Organs Differential Diagnosis: Motor Neurone Disease (MND) Inflammatory Myopathy: Investigations Electromyogram (EMG): Features Magnetic Resonance Imaging (MRI) Muscle: Features Muscle Biopsy: Features Muscle Ultrasound High Resolution Chest CT: Features Other Tests Inflammatory Myopathy: Treatment Acute Treatment Long-term Treatment Treatment of Refractory Cases JAK Inhibitors: Indications JAK Inhibitors: Types Glycogen Storage Diseases Pompe Disease (GSD Type II): Clinical Features Neuromuscular Features Intracranial Features Skeletal Features Gastrointestinal Features Cardiorespiratory Features Differential Diagnosis Pompe Disease (GSD Type II): Investigations Dried Blood Spot (DBS) Test Tissue GAA Activity Mutational Analysis Prenatal Diagnosis: Techniques Cardiorespiratory Assessments Other Tests Pompe Disease (GSD Type II): Enzyme Replacement Therapy (ERT) Benefits Indications Use in Pregnancy Limitations McArdle’s Disease (GSD Type V): Clinical Features Exercise-induced Symptoms Second Wind Phenomenon Muscle Wasting Other Features Differential Diagnosis Complications McArdle’s Disease (GSD Type V): Management Non-ischaemic Forearm Test Function Tests Muscle Biopsy Other Neurological Tests Genetics Treatment Lipid Storage Myopathies Carnitine Palmitoyl Transferase (CPT II) Deficiency: Clinical Features Epidemiology and Pathology Onset Types Clinical Features Common Triggers for Attacks Uncommon Triggers for Attacks Differential Diagnosis: McArdle’s Disease Synonym Carnitine Palmitoyl Transferase (CPT II) Deficiency: Management Blood Tests Mutations in Blood Cells Muscle Biopsy Treatment Synonym Multiple Acyl-CoA Dehydrogenase Deficiency (MADD): Clinical Features Genetics Pathogenesis Types Muscle Features Systemic Features Features in Crises Other Features Differential Diagnosis Synonym Multiple Acyl-CoA Dehydrogenase Deficiency (MADD): Management Blood Tests Urinary Acid Excretion Muscle Biopsy Magnetic Resonance Imaging (MRI) Muscle Magnetic Resonance Imaging (MRI) Brain: Location of High Signal Changes Treatment Synonym Muscle Channelopathies Neurological Channelopathies: Classification Muscle Channelopathies: Periodic Paralyses Muscle Channelopathies: Non-Dystrophic Myotonias Muscle Channelopathies: Ryanodinopathies Epileptic Channelopathies Pain Syndromes Ataxic Syndromes Other Channelopathy Syndromes Muscle Channelopathies: General Features Myotonia Paramyotonia Episodic Weakness: Triggers Other Features Investigation of Periodic Paralysis Treatment of Non-Dystrophic Myotonia Hypokalaemic Periodic Paralysis: Clinical Features Genetics Transmission Genetic Types Prodrome Clinical Features Differential Diagnosis: Hyperkalaemic Periodic Paralysis Hypokalaemic Periodic Paralysis: Treatment Oral Potassium Intravenous Potassium Potassium Sparing Diuretics Other Diuretics Other Treatments of Attacks Triggers to Avoid Preventative Diet Preventative Potassium Intake Perioperative Measures Hyperkalaemic Periodic Paralysis: Clinical Features Clinical Features Triggers Hyperkalaemic Periodic Paralysis: Management Electromyogram (EMG): McManis Test Preventative Activities Things to Avoid Acute Treatment Thyrotoxic Periodic Paralysis: Clinical Features Genetics and Pathology Demographic Features Prodromal Features Features of Weakness Drug Triggers Other Triggers Differential Diagnosis Thyrotoxic Periodic Paralysis: Management Urine Spot Test Blood Tests Electrocardiogram (ECG) Treatment Malignant Hyperthermia (MH): Clinical Features Neurological Features Rhabdomyolysis Hyperthermia Cardiorespiratory Features Other Reported Associations Complications Malignant Hyperthermia (MH): Management Basic Investigations Magnetic Resonance Imaging (MRI) Muscle In-vitro Contracture Test (IVCT): Types Acute Treatment Prevention of Exertional Rhabdomyolysis Muscular Dystrophies Duchenne Muscular Dystrophy (DMD): Clinical Features Genetics Mobility and Gait Neuromuscular Features Skeletal Deformities Cognitive and Psychiatric Features Cardiorespiratory Complications Gastrointestinal Complications Other Complications Duchenne Muscular Dystrophy (DMD): Cardiac MANAGEMENT Cardiac Monitoring Cardiac Monitoring Tools Electrocardiogram (ECG) Features Echocardiogram Features Cardiac Magnetic Resonance Imaging (MRI) Features Cardiac Treatments Duchenne Muscular Dystrophy (DMD): General Treatments Immunisations Steroids Bone Health Management Other Drugs Orthopaedic Treatments Duchenne Muscular Dystrophy (DMD): Genetic Treatments Eteplirsen (Exondys 51) Ataluren (Translana) Drisapersen Golodirsen Viltolersen Becker Muscular Dystrophy (BMD): Clinical Features Genetics Clinical Features Atypical Features Severe Phenotype Becker Vs Duchenne Muscular Dystrophy (DMD) Treatment Facioscapulohumeral Muscular Dystrophy (FSHD): Genetic Classification FSHD Type 1A (FSHD1A) FSHD Type 1B (FSHD1B) FSHD Type 2 Acronym Facioscapulohumeral Muscular Dystrophy (FSHD): Clinical Features Clinical Phenotypes Features of Facial Weakness Features of Limb Weakness Coat’s Disease Respiratory Impairment: Risk Factors Cardiac Features Other Features Differential Diagnosis Emery–Dreifuss Muscular Dystrophy (EDMD): Clinical Features Genetics Classical Triad Contractures: Affected Joints Contractures: Clinical Features Humeroperoneal Weakness and Atrophy: Affected Muscles Skeletal Features Creatinine Kinase (CK) Cardiac Features Differential Diagnosis Myotonic Dystrophy Type 1: Neurological Features Genetics CTG Repeats Facial Appearance Central Features Peripheral Muscle Features Peripheral Neuropathy (PN) Magnetic Resonance Imaging (MRI): Brain Magnetic Resonance Imaging (MRI): Sites of Fatty Muscle Infiltration Acronym Myotonic Dystrophy Type 1: Major Systemic Features Cardiac Features Respiratory Features Gastrointestinal Features Endocrine and Immune Features Cutaneous Features Myotonic Dystrophy Type 1: Assessments and Monitoring Baseline Assessments Annual Clinical Monitoring Annual Test Monitoring Three-yearly Test Monitoring Neuromuscular Respiratory Specialist Referral: Indications Cardiac Investigations: Indications Sleep Studies: Indications Chapter 16 Tumours Primary Brain Tumours Brain Tumours: Risk Factors Genetic Risk Factors Chemical and Environmental Risk Factors Individual Risk Factors Other Risk Factors Doubtful Risk Factors Factors Which Do Not Increase the Risk of Brain Tumours Factors Which May Reduce the Risk of Brain Tumours Brain Tumour Headaches Epidemiology General Features Migraine-type Headache Features Cluster-type Headache: Causes Atypical Facial Pain Brain Tumour Related Epilepsy (BTRE): Clinical Features Characteristics of Seizure-Related Tumours Seizure Types Poor Seizure Prognostic Features Brain Tumours: Differential Diagnosis Brain Abscess Other Infective Differentials Congenital Infections Vascular Differentials Inflammatory Differentials Phakomatoses and Histiocytosis Other Differentials Low Grade Gliomas: Clinical Features Types Scoring Systems Seizures Poor Prognostic Markers Good Prognostic Markers Meningiomas: Risk Factors Major Tumour Syndromes Copy Number Alterations (CNAs) Major Cancer Predisposition Mutations Other Cancer Predisposition Mutations Epigenomic Alterations: DNA Methylation Epigenomic Alterations: Micro RNA Non-genetic Risk Factors Meningiomas: Radiological Differentials Neoplastic Granulomatous Other Differentials Germ Cell Tumours: Clinical Features Onset Age Frequent Sites Features of Raised Intracranial Pressure (ICP) Visual Features Cranial Nerve Palsies Motor Features Endocrine Features Psychiatric Features Other Features Primary Central Nervous System Lymphoma (PCNSL): Clinical Features Epidemiology Risk Disorders Typical Sites Clinical Features Poor Prognostic Markers Secondary Brain Tumours Brain Metastases: Skull Base Syndromes Orbital Syndrome Parasellar Syndrome Gasserian Ganglion Syndrome Jugular Foramen Syndrome Occipital Condyle Syndrome Neoplastic Meningitis: Clinical Features Epidemiology Typical Primary Sites Meningeal Features Cranial Nerve Features Other Features Synonyms Neoplastic Meningitis: Investigations Cerebrospinal Fluid (CSF): Routine Tests CSF Tumour Markers Measures to Improve CSF Yield Contrast Magnetic Resonance Imaging (MRI) Brain and Spine: Features Radionuclide Studies Meningeal Biopsy Synonyms Paraneoplastic Syndromes Paraneoplastic Neurological Syndromes: Classification Cranial Syndromes Spinal Syndromes Anterior Horn Cell and Plexus Syndromes Ophthalmic Syndromes Autonomic Syndromes Neuromuscular Junction (NMJ) Syndromes Myopathy Syndromes Neuropathy Syndromes Paraneoplastic Neurological Syndromes: Cancer Screening Small Cell Lung Cancer (SCLC) and Thymoma Breast Cancer Ovarian Teratoma and Cancer Testicular Cancer Dermatomyositis: Females Dermatomyositis: Males Phakomatoses Neurofibromatosis Type 1 (NF1): Diagnostic and Neurological Features Diagnostic Criteria Central Neurological Features Peripheral Neuropathy (PN): Types Mosaic Neurofibromatosis Type 1 (MNF1) Neurofibromatosis Type 1 (NF1): Tumours Gliomas Glomus Tumours Neurofibromas: Complications Systemic Tumours Unidentified Bright Objects (UBOs) Emerging Investigations Investigational Drugs for Plexiform Neurofibromas Neurofibromatosis Type 2 (NF2): Clinical Features Features of Vestibular Schwannomas Ophthalmic Features Other Features Differential Diagnoses of Unilateral Vestibular Schwannomas Neurofibromatosis Type 2 (NF2): Tumours Vestibular Schwannomas Schwannomas: Other Locations Meningiomas: Locations Meningiomas: Implications Gliomas Other Tumours Schwannomatosis (SWN): Clinical Features Genetic Features Genetic Mutations Demographic Features Presenting Features Synonym Schwannomatosis (SWN): Tumours Typical Features Usual Locations Unusual Locations Associated Tumours Synonym Tuberous Sclerosis Complex (TSC): Neuropsychiatric Features Seizure Features TSC-associated Neuropsychiatric Disorder (TAND) Cognitive Impairment: Predictors Other Neurological Features Tuberous Sclerosis Complex (TSC): Lesions Cortical Tubers: Imaging Features Hamartomas: Locations Other Lesions Systemic Lesions Sturge–Weber Syndrome (SWS): Clinical Features Genetics Central Neurological Features Peripheral Neurological Features Ophthalmic Features Cutaneous Haemangiomas Juvenile Ossifying Fibroma Differential Diagnosis Synonym Acronym Von Hippel-Lindau Disease (VHL): Clinical Features Type 1 VHL Type 2 VHL Clinical Features Monitoring Chapter 17 Metabolic and Mitochodrial Disorders Lysosomal Storage Diseases Fabry Disease: Neurological Features Genetic Features Demographic Features Thrombotic Stroke Embolic Stroke: Risks Other Central Nervous System Features Small Fiber Peripheral Neuropathy (PN) Episodic Pain (Fabry Crises): Triggers Acroparaesthesias: Differential Diagnosis Fabry Disease: Systemic Features Cardiac Features Ophthalmic Features Angiokeratoma Corporis Diffusum Other Dermatological Features Other Systemic Features Fabry Disease: Management Blood Tests Magnetic Resonance Imaging (MRI) Brain: Features Magnetic Resonance Spectroscopy (MRS) Slit Lamp Examination Findings Other Investigations Treatment of Crises Definitive Treatments: Migalastat Definitive Treatments: Others Niemann–Pick C (NPC): Clinical Features Genetic Mutations Pathological Features Neurological Features Psychiatric Features Vertical Supranuclear Gaze Palsy (VSGP) Slow Saccades Systemic Features Differential Diagnosis Krabbe Disease: Clinical Features Genetics and Pathology Types Developmental Features Pyramidal Features Movement Disorders Other Features Magnetic Resonance Imaging (MRI) Features Synonym Leukodystrophies Alexander Disease: Clinical Features Genetics and Pathology Types Brainstem Features Other Features Rare Features Features of Infantile Onset Adrenoleukodystrophy (ALD): Neurological Features Genetics and Pathology Clinical Phenotypes Cerebral Features Psychiatric Features Other Neurological Features Features of Adrenomyeloneuropathy (AMN) Course and Outcome Adrenoleukodystrophy (ALD): Systemic Features Addisonian Features Other Endocrine Features Features of Female Heterozygote Carriers Acronym Peroxisomal Disorders Refsum’s Disease: Clinical Features Genetic Mutations Pathology Tapetoretinal Degeneration: Features Other Ophthalmic Features Skeletal Malformations Neurological Features Systemic Features Triggers for Deterioration Cerebrotendinous Xanthomatosis (CTX): Clinical Features Genetics and Pathology Main Features Cranial Features Spinal Features Peripheral Features Psychiatric Features Systemic Features Differential Diagnosis Tangier Disease Genetics Pathology Lymphoid-related Features Peripheral Neuropathy (PN) Ophthalmic Features Other Features Differential Diagnosis Urea Cycle Disorders Urea Cycle Disorders: Clinical Features Types of Urea Cycle Disorders Demographic Features Clinical Features Triggers for Crises Ornithine Transcarbamylase (OTC) Deficiency: Clinical Features Genetics and Pathology Clinical Features Triggers of Hyperammonemic Crises Outcome Ornithine Transcarbamylase (OTC) Deficiency: Management Serum Tests Other Tests Nutritional Supplementation Treatment: Sodium Scavenging Drugs Treatment: Others Porphyria Porphyria: Clinical Features and Treatment Neurological Features Systemic Features Triggers for Acute Attacks Chronic Features Treatment Investigational Treatment Porphyria: Peripheral Neuropathy (PN) Clinical Features Autonomic Features Differential Diagnosis Cerebrospinal Fluid (CSF) Analysis Nerve Conduction Studies (NCS) Prevention Porphyria: Drug Safety Unsafe Drugs Potentially Unsafe Drugs Potentially Safe Drugs Mitochondrial Disorders: Phenotypes and Features Mitochondrial Diseases: Neurological Features Peripheral Neuropathy (PN): Common Causes Movement Disorders Ophthalmic Features Miscellaneous Neurological Features Psychiatric Features Mitochondrial Stroke-Like Episodes (SLEs) Pathology Epidemiology Clinical Features Radiological Features Manifestations of Chronic Lesions Potential Treatments Acronym Mitochondrial Epilepsies Mitochondrial Diseases With Epilepsy Presentations Clinical Features Mitochondrial Optic Neuropathies and Myopathies Causes of Mitochondrial Optic Neuropathy Role of Mitochondria in Other Optic Neuropathies Causes of Mitochondrial Myopathy Mitochondrial Diseases: Systemic Features Cardiorespiratory Features Renal Features Endocrine Features Gastrointestinal Features Haematological Features Other Systemic Features Neurological Mitochondrial Disorders Chronic Progressive External Ophthalmoplegia (CPEO) Genetic Mutations Ophthalmic Features Muscle Features Cardiac Features Other Features Acronym Kearns-Sayre Syndrome (KSS) Ophthalmic Features Neurological Features Endocrine Features Cardiac Features Cerebrospinal Fluid (CSF) Analysis Magnetic Resonance Imaging (MRI): Sites of Lesions Magnetic Resonance Imaging (MRI): Other Features Treatment With Coenzyme Q Leber Hereditary Optic Neuropathy (LHON): Clinical Features Genetic Point Mutations Genetics Onset Features Central Visual Loss Visual Signs Cardiac Features Neurological Features LHON+ MELAS: Clinical Features Stroke-like Features Headaches Cognitive and Encephalopathic Features Other Features Suggested Expanded Phenotype: MCARNE Differential Diagnosis Acronym MERRF: Clinical and Laboratory Features Genetics: A8344G Neurological Features Systemic Features Magnetic Resonance Imaging (MRI) Muscle Biopsy Acronym Mitochondrial Polymerase Gamma (POLG): Phenotypes Progressive External Ophthalmoplegia (PEO) Epilepsy: Types SANDO Childhood-onset Alpers Syndrome Distal Myopathy With Cachexia Childhood-onset Developmental Syndrome SANO Other Phenotypes Acronyms Mitochondrial Disorders Management Mitochondrial Diseases: Investigations Blood Tests Genetic Tests Cardiorespiratory Tests Urine Analysis Cerebrospinal Fluid (CSF) Electroencephalogram (EEG) Brain Imaging: Indications Muscle Biopsy: Histochemistry Muscle Biopsy: Other Studies Other Investigations Mitochondrial Diseases: Surveillance Monitoring Ophthalmology Referral: Indications Other Referrals Mitochondrial Diseases: Specific Treatments Metabolic Treatments L-Arginine Treatments of MNGIE Other Treatments Acronyms Mitochondrial Diseases: Symptomatic Treatments Treatment of Seizures Treatments of Myoclonus in MERRF Cardiac Treatments Other Treatments Drugs and Activities to Avoid Acronyms Chapter 18 Developmental Disorders Systemic Developmental Disorders Cerebral Palsy (CP): Clinical Features Risk Factor Types Visual Features Neurological Features Feeding Difficulties Peripheral Features Autonomic Features Skeletal Features Cerebral Palsy (CP): Management Neuroimaging Electroencephalography (EEG) Screening Metabolic and Genetic Tests: Indications Treatment of Dystonia Single Level Selective Dorsal Rhizotomy (SDR): for Spasticity Investigational Treatments Autism Spectrum Disorders (ASD): Risk Factors PTEN Gene Mutation Epilepsy Other Neurological Risk Factors Other Risk Factors Autism Spectrum Disorders (ASD): Clinical Features Motor Features Cognitive Features Sleep-related Features Psychiatric Features Gastrointestinal Features Other Features Differential Diagnosis: Childhood Disintegrative Disorder (CDD) Intracranial Developmental Disorders Arachnoid Cysts: Features Pathology Genetic Transmission of Familial Types Locations Cognitive Impairments Clinical Features Complications of Cyst Rupture Associated Disorders Radiological Differential Diagnosis Arachnoid Cysts: Treatment Surgical Indications Surgical Treatments Complications of Treatment Precautions Dandy–Walker Syndrome Diagnostic Features Other Cranial Features Systemic Features Pettigrew Syndrome (X-Linked Dandy–Walker Syndrome) Radiological Features Treatment Agenesis of the Corpus Callosum: Clinical Features Anatomical Associations Abnormal Systems Clinical Associations Spinal Developmental Disorders Chiari Malformation: Classification Classification of Chiari Malformation Chiari Malformation: Clinical Features Headaches: Exacerbating Manoeuvres Lower Brainstem and Cerebellar Features Features of Raised Intracranial Pressure (ICP) Spinal Cord Features Sleep Disorders Other Features Clinical Outcome Measures Spina Bifida: Pathology and Risk Factors Pathology Types Genetic Risk Factors Maternal Risk Factors Possible Risk Factors Spina Bifida: Clinical Features Spinal Cord Features Skeletal Abnormalities Features of Tethered Cord Local Skin Lesions Ocular Abnormalities Urogenital Features Other Features Spina Bifida: Complications and Management Complications Magnetic Resonance Imaging (MRI): Features Screening for Spina Bifida Treatment Syringomyelia Classification Causes Clinical Features Differential Diagnosis Treatment Chapter 19 Allied Neurological Disorders Neuro-Ophthalmology Ptosis Central Neurological Causes Peripheral Neurological Causes Congenital Causes Structural Causes Medical Conditions Enhanced Ptosis Differential Diagnosis of Ptosis Acronym Ross Syndrome Clinical Features Pathology Associations Differential Diagnosis Treatment Argyll Robertson Pupil Clinical Features Causes Differential Diagnosis Harlequin Syndrome Clinical Features Possible Associated Features Pathology Neurological Causes Iatrogenic Causes Neoplastic Causes Vascular Causes Other Causes Treatment Horner’s Syndrome Central Causes Preganglionic Causes Postganglionic Causes Clinical Features: Anhidrosis Clinical Features: Others Assessments Reverse Horner’s Syndrome Pathogenesis Iatrogenic Causes Other Causes Clinical Features Treatment Synonym Neurotology Dizziness: Causes Vestibular Neurological Cardiovascular Medical Functional Persistent Postural Perceptual Dizziness (PPPD) Pathology and Epidemiology Core Features Associated Features Provoking Factors and Triggers Diagnostic Criteria Treatment Synonyms Vertigo: Medical Causes Vestibular Causes Neurological Causes Systemic Causes Posterior Canal BPPV Causes Clinical Features Risk Factors Co-morbidities Provoking Tests Evidenced Treatments Insufficient-evidenced Treatments Non-evidenced Treatments Acronym Horizontal Canal BPPV Demographic Features Secondary Causes Diagnostic Manoeuvres Treatment: Vannucchi-Asprella (Liberatory) Manoeuvre Acronym Tinnitus: Causes Neurological Causes Otological Causes Systemic Causes Pulsatile Tinnitus: Causes Vascular Fistulae Other Vascular Causes Neoplastic Causes Downbeat Nystagmus Classification Idiopathic: Types Secondary: Causes Clinical Features Functional Magnetic Resonance Imaging (MRI) Treatment Psychiatry Othello Syndrome Clinical Features Neurodegenerative Causes Focal Cerebral Causes Psychiatric Causes Drug-induced Capgras Syndrome: Clinical Features Pathology Disconnected Areas People Frequently Presumed to Be Replaced Variations of Affected Replaced People Or Places Associated Hallucinations Synonyms Capgras Syndrome: Causes Psychoses Neurodegenerative Diseases Neurological Infections Other Neurological Causes Toxic Causes Metabolic Causes Other Causes Synonyms Attention Deficit Hyperactivity Disorder (ADHD): Clinical Features Epidemiology Core Clinical Features Subtypes Manifestations Adult ADHD Rating Scales Attention Deficit Hyperactivity Disorder (ADHD): Risk Factors and Comorbidities Acquired Risk Factors Genetic Risk Factors Psychiatric Co-Morbidities Cognitive Co-Morbidities Neurological Co-Morbidities Medical Co-Morbidities Neurosurgery Post-concussion Syndrome (PCS) Definitions Related to Concussion Risk Factors General Features Neuropsychiatric Features Other Features Risk Factors for Progression Treatment Normal Pressure Hydrocephalus (NPH): Risk Factors Acquired Risk Factors Genetic Risk Factors Normal Pressure Hydrocephalus (NPH): Clinical Features Onset Features Diagnostic (Hakim) Triad Cognitive Features Gait Disturbance: Types Gait Features Posture Features Urinary Impairment Associated Features Co-morbid Conditions Normal Pressure Hydrocephalus (NPH): MRI Features MRI Measurements Ventricular Features Cortical Features Pain Management Complex Regional Pain Syndrome (CRPS): Triggers Trauma Neurological Medical Infective Drugs Other Possible Triggers Complex Regional Pain Syndrome (CRPS): Clinical Features Pain Features Motor Features Movement Disorders Autonomic Features Other Features Co-morbidities Facial Pain: Typical Causes Ocular Causes Facial Nerve Related Facial Neuralgias Primary Headaches Vascular Causes Structural Causes Acronym Facial Pain: Atypical Causes Pourfour Du Petit’s Syndrome Referred Facial Pain: Origins Other Atypical Causes Neuroradiology Bilateral Thalamic Lesions Toxic Causes Metabolic Causes Degenerative Causes Vascular Causes Inflammatory and Infective Causes Neoplastic Causes Cerebellopontine Angle (CPA) Lesions Vestibular Schwannomas Other Tumours Non-tumour CPA Lesions Uncommon Lesions Enhancing Meningeal Lesions Extra-axial Leptomeningeal (pia-Arachnoid) Linear Pachymeningeal (dura-Arachnoid) Superficial Gyral Nodular Subcortical Deeper Lesions Infective Causes Other Causes Neuropharmacology Intravenous Immunoglobulins (IVIg): Use Definite Indications: Level A Evidence Conditional Indications: Second- Or Third-Line Treatments Conditions With Insufficient Evidence of Benefit Precautions Monitoring Tests Intravenous Immunoglobulins (IVIg): Complications Thromboembolic Stroke Other Major Complications Other Complications Steroid Therapy Main Precautions Dietary Precautions Bone Protection Precautions Tuberculosis Prophylaxis: Indications Pneumocystis Jirovecii (Carinii) Prophylaxis Other Precautions Indications for Not Restricting Live Vaccines On Steroids Monitoring Tests On Steroids Obstetric Neurology Epilepsy: Management in Pregnancy Pre-conception Counselling Pre-conception Supplementation Intra-partum Management Post-partum Management: If Mother Is On an Enzyme-Inducing AED Antiepileptic Drugs (AEDs) in Pregnancy Guidelines for Using Contraceptives in Epilepsy Considerations for Using Valproate in Women With Childbearing Potential Blood Monitoring of AED Levels in Pregnancy: Indications Causes of Headaches in Pregnancy Preeclampsia: Criteria Migraine Other Primary Headaches Meningitis Vasculopathies Other Causes Causes of Acute Post-Partum Headache Migraine Treatment in Pregnancy Antiemetics Triptans Aspirin Other Analgesics Betablockers Other Prophylactic Drugs Contraindicated Drugs Relatively Contraindicated Steroids Stroke in Pregnancy: Causes Pregnancy-induced Gestational Hypertension: Risk Factors Preeclampsia and Eclampsia: Features HELLP Syndrome: Features Hypercoagulable States Other Causes of Ischaemic Stroke Causes of Subarachnoid Haemorrhage (SAH) Causes of Intracerebral Haemorrhage (ICH) Neurological Complications of Labour Post-partum Nerve Injuries Transient Neurological Symptoms (TNS) With Caesarean Delivery Other Neurological Complications of Labour Functional Neurology Functional Movement Disorders: General Features Demographic Features Types Clinical Features The ‘whack-A-Mole’ Sign The ‘Huffing and Puffing’ Sign Associations Functional Dystonia General Features Functional Generalised Dystonia Functional Blepharospasm Functional Dysphonia Functional Tremor Demographic Features Course Characteristics Associated Disorders Positive Entrainment Test Positive Co-Activation Test Other Positive Tests Treatment Functional Parkinsonism General Features Characteristics of Tremor Characteristics of Rigidity Characteristics of Bradykinesia Other Features Functional Seizures General Features Characteristic Movements Panic Symptoms Eye Features Cognitive Features Frequent Co-Morbidities Synonyms Functional Hemiparesis Clinical Features Distinctive Clinical Signs Co-morbid Psychiatric Disorders Social Impact Predictors of Good Outcome Chapter 20 Systemic Neurological Disorders Cardiac Atrial Fibrillation (AF) and Stroke Risk Embolic Stroke Risk With AF Embolic Stroke Risk Factors With AF CHA2DS2-VASc Stroke Risk Prediction Items CHA2DS2-VASc Risk Estimation Patent Foramen Ovale (PFO) and Migraine Epidemiology of PFO and Migraine Drug Treatment Preventive Measures Benefit of PFO Closure Indications for PFO Closure Complications of PFO Closure Patent Foramen Ovale (PFO) and Stroke: Clinical Aspects Epidemiology of PFO and Stroke Predictors of Stroke With PFO Predictors of Recurrent Stroke With PFO Medical Treatment Patent Foramen Ovale (PFO) and Stroke: PFO Closure Benefits for PFO Closure Precautions Before PFO Closure PFO Closure Techniques Risks of PFO Closure Device-related PFO Closure Adverse Events Syncope Syncope: Classification Cardiac Syncope: Types Reflex Syncope: Types Situational Syncope: Causes Drug-induced Syncope: Causes Other Forms of Syncope Neurocardiogenic Syncope Clinical Features Triggers Differential Diagnosis Synonym Syncope: Differential Diagnosis Neurological Differentials Medical Differentials Cardiovascular Differentials Syncope: Differential Diagnosis From Seizures Prodromal Predictors of Syncope Ictal Predictors of Syncope Post-ictal Predictors of Syncope Therapeutic Predictors of Syncope Electroencephalogram (EEG) Predictors of Syncope Syncope: Preventive Manoeuvres Adequate Fluid Intake Preventative Postures Other Preventative Measures Syncope: Interventional Treatments Midodrine: First Line Droxidopa: First Line Fludrocortisone: Second Line Pyridostigmine Other Drugs to Consider Pacemaker: Indications Respiratory Neuromuscular Respiratory Dysfunction: Causes Anterior Horn Cell (AHC) Disorders Peripheral Nerve Disorders Neuromuscular Junction (NMJ) Disorders Muscle Diseases Toxins Other Causes Pulmonary Arteriovenous Malformation (PAVM) Genetic Mutations Cardiorespiratory Features Neurological Presentations Systemic Features 100% Oxygen Screening Test Investigations Treatment Hereditary Haemorrhagic Telangiectasia (HHT) Genetic Types Curacao Diagnostic Criteria Arteriovenous Malformations (AVMs): Epidemiology Arteriovenous Malformations (AVMs): Sites Telangiectasias (Dilated Blood Vessels): Sites Haemorrhagic Features Cerebral Features Pulmonary Features Brain Magnetic Resonance Imaging (MRI) Synonym Rheumatology Antiphospholipid Syndrome (APS): Neurological Features Demographic Features Vascular Features Neuroinflammatory Features Cognitive Features Movement Disorders Psychiatric Features Peripheral Neurological Features Other Features Antiphospholipid Syndrome (APS): Systemic Features Underlying Disorders Thromboembolic Features Haematological Features Dermatological Features Obstetric Features Vascular Features Systemic Lupus Erythematosus (SLE): Neurological Features Inflammatory and Immune Psychiatric Peripheral Other Features Rheumatoid Meningitis Epidemiology Features of Rheumatoid Meningitis Features of Rheumatoid Pachymeningitis Autoimmune Tests Magnetic Resonance Imaging (MRI) Brain: Features Cerebrospinal Fluid (CSF) Analysis Brain Biopsy: Features Treatment Treatment of Rheumatoid Pachymeningitis Sjogren’s Syndrome: Neurological Features Demographic Features Variant Presentations Central Features Dorsal Root Ganglionopathy (DRG) Cranial Mononeuropathies Peripheral Neuropathy (PN): Types Other Features Systemic Sclerosis (SS): Neurological Features Central Neurological Features Psychiatric Features Peripheral Neuropathy (PN) Mononeuropathies Cranial Neuropathies Myopathies Other Features Synonym Endocrine Thyrotoxicosis Ophthalmological Features Cerebral Features Psychiatric Features Vascular Features Other Features Autoimmune Neurological Associations Hypothyroidism Central Neurological Features Peripheral Neuropathy Muscle Features Muscle Syndromes Psychiatric Features Diabetic Neuropathy: Types Diabetic Peripheral Neuropathy Types Diabetic Cranial Neuropathy Diabetic Mononeuropathy Diabetic Radiculopathy Insulin Neuritis Haematology Sickle Cell Disease (SCD): Neurological Features Stroke: Types Other Haemorrhages Acute Painful Crisis Chronic Pain Other Neurological Features Hodgkin’s Lymphoma: Neurological Features Parenchymal Features Leptomeningeal Features Paraneoplastic Features Radiotherapy-related Features Chemotherapy-related Features Other Affected Sites Non-Hodgkin’s Lymphoma (NHL): Neurological Features Guillain–Barre Syndrome (GBS) Spinal Cord Features Peripheral Nerve Features Neurolymphomatosis Other Features Nutritional Subacute Combined Degeneration (SCD) Causes Clinical Features Associated Features of B12 Deficiency Magnetic Resonance Imaging (MRI): Features Differential Diagnosis Treatment Alcohol Syndromes: Classification Direct Alcohol Effects Alcohol-related Movement Disorders Alcohol Withdrawal-Related Movement Disorders Metabolic Syndromes Degenerative and Demyelinating Syndromes Other Alcohol Syndromes Alcohol-triggered Neurological Disorders Bariatric Surgery: Neurological Syndromes Peripheral Neuropathies Mononeuropathies Stretch and Traumatic Injuries Other Neurological Syndromes Prevention Gluten Sensitivity Neurology Movement Disorders Gobbi Syndrome Other Epilepsy Syndromes Other Central Neurological Features Psychiatric Features Peripheral Neurological Features Restless Legs Syndrome (RLS) Magnetic Resonance Imaging (MRI) Features Disputed Gluten Sensitivity Syndromes Synonym Renal Uraemic Encephalopathy Cognitive Features Frontal Lobe Dysfunction Movement Disorders Neuropsychiatric Features Pyramidal Features Other Features Electroencephalogram (EEG) Renal Dialysis: Neurological Complications Vascular Complications Sleep Impairments Peripheral Complications Wernicke’s Encephalopathy: Risk Factors Other Neurological Complications of Dialysis Imaging Features Vasculitis Vasculitis: Classification Large Vessel Vasculitis Medium Vessel Vasculitis Small Vessel Vasculitis: ANCA Associated (AAV) Small Vessel Vasculitis: Others Variable Vessel Vasculitis Single Organ Vasculitis Vasculitis With Systemic Diseases Vasculitis With Probable Aetiology Other Primary Vasculitis Syndromes Vasculitis: Manifestations Systemic Symptoms Features of Large Vessel Vasculitis Features of Medium Vessel Vasculitis Features of Small Vessel Vasculitis Giant Cell Arteritis (GCA): Clinical Features Phenotypes Systemic Features Ophthalmological Features Neurological Features Differential Diagnosis Synonym Surgery Neurological Complications of Cardiac Surgery Stroke: Risk Factors Stroke: Risk Prediction Encephalopathy: Causes Miscellaneous Complications Neurological Complications of Organ Transplantation Demographic Features Causes and Risk Factors Vascular Complications Neoplastic Complications Post-transplant Autoimmune Encephalitis Other Neurological Complications Calcineurin-inhibitor Neurotoxicity: Features Magnetic Resonance Imaging (MRI): Features Neurochecklists Complete Index of Online Topics A.disorders of Cognition and Consciousness AA. Dementia AB. Amnestic Syndromes AC. Encephalopathy AD. Speech Disorders AE. Prion Diseases AF. Disorders of Consciousness B. Epilepsy BA. Seizure Clinical Features BB. Major Epilepsy Syndromes BC. Myoclonus Syndromes BD. Progressive Myoclonic Epilepsy (PME) BE. Complicated Epilepsy Syndromes BF. Epilepsy and Medical Disorders BG. Epileptic Encephalopathy BH. Epilepsy Management C. Sleep Disorders CA. Sleep Disorders: General Aspects CB. Primary Sleep Disorders CC. Rem Sleep Parasomnias CD. Other Sleep Disorders D. Movement Disorders DA. Parkinson’s Disease (PD) DB. Parkinsons Plus Syndromes DC. Dystonia DD. Tremor Ataxia DF. Chorea DG. Drug-induced Movement Disorders DH. Other Movement Disorders E. Neuroinflammatory and Autoimmune Disorders EA. Multiple Sclerosis (MS) EB. Neuromyelitis Optica (NMO) EC. Neurosarcoidosis ED. Other Neuroinflammatory Disorders EE. Autoimmune Encephalitis EF. Peripheral Autoimmune Disorders F. Infections FA. Viral Infections FB. Bacterial Infections FC. Parasitic Infections FD. Fungal Infections FE. Non-infective Meningitis FF. Cerebrospinal Fluid (CSF) G. Headache GA. Migraine GB. Trigeminal Autonomic Cephalalgias (TACS) GC. Idiopathic Intracranial Hypertension (IIH) GD. Low Intracranial Pressure Headaches GE. Tension and Other Chronic Headaches GF. Distinctive and Unusual Headaches H. Vascular Disorders HA. Ischaemic Stroke HB. Haemorrhagic Stroke HC. Vascular Malformations HD. Vasculopathies HE. Venous Disroders HF. Small Vessel Disorders HG. Miscellaneous Vascular Disorders I. Cranial Nerve Disorders IA. Optic Nerve IB. Trigeminal Nerve IC. Facial Nerve ID. Vagus Nerve IE. Vestibulochochlear Nerve IF. Other Cranial Nerves IG. Cranial Nerve Associated Disorders J. Spinal Cord Disorders JA. Myelopathy JB. Hereditary Spastic Paraplegia (HSP) JC. Spinal Cord Tumours JD. Other Spinal Cord Disorders K. Anterior Horn Cell Disorders KA. Motor Neurone Disease (MND) KB. Spinal Muscular Atrophy (SMA) KC. Other Anterior Horn Cell Disorders L. Roots and Plexus Disorders LA. Radicular Disorders LB. Plexus Disorders M. Peripheral Nerve Disorders MA. Neuropathy Causes and Clinical Assessments MB. Axonal Neuropathy MC. Acquired Demyelinating Neuropathies MD. Charcot–Marie–Tooth Disease (CMT) ME. Other Hereditary Neuropathies MF. Paraproteinaemic Neuropathies MG. Mononeuropathies MH. Neuropathy Investigations N. Neuromuscular Junction Disorders NA. Myasthenia Gravis (MG) NB. Lambert–eaton Myasthenic Syndrome (LEMS) NC. Congenital Myasthenic Syndromes (CMS) O. Muscle Disorders OA. Muscle Symptoms and Signs OB. Inflammatory Myopathies OC. Glycogen Storage Diseases (GSD) OD. Lipid Storage Myopathies OE. Muscle Channelopathies OF. Congenital Myopathies OG. Other Myopathy Syndromes OH. Drug-induced Myopathies OI. Cramps and Rhabdomyolysis OJ. Muscular Dystrophy OK. Muscle Investigations P. Tumours PA. Primary Brain Tumours PB. Secondary Brain Tumours PC. Paraneoplastic Syndromes PD. Histiocytic Tumours PE. Phakomatoses PF. Other Brain Tumours and Cysts Q. Metabolic Disorders QA. Metabolic Disorders: Classifications QB. Lysosomal Storage Disorders QC. Leukodystrophies QD. Peroxisomal Disorders QE. Urea Cycle and Fatty Acid Disorders QF. Other Metabolic Disorders R. Mitochondrial Disorders RA. Mitochondrial Disorders: Features and Phenotypes RB. Neurological Mitochondrial Disorders RC. Other Mitochondrial Disorders RD. Mitochondrial Diseases Management S. Developmental Disorders SA. Systemic Developmental Disorders SB. Intracranial Developmental Disorders SC. Corpus Callosum Disorders SD. Cranial Developmental Disorders SE. Spinal Developmental Disorders SF. Ciliopathies SG. Neurochristopathies SH. Rasopathies SI. Other Developmental Disorders T. Allied Neurological Disorders TA. Neurophthalmology TB. Neurotology TC. Psychiatry TD. Neurosurgery TE. Pain Management TF. Neuroradiology TG. Neurogenetics TH. Neuropharmacology TI. Obstetric Neurology TJ. Functional Neurology TK. Neurotoxicity TL. Other Allied Neurology TM. Neurology Guidance U. Systemic Neurological Disorders UA. Cardiac UB. Syncope UC. Respiratory UD. Rheumatology UE. Endocrine UF. Haematology UG. Nutritional UH. Renal UI. Vasculitis UJ. Surgery UK. Diving and Flying