Merritt’s Neurology

 

Description:

For more than 60 years, Merritt’s Neurology has remained a trusted landmark text in clinical neurology, providing unparalleled guidance on neurologic protocols, treatment guidelines, clinical pathways, therapeutic recommendations, and imaging. The fourteenth edition reflects the state of today’s practice, with fully updated content and timely new sections and chapters.  With this edition, Dr. James Noble joins Drs. Elan Louis and Stephan A. Mayer as co-editor, all of whom trained at Columbia University where Dr. H. Houston Merritt wrote the initial editions of this book. Lauded for its comprehensive coverage, colorful and dynamic visual style, readability, and ease of use, this up-to-date reference is ideal for neurologists, primary care physicians, and residents alike.

 

Table of Contents:

 

SECTION I GLOBAL BURDEN OF NEUROLOGIC DISEASE

1 Worldwide Epidemiology of Neurologic Disease

INTRODUCTION

THE CLASSIFICATION OF DISEASE: WHAT IS A NEUROLOGIC DISORDER?

ESTIMATING DISEASE BURDEN

Mortality

Morbidity

Life Expectancy

THE SHIFTING CHARACTER OF NEUROLOGIC DISEASE OVER TIME

THE EPIDEMIOLOGIC TRANSITION AND THE DOUBLE BURDEN OF DISEASE

DISPARITIES

Stroke

Chronic Neurological Diseases

Access to Care

SOCIOCULTURAL DIFFERENCES IN INTERPRETATION OF DISEASE

SUMMARY

2 Global Delivery of Neurologic Care

GAPS IN GLOBAL ACCESS TO NEUROLOGIC CARE

Components Required for Delivery of Neurologic Care

CHALLENGES IN ADDRESSING NEUROLOGIC DISORDERS

Stigma Related to Neurologic Disorders

Challenges in Health-Seeking Behavior

Limited Global Access to Diagnostics and Therapeutics for Neurologic Disorders

Gaps in National Policies and Guidelines for Brain Health

Approaches and Strategies to Improve Delivery of Neurologic Care Globally

Collaborative Partnerships and Coordinated Action

Improving Access to Neurologic Care: Primary Care Workforce

TELEMEDICINE AND INFORMATION AND COMMUNICATION TECHNOLOGY

Synchronous Approaches

Asynchronous Approaches

Training Approaches to Build Capacity

Community Engagement

Programs Aimed to Raise Public Awareness

CONCLUSION

SECTION II APPROACH TO THE NEUROLOGIC PATIENT

3 Neurologic History Taking, Localization, and Differential Diagnosis

INTRODUCTION TO CLINICAL DIAGNOSTIC REASONING

INTENTIONAL EXPLORATION OF THE PATIENT HISTORY

Identifying Localization

HISTORY OF PRESENT NEUROLOGIC ILLNESS

Sources of Information

Approach to Taking the History

Primary Components of the History

Localization

Timing, Onset, Tempo, and Duration

Quality of Subjective Experiences

Alleviating and Aggravating Factors

Related Symptoms

Behaviors Only Observable by Others

Further Shaping the History and Differential

Age of the Patient

Gender Specificity

Technology as an Assistive Tool

Presymptomatic or Asymptomatic Genetic Variants

Neurologic Symptoms in Nonneurologic Disease

Problem-Based History

PAST MEDICAL, PSYCHIATRIC, AND SURGICAL HISTORIES

NEUROLOGIC REVIEW OF SYSTEMS

SOCIAL HISTORY

Travel and Occupational History

Dietary and Nutritional History

FAMILY HISTORY

DIAGNOSTIC SYNTHESIS

ACKNOWLEDGMENT

LEVEL 1 EVIDENCE

4 The Neurologic Examination

INTRODUCTION

Setting Goals of Examination Versus Neurologic Tests

A Top-Down Approach

Helping Your Patient Through the Neurologic Examination

Soft Versus Hard Neurologic Examination Findings

Descriptions Versus Impressions

Positioning the Patient (and Examiner)

Careful Observation and Examination Interpretation

THE PATIENT EXAMINATION

General Medical Examination

Mental Status Examination

Determining Alertness

Language

Memory Testing

Calculation and Other Tasks of Attention and Concentration

Executive Function

Visuospatial Function

Higher Order Cognition

Key Psychiatric Examination Elements

Cranial Nerve Examination

Cranial Nerve I (Olfactory Nerve)

Cranial Nerve II (Optic Nerve)

Extraocular Movements (Cranial Nerves III [Oculomotor Nerve], IV [Trochlear Nerve], and VI [Abducens Nerve])

Cranial Nerve V (Trigeminal Nerve)

Cranial Nerve VII (Facial Nerve)

Cranial Nerve VIII (Vestibulocochlear Nerve)

The Lower Cranial Nerves Involved in Speech (IX [Glossopharyngeal Nerve], X [Vagus Nerve], and XII [Hypoglossal Nerve])

Cranial Nerve XI (Accessory Nerve)

Motor Examination

Coordination

Gait, Station, and Body Movements

Deep Tendon and Other Reflexes

Direct Muscle Percussion

Sensory Examination

Additional Neurologic Tests for Special Conditions

Dix-Hallpike Maneuver

The Head Impulse Test

Phalen and Tinel Signs

Straight Leg Raise

Additional Spinal Reflexes (Male Patients Only)

Anal sphincter Tone

Tests of Malingering

SECTION III COMMON PROBLEMS IN NEUROLOGY

5 Dizziness, Vertigo, and Hearing Loss

INTRODUCTION

VERTIGO

Neuroanatomy

Diagnosis

Focused History

Focused Examination

Diagnostic Testing

Treatment

TINNITUS

Neuroanatomy

Diagnosis

Examination and Diagnostic Testing

Treatment

HEARING LOSS

Diagnosis

Sudden Hearing Loss

Examination

Treatment

6 Syncope

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Causes of Syncope

Neurally Mediated Reflex Syncope

Cardiac Syncope

Syncope Due to Orthostatic Hypotension

DIAGNOSIS

Differentiating Syncope From Seizure

Clinical Features of Syncope

Clinical Features of Seizures

Clinical Features of Other Spells Potentially Mistaken for Syncope

Focused Physical Examination

Diagnostic Tests

MANAGEMENT

OUTCOME

7 Seizures

INTRODUCTION

MANAGEMENT STRATEGY

INITIAL DIAGNOSIS

History

Exam

Differential Diagnosis

Causes of Seizures: Provoked Seizures, Unprovoked Seizures, and Lowered Seizure Threshold

EMERGENT MANAGEMENT

Prehospital Stabilization and Treatment

Hospital Stabilization and Treatment

Initial Workup

Indications for Hospital Admission

STATUS EPILEPTICUS

Second-Line Therapy

Refractory Status Epilepticus and Anesthetic (Third-Line) Treatment

ELECTROENCEPHALOGRAM MONITORING DURING STATUS EPILEPTICUS

LEVEL 1 EVIDENCE

8 Pain, Numbness, and Paresthesias

INTRODUCTION

MANAGEMENT STRATEGY

Types of Sensory Symptoms

Neuropathic Pain

NEUROANATOMY AND LOCALIZATION

Central Sensory Syndromes

Cortex

Brain Stem

Spinal Cord

Peripheral Sensory Syndromes

Nerve Roots and Dorsal Root Ganglia

Plexuses

Peripheral Nerves

FOCUSED HISTORY

Numbness and Paresthesias

Pain

Past Medical History

FOCUSED EXAM

General Medical Examination

Neurologic Examination

Mental Status

Cranial Nerves

Motor Examination

Sensory Examination

Reflexes

Gait and Station

DIAGNOSTIC EVALUATION

Serologic Tests

Neuroimaging

Lumbar Puncture

Electrodiagnostic and Quantitative Sensory Testing

Skin and Nerve Biopsy

INITIAL MANAGEMENT

Identify Potentially Serious Conditions

Symptomatic Treatment

9 Headache and Facial Pain

INTRODUCTION

GENERAL PRINCIPLES

DIAGNOSIS

History

Physical Examination

Primary Versus Secondary Headache

“Red Flags”

“Green Flags”

PRIMARY HEADACHE DISORDERS

SECONDARY CAUSES OF HEADACHE

Meningitis

Subarachnoid Hemorrhage and Intracranial Aneurysms

Brain Tumor

Subdural Hematoma

Cervical Artery Dissection

Cerebral Venous Sinus Thrombosis

Reversible Cerebral Vasoconstriction Syndrome

Giant Cell Arteritis

Spontaneous Intracranial Hypotension

Idiopathic Intracranial Hypertension

EMERGENCY DEPARTMENT EVALUATION AND MANAGEMENT

Diagnostic Evaluation

Migraine Treatment in the Emergency Department

Goals of Treatment

Parenteral Therapies

Inpatient Admission

Opioids

Treatment of Other Primary Headache Disorders

CRANIOFACIAL PAIN

Neuralgic Pain

Triggers

Dental Pain

Atypical Facial Pain

FACIAL PAIN SYNDROMES AND THE CRANIAL NEURALGIAS

LEVEL 1 EVIDENCE

10 Visual Disturbances

INTRODUCTION

NEUROANATOMY

Afferent Visual Pathway

Efferent Visual Pathway

Pupil and Eyelid Anatomy

VISUAL SYMPTOM HISTORY

Defining Severity of Symptoms

Defining Type of Symptoms

Defining How the Eyes Work Together

Remainder of History

VISUAL PATHWAY EXAMINATION

Central Vision

Visual Acuity

Color

Visual Distortion

Peripheral Vision

Confrontation Visual Field Testing

Comparative Visual Field Testing

Ophthalmic Examination

External Exam

Anterior Exam

Funduscopic Exam

Ocular Motility

Examine How the Eyes Move Individually and Together

Determine Eye Alignment

Characterize Extra Eye Movements

Pupil Size and Function

Comparing Pupils to Each Other

Comparing Optic Nerves to Each Other Using the Pupil

Neurologic Exam

ANCILLARY OPHTHALMIC TESTING

Fundus Photography

Optical Coherence Tomography

Perimetry

Visual Electrophysiology

TRANSIENT VISUAL SYMPTOMS

Transient Ischemic Attack

Ophthalmic Causes of Transient Visual Symptoms

Optic Nerve Causes of Transient Visual Symptoms

Cerebral Causes of Transient Visual Symptoms

Transient Diplopia

COMMON PATTERNS OF AFFERENT PATHWAY DISEASE

Ophthalmic

Optic Neuropathy

Acute Optic Neuropathies

Progressive Optic Neuropathies

Papilledema

Optic Chiasm

Optic Tract

Lateral Geniculate Nucleus

Optic Radiations

Occipital Lobe

Higher Order Visual Dysfunction

COMMON PATTERNS OF EFFERENT VISUAL PATHWAY DYSFUNCTION

Supranuclear Causes of Diplopia

Third Nerve Palsy

Fourth Nerve Palsy

Sixth Nerve Palsy

Multiple Cranial Neuropathies

Cavernous Sinus Syndrome

Neuromuscular Junction

Myopathies

AFFERENT AND EFFERENT DYSFUNCTION

Sellar Pathology

Orbital Apex Syndrome

High Intracranial Pressure

11 Delirium

INTRODUCTION

CAUSES OF DELIRIUM

EPIDEMIOLOGY

PATHOBIOLOGY

DIAGNOSIS

Focused History

Focused Exam

Initial Laboratory Workup

Arousal Level

Delirium Assessment

Diagnosis of Delirium After Neurologic Injury

Electroencephalogram Findings in Delirium

Delirium Versus Dementia: A Diagnostic Challenge

MANAGEMENT

Pharmacologic Prevention

Pharmacologic Therapy

Environmental Optimization

CONCLUSION

LEVEL 1 EVIDENCE

12 Dementia and Memory Loss

INTRODUCTION

Terminology and Classification

Dementia Versus Mild Cognitive Impairment

Dementia Versus Delirium

Dementia Versus Depression

EPIDEMIOLOGY

PATHOBIOLOGY

Neurodegenerative Dementias

Alzheimer Disease

Lewy Body Dementia

Frontotemporal Dementias

Less Common Neurodegenerative Dementias

Vascular Dementia

Structural Causes of Dementia

Metabolic Causes of Dementia

Infectious Causes of Dementia

Dementia From Endocrine Dysfunction

Inflammatory Causes of Dementia

DIAGNOSIS

Focused History and Differential Diagnosis

Questions to Ask

Focused Examination

Mental Status Examination

Bedside Standardized Cognitive Testing

General Neurologic Examination

Diagnostic Testing

Neuropsychological Testing

Laboratory Blood Testing

Laboratory Cerebrospinal Fluid Testing

Neurophysiologic Testing

Neuroimaging

TREATMENT

OUTCOME

LEVEL 1 EVIDENCE

13 Involuntary Movements

INTRODUCTION

FOCUSED HISTORY

FOCUSED EXAMINATION

DESCRIPTION OF INVOLUNTARY MOVEMENTS

TYPES OF HYPERKINETIC PHENOMENOLOGIES

Myoclonus

Dystonia

Chorea, Athetosis, and Ballism

Tics

Stereotypies

Tremor

Akathisia

Other Hyperkinetic Movements With Variable Phenomenologic Features

Dyskinesias

Asynergia and Ataxia

Hyperekplexia

Functional Involuntary Movements

NEUROANATOMY

DIAGNOSIS

TREATMENT

14 Muscle Weakness, Cramps, and Stiffness

INTRODUCTION

MANAGEMENT STRATEGY

NEUROANATOMY AND LOCALIZATION OF WEAKNESS

Central (Upper Motor Neuron) Weakness

Cortical Lesions

Subcortical Lesions

Spinal Cord Lesions

Peripheral (Lower Motor Neuron) Weakness

Brachial Plexus

Lumbosacral Plexus

Peripheral Nerves

Neuromuscular Junction

Myopathy

FOCUSED HISTORY

Demographics and Past Medical History

Tempo of Weakness

Variability of Weakness

Distribution of Weakness

Sensory Symptoms

Pain

Stiffness and Cramps

Visual and Speech Disturbances

Respiratory Symptoms

Autonomic Disturbances

FOCUSED EXAM

Mental Status

Cranial Nerves

Motor Examination

Inspection

Adventitious Movements

Tone

Power

Sensory Examination

Reflexes

Coordination

Gait

DIAGNOSIS AND INITIAL TREATMENT

Recognition of Emergent Situations

Laboratory Testing

Imaging Studies

Lumbar Puncture

Electrodiagnostic Testing

Muscle or Nerve Biopsy

ACKNOWLEDGMENT

15 Gait Disorders

INTRODUCTION

INTRODUCTION TO THE GAIT CYCLE

NEURAL CONTROL OF GAIT

Activation and Visual Guidance of Gait

Regulation of Gait

Execution of Gait

EPIDEMIOLOGY OF GAIT DISORDERS

FALLS IN THE ELDERLY

CLINICAL ASSESSMENT OF STANCE, GAIT, AND FALLS

History

Medications

Physical Examination

Balance and Gait Assessment

Upper Extremities

Lower Extremities

Assessment of Gait

CLASSIFICATION OF GAIT DISORDERS

16 Acute Stroke: The First Hour

INTRODUCTION

PHASE 1: INITIAL MANAGEMENT AND IMAGING

Initial Survey and Vital Signs Prior to Computed Tomography

Level of Consciousness

Blood Pressure

Heart Rate

Respirations and Breathing

Temperature

Initial History

Establish Intravenous Access and Draw Admission Labs

National Institutes of Health Stroke Scale Examination

Perform Head Computed Tomography Imaging

Analysis for Hemorrhage

Analysis for Ischemic Stroke

PHASE 2: POSTCOMPUTED TOMOGRAPHY EMERGENCY MANAGEMENT

Intracerebral Hemorrhage

Diagnose and Reverse Coagulopathy and Platelet Dysfunction

Control Severe Hypertension

Treat Elevated Intracranial Pressure or Symptomatic Mass Effect

Consider External Ventricular Drain Placement or Placement of an Intracranial Pressure Monitor

Consider Emergent Surgical Hematoma Evacuation

Consider Anticonvulsant Therapy

Consider Advanced Imaging

Disposition

Acute Ischemic Stroke

Indications for Intravenous Tissue Plasminogen Activator

Contraindications to Tissue Plasminogen Activator Administration

Tissue Plasminogen Activator and Stroke Mimics

Dosage of Intravenous Tissue Plasminogen Activator

Post–Tissue Plasminogen Activator Blood Pressure Management

Aspirin

Role of Endovascular Therapy

Disposition

LEVEL 1 EVIDENCE

17 Acute Spinal Cord Syndromes

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Spinal Cord Anatomy

Motor System

Sensory System

Autonomic System

Vascular Anatomy

Syndromic Pathology

Inflammation

Ischemia

Infection

Compression

DIAGNOSIS

Focused History

Etiology

Localization

Focused Exam

General Exam

Neurologic Exam

Imaging

Laboratory Evaluation

MANAGEMENT

Initial Stabilization

Spinal Stabilization

Intensive Care Unit Admission

Pulmonary Management

Cardiovascular Management

Etiology-Specific Treatment

Inflammation

Ischemia

Infection

Compression

OUTCOME

18 Focal Mass Lesions

INTRODUCTION

EMERGENT MANAGEMENT OF SYMPTOMATIC INTRACRANIAL MASS EFFECT

CAUSES OF FOCAL BRAIN LESIONS

Brain Tumors

Cerebritis and Abscesses

Other Inflammatory Lesions

FOCUSED HISTORY

Demographics

Time Course

Past Medical History

Social History

Review of Systems

FOCUSED EXAM

DIAGNOSIS

Imaging

Lumbar Puncture and Biopsy

INITIAL MANAGEMENT

LEVEL 1 EVIDENCE

19 Stupor and Coma

INTRODUCTION

DEFINITIONS

NEUROANATOMY

MANAGEMENT STRATEGY

Initial Resuscitation

History and General Physical Examination

Focused Neurologic Examination

Motor Responses

Respiration

Pupils

Eyelids and Eye Movements

DIAGNOSTIC TESTS

COMA PRESENTATIONS AND TREATMENT

Coma From Supratentorial Herniation Syndromes

Coma From Infratentorial Structural Lesions

Coma From Metabolic or Diffuse Brain Disease

HYSTERIA AND CATATONIA

LOCKED-IN SYNDROME

VEGETATIVE STATE AND MINIMALLY CONSCIOUS STATE

BRAIN DEATH

FUTURE DIRECTIONS

LEVEL 1 EVIDENCE

20 Brain Death

INTRODUCTION

THE CLINICAL DIAGNOSIS OF BRAIN DEATH

Determine the Cause of Brain Death

Imaging Confirmation of Brain Death

Clinical Examination

Cranial Nerves

Motor Responses to Pain

Apnea Testing

Ancillary Tests to Confirm the Diagnosis of Brain Death

BRAIN DEATH IN CHILDREN

COMMON DIAGNOSTIC CHALLENGES AND PITFALLS

INTENSIVE CARE UNIT MANAGEMENT OF THE POTENTIAL ORGAN DONOR

ETHICAL ISSUES

CONCLUSIONS

SECTION IV DIAGNOSTIC TESTS

21 Computed Tomography

INTRODUCTION

Description

Windows and Levels

ROLE OF COMPUTED TOMOGRAPHY

Head Trauma

Headache

Subarachnoid Hemorrhage

Stroke

COMPUTED TOMOGRAPHY PERFUSION AND ANGIOGRAPHY

IODINATED CONTRAST ADMINISTRATION

22 Magnetic Resonance Imaging

INTRODUCTION

PHYSICS OF MAGNETIC RESONANCE IMAGING

Image Creation

CLINICAL MAGNETIC RESONANCE IMAGING SYSTEMS

MAGNETIC RESONANCE IMAGING SAFETY

BASIC SEQUENCES

T1-Weighted and T2-Weighted Imaging

Quantification of T1 and T2 Times

Diffusion-Weighted Imaging

Gradient-Recalled Echo and Susceptibility-Weighted Imaging

Gadolinium Contrast Enhancement

Magnetic Resonance Angiography and Magnetic Resonance Venography

MAGNETIC RESONANCE IMAGING ARTIFACTS

CLINICAL MAGNETIC RESONANCE IMAGING

Cerebrovascular Disease

White Matter Disease

Brain and Spinal Cord Tumors

Transverse Myelitis

ADVANCED MAGNETIC RESONANCE IMAGING METHODS

Functional Magnetic Resonance Imaging

Diffusion Tensor Imaging

Magnetic Resonance Spectroscopy

Magnetic Resonance Perfusion Imaging

EMERGING MAGNETIC RESONANCE IMAGING METHODS

23 Positron Emission Tomography and Single-Photon Emission Computed Tomography

INTRODUCTION

BASIC PRINCIPLES

RADIOPHARMACEUTICALS AND SELECTION OF RADIONUCLIDES

DETECTION, ACQUISITION, AND DISPLAY OF SIGNAL

CLINICAL APPLICATIONS

Glucose Imaging

Positron Emission Tomography Using [18F]Fluorodeoxyglucose in Epilepsy

Positron Emission Tomography Using [18F]Fluorodeoxyglucose in Dementia

Dopamine Imaging

Perfusion Imaging

Amyloid Imaging

Tau Imaging

RADIOPHARMACEUTICALS CURRENTLY IN DEVELOPMENT WITH POTENTIAL FOR CLINICAL USE

Synaptic Density Imaging

24 Neurovascular Ultrasound

INTRODUCTION

EXTRACRANIAL ULTRASOUND (DUPLEX ULTRASOUND OR COLOR DOPPLER)

Carotid Ultrasound Criteria for Detection of Carotid Stenosis

Assessment of Carotid Artery After Carotid Endarterectomy and Stenting

Sensitivity and Specificity of Duplex Ultrasound in Detecting Carotid Stenosis

Carotid Ultrasound of Carotid Plaque Size, Morphology, and Carotid Intima-Media Thickness

Duplex Ultrasound of the Vertebral Arteries

Multiparametric Extracranial Ultrasound

Indications for Extracranial Ultrasound Examination

INTRACRANIAL ULTRASOUND (TRANSCRANIAL DOPPLER)

Indications for Transcranial Doppler Ultrasound

Sickle Cell Disease

Intracranial Stenosis and Occlusion

Sensitivity and Specificity of Transcranial Doppler in Detecting Intracranial Stenosis

Detection of Arteriovenous Malformations

Vasospasm in Subarachnoid Hemorrhage

Cerebral Microemboli Detection

Cerebral Vasomotor Reactivity Testing

Sonothrombolysis

Limitations of Transcranial Doppler

SUMMARY

LEVEL 1 EVIDENCE

25 Angiography and Neuroendovascular Surgery

INTRODUCTION

HISTORY

PROCEDURAL OUTLINE AND CONSIDERATIONS

Preprocedural Considerations

Procedure

Postprocedural Care

COMPLICATIONS OF ANGIOGRAPHY

Access Site Complications

Contrast Allergy

Contrast-Induced Nephropathy

Neurologic Complications

SELECTED CONDITIONS DIAGNOSED AND TREATED WITH NEUROINTERVENTIONAL PROCEDURES

Cerebral Aneurysms

Unruptured Aneurysms

Surgical Versus Endovascular Treatment

Overview of Endovascular Treatment Strategies for Aneurysms

Subarachnoid Hemorrhage

Acute Ischemic Stroke

Extracranial Carotid Artery Disease

Arteriovenous Malformations

Dural Arteriovenous Fistulas

Other Diagnostic Uses

EMERGING CONSIDERATIONS

Chronic Subdural Hematoma

Idiopathic Intracranial Hypertension

Intracranial Stenting

Tumors

SUMMARY

LEVEL 1 EVIDENCE

26 Electroencephalography and Evoked Potentials

ELECTROENCEPHALOGRAPHY

Introduction

Normal Electroencephalography

Abnormal Electroencephalography

Epilepsy

Focal Brain Lesion or Dysfunction

Diffuse Brain Dysfunction or Injury

Long-term Monitoring

Epilepsy Monitoring Unit

Intraoperative Neuromonitoring

Critical Care Electroencephalography Monitoring

EVOKED POTENTIALS

Introduction

Somatosensory-Evoked Potentials

Visual-Evoked Potentials

Brainstem Auditory–Evoked Responses

Motor-Evoked Potentials

MAGNETOENCEPHALOGRAPHY

LEVEL 1 EVIDENCE

27 Electromyography, Nerve Conduction Studies, and Magnetic Stimulation

INTRODUCTION

NERVE CONDUCTION STUDIES

Sensory and Motor Nerve Conduction Studies

Late Responses

Other Specialized Reflex Tests

NEEDLE ELECTROMYOGRAPHY

Basic Concepts

Insertional and Spontaneous Activity

Motor Unit Configuration

Recruitment and Interference Patterns

NERVE CONDUCTION STUDIES AND ELECTROMYOGRAPHY APPLICATIONS

TESTS OF NEUROMUSCULAR TRANSMISSION

Repetitive Nerve Stimulation

Postsynaptic Neuromuscular Junction Dysfunction

Presynaptic Neuromuscular Junction Dysfunction

Single-Fiber Electromyography

TRANSCRANIAL MAGNETIC STIMULATION

Single-Pulse Transcranial Magnetic Stimulation

Silent Period

Repetitive Transcranial Magnetic Stimulation

ACKNOWLEDGMENTS

28 Autonomic Testing

INTRODUCTION

SELECTED TESTS

Heart Rate Variability

Sympathetic Measures

Standing and the 30:15 Ratio

Tilt Table Studies

Tests of Sudomotor Function

Other Organ/System-Specific Tests

USE IN CLINICAL PRACTICE

29 Evaluation of the Special Senses (Vision, Hearing, Smell, Vestibular System)

INTRODUCTION

VISION TESTING

Visual Acuity

Color Vision

Visual-Evoked Potentials

Electroretinogram

Visual Fields

Optical Coherence Tomography

Adaptive Optics

HEARING TESTING

Audiogram

Brainstem Auditory-Evoked Potentials

Smell and Taste Testing

VESTIBULAR SYSTEM

Electronystagmography/Videonystagmography

Rotary Chair

VIDEO HEAD IMPULSE TESTING

Vestibular-Evoked Myogenic Potentials

Computerized Dynamic Posturography

Gait Analysis

30 Sleep Studies

INTRODUCTION

INDICATIONS FOR DIAGNOSTIC SLEEP TESTING

Polysomnography

Evoking Parasomnias

Evaluating Rapid Eye Movement Behavior Disorder

Home Sleep Apnea Test

Multiple Sleep Latency Test

Maintenance of Wakefulness Test

EVALUATION OF CIRCADIAN RHYTHM DISTURBANCES

Actigraphy

Biomarkers of Circadian Rhythm

SUBJECTIVE REPORTING OF SLEEP

INDICATIONS FOR LABORATORY TESTING

Narcolepsy

Restless Legs Syndrome

IMAGING

31 Neuropsychological Evaluation

STRATEGY OF NEUROPSYCHOLOGICAL TESTING

TEST SELECTION

TESTS USED IN A NEUROPSYCHOLOGICAL EVALUATION

Intellectual Ability

Memory

Processing Speed

Perception

Construction

Language

Executive Function

Working Memory

Inhibition

Set Shifting

Motor and Praxis

Attention

Concept Formation and Reasoning

Personality and Emotional Status

CLINICAL OBSERVATION

MENTAL STATUS TESTING

DIAGNOSIS-SPECIFIC ISSUES

Dementia

Other Brain Disease

Epilepsy

Deep Brain Stimulation

Toxic Exposure

Medication

Psychiatric Disorders

Learning Disability

EXPECTATIONS FROM A NEUROPSYCHOLOGICAL EVALUATION

HOW TO REFER

32 Lumbar Puncture and Cerebrospinal Fluid Analysis

INTRODUCTION

LUMBAR PUNCTURE

Techniques

Ultrasound Guidance

CEREBROSPINAL FLUID ANALYSIS

Cerebrospinal Fluid Pleocytosis

Hypoglycorrhachia

Cerebrospinal Fluid Findings in Inflammatory and Demyelinating Disorders

Cerebrospinal Fluid Cytology

Precaution for Patients With Hemicraniectomy

33 Brain, Muscle, Nerve, and Skin Biopsy

INTRODUCTION

BRAIN BIOPSY

Indications

Brain Biopsy Requires an Interdisciplinary Approach

Neuropathologic Interpretation of the Brain Biopsy

MUSCLE BIOPSY

NERVE BIOPSY

SKIN BIOPSY

34 Intracranial Pressure and Neurocritical Care Monitoring

INTRODUCTION

INTRACRANIAL PRESSURE AND CEREBRAL PERFUSION PRESSURE MONITORING

Physiologic Principles

Intracranial Pressure

Waveforms

Cerebral Perfusion Pressure and Pressure Reactivity

Pressure Reactivity Indices

External Ventricular Drainage

Intraparenchymal Intracranial Pressure Monitors

Indications for Intracranial Pressure Monitoring

BRAIN OXYGENATION MONITORING

Physiologic Principles

Jugular Venous Oxygen Saturation Monitoring

Brain Tissue Oxygen Pressure Monitoring

Near-Infrared Spectroscopy

CEREBRAL BLOOD FLOW MONITORING

Physiologic Principles

Continuous Cerebral Blood Flow Monitoring

Thermal Diffusion Flowmetry

CEREBRAL MICRODIALYSIS

Physiologic Principles

Microdialysis Monitoring

Clinical Use

CONTINUOUS ELECTROENCEPHALOGRAPHY

Seizure Detection

Titration of Therapy for Status Epilepticus

Monitoring for Delayed Cerebral Ischemia After Subarachnoid Hemorrhage

Coma Outcome

Limitations of Continuous Electroencephalography

MULTIMODALITY MONITORING DATA ACQUISITION AND ANALYSIS

35 Genetic Testing and DNA Diagnosis

INTRODUCTION

THE PROCESS OF GENETIC COUNSELING

Obtaining the Family History

The Genetic Counseling Discussion

GENETIC PHENOMENOLOGY

Incomplete and Age-Dependent Penetrance

Phenotypic Variability

Polynucleotide Repeat Expansion and Anticipation

Genetic Heterogeneity

Pleiotropy

Phenocopies

TYPES OF GENETIC TESTS

Microarray

Testing for Polynucleotide Repeat Disorders

Sanger Sequencing

Next-Generation Sequencing (Next-Gen)

TESTING STRATEGY

SECTION V CEREBROVASCULAR DISEASES

36 Acute Ischemic Stroke

INTRODUCTION

Epidemiology

Pathobiology

CEREBRAL BLOOD FLOW REDUCTION

Infarct Core and Penumbra

Completed Infarction

ETIOLOGY OF ISCHEMIC STROKE

Large Artery Atherosclerosis

Cardioembolic Stroke

Small-Vessel Infarction

Undetermined Cause of Stroke

Other Causes of Stroke

CLINICAL MANIFESTATIONS

Middle Cerebral Artery Infarction

Anterior Cerebral Artery Infarction

Internal Carotid Artery Infarction

Border Zone Infarction

Anterior Choroidal Artery Infarction

Posterior Cerebral Artery

Vertebrobasilar Territory Infarction

DIAGNOSIS

Symptom Recognition

Initial Hospital Evaluation

Imaging

Computed Tomography

Magnetic Resonance Imaging

Vascular Imaging

Perfusion Imaging

ACUTE TREATMENT

Intravenous Thrombolysis

Contraindications to Intravenous Thrombolysis

Complications of Thrombolysis

Hemorrhagic Conversion

Angioedema

Endovascular Therapies

Intra-arterial Thrombolysis

Mechanical Thrombectomy

Complications

CRITICAL CARE IN STROKE

Clinical Syndromes

Malignant Middle Cerebral Artery Infarction

Cerebellar Infarction

Basilar Artery Occlusion

Cervical Artery Dissection

Complications of Stroke

Neurologic Deterioration

Hypertension

Infectious Complications

Venous Thromboembolism

STROKE OUTCOMES

LEVEL 1 EVIDENCE

37 Transient Ischemic Attack

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

DIAGNOSIS

MANAGEMENT

OUTCOME

LEVEL 1 EVIDENCE

38 Hypoxic-Ischemic Encephalopathy

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY: CAUSES OF HYPOXIC-ISCHEMIC ENCEPHALOPATHY

Cardiac Arrest

Prolonged Hypotension

Hypoxia

Carbon Monoxide Poisoning

Cyanide Poisoning

Profound Hypoglycemia

CLINICAL FEATURES

DIAGNOSIS

Imaging

Somatosensory-Evoked Potentials

Electroencephalography

TREATMENT

General Concepts

Seizures

Therapeutic Hypothermia

OUTCOME

LEVEL 1 EVIDENCE

39 Intracerebral Hemorrhage

INTRODUCTION

EPIDEMIOLOGY

RISK FACTORS

PATHOBIOLOGY

CLINICAL MANIFESTATIONS

DIAGNOSIS

MEDICAL MANAGEMENT

Hemostasis and Emergency Reversal of Anticoagulation

Hemostatic Therapy for Noncoagulopathic Intracerebral Hemorrhage

Reversal Agents for Anticoagulation-Related Intracerebral Hemorrhage

Blood Pressure Control

Blood Pressure Targets

Antihypertensive Agents

Cerebral Edema and Intracranial Pressure

Seizures

Medical Support

SURGICAL MANAGEMENT

Ventricular Drainage

Craniotomy

Minimally Invasive Surgery

SECONDARY PREVENTION

PROGNOSIS

LEVEL 1 EVIDENCE

40 Subarachnoid Hemorrhage

INTRODUCTION

PATHOLOGY AND EPIDEMIOLOGY OF INTRACRANIAL ANEURYSMS

CLINICAL FEATURES

DIAGNOSIS

Computed Tomography

Lumbar Puncture

Angiography

Laboratory Testing

COMPLICATIONS OF ANEURYSMAL SUBARACHNOID HEMORRHAGE

Rebleeding

Vasospasm

Hydrocephalus

Global Cerebral Edema and Early Brain Injury

Seizures

Fluid and Electrolyte Disturbances

Neurogenic Cardiac and Pulmonary Disturbances

Fever

Anemia

TREATMENT

Emergency Management

Surgical Management

Endovascular Therapy

Intensive Care Management

OUTCOME

OTHER KINDS OF MACROSCOPIC ANEURYSMS

Fusiform or Dolichoectatic Aneurysms

Mycotic Aneurysm

Pseudoaneurysm

Vascular (Arteriovenous) Malformations

LEVEL 1 EVIDENCE

41 Cerebral Venous and Sinus Thrombosis

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

CLINICAL FEATURES

DIAGNOSIS

MANAGEMENT

Anticoagulation Therapy

Endovascular Therapy

Treatment of Comorbid Conditions

Treatment of Cerebral Venous Thrombosis in Children

OUTCOME

LEVEL 1 EVIDENCE

42 Vascular Malformations

INTRODUCTION

ARTERIOVENOUS VASCULAR MALFORMATIONS

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Treatment

Unruptured Arteriovenous Malformations

Ruptured Arteriovenous Malformations

Outcome

DURAL ARTERIOVENOUS FISTULAS

Pathobiology

Clinical Features

Diagnosis

Management and Outcome

CAVERNOUS MALFORMATIONS

Pathobiology

Diagnosis

Clinical Features

Management

VENOUS MALFORMATIONS

TELANGIECTASIAS

Sturge-Weber Disease

Sinus Pericranii

VASCULAR TUMORS

Hemangioblastomas

Angioblastic Meningioma and Hemangiopericytoma

LEVEL 1 EVIDENCE

43 Central Nervous System Vasculitis

INTRODUCTION

PRIMARY ANGIITIS OF THE CENTRAL NERVOUS SYSTEM

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Laboratory Findings

Imaging

Management

Outcome

AMYLOID –RELATED ANGIITIS

CENTRAL NERVOUS SYSTEM VASCULITIS ASSOCIATED WITH SYSTEMIC VASCULITIS

Large-Vessel Vasculitis

Giant Cell (Temporal) Arteritis

Takayasu Vasculitis

Medium-Vessel Vasculitis

Polyarteritis Nodosa

Kawasaki Disease

Small-Vessel Vasculitis

The Antineutrophil Cytoplasmic Antibody–Positive Vasculitides

Immunoglobulin A Vasculitis Henoch-Schönlein Purpura

Cryoglobulinemic Vasculitis

Variable-Vessel Vasculitis

Behçet Syndrome

Cogan Syndrome

Vasculitis Associated With Systemic Disease

MISCELLANEOUS CAUSES AND MIMICS OF CENTRAL NERVOUS SYSTEM VASCULITIS

Reversible Cerebral Vasoconstriction Syndrome

Intravascular Large Cell Lymphoma

Epidemiology

Pathobiology

Clinical Manifestations

Diagnosis

Treatment and Outcome

Infectious Central Nervous System Vasculitis

Drug-Induced Central Nervous System Vasculitis

Fibromuscular Dysplasia

44 Posterior Reversible Encephalopathy Syndrome and Other Cerebrovascular Syndromes

POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME

Introduction

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

REVERSIBLE CEREBRAL VASOCONSTRICTION SYNDROME

Introduction

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

FIBROMUSCULAR DYSPLASIA

Introduction

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

MOYAMOYA DISEASE

Introduction

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

BINSWANGER DISEASE

Introduction

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

SUSAC SYNDROME

Introduction

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

SNEDDON SYNDROME

Introduction

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

MARANTIC ENDOCARDITIS

Introduction

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

CEREBRAL AUTOSOMAL DOMINANT ARTERIOPATHY WITH SUBCORTICAL INFARCTS AND LEUKOENCEPHALOPATHY

Introduction

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

LEVEL 1 EVIDENCE

45 Primary and Secondary Stroke Prevention

INTRODUCTION

PRIMORDIAL STROKE PREVENTION

Diet

Smoking

Exercise

PRIMARY STROKE PREVENTION

Hypertension

Diabetes Mellitus

Hypercholesterolemia

Atrial Fibrillation

Carotid Stenosis

SECONDARY STROKE PREVENTION

Stroke Mechanism and Evaluation

Neuroimaging

Cardiac Evaluations

Blood Work and Other Laboratory Testing

Prevention Early After Transient Ischemic Attack and Stroke

Antiplatelet Therapy

Mechanism of Action and Dosing

Impact on Outcomes

Dual Antiplatelet Therapy

Anticoagulation

Carotid Endarterectomy, Angioplasty, and Stenting

Cardiac Abnormalities

MODIFIABLE RISK FACTOR MANAGEMENT

Hypertension

Hyperlipidemia

Diabetes Mellitus

Lifestyle Modification

HEMORRHAGIC STROKE

Intracerebral Hemorrhage

Hypertension

Cerebral Amyloid Angiopathy

Issues Related to Risk of Hemorrhage

Anticoagulation-Associated Hemorrhage

Management of Atrial Fibrillation in Patients at High Risk for Intracerebral Hemorrhage

Hemorrhage Related to Anticoagulation

Arteriovenous Malformations

Aneurysmal Subarachnoid Hemorrhage

STROKE REHABILITATION

LEVEL 1 EVIDENCE

SECTION VI NEUROTRAUMA

46 Concussion and Chronic Traumatic Encephalopathy

CONCUSSION

Introduction

Definition

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

CHRONIC TRAUMATIC ENCEPHALOPATHY

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

47 Traumatic Brain Injury

EPIDEMIOLOGY

PATHOBIOLOGY

Skull Fractures

Cerebral Concussion and Axonal-Shearing Injury

Brain Swelling and Cerebral Edema

Parenchymal Contusion and Hemorrhage

Subdural Hematoma

Epidural Hematoma

Traumatic Subarachnoid Hemorrhage

INITIAL ASSESSMENT AND STABILIZATION

Cardiopulmonary Resuscitation

Initial Neurologic Assessment and Stabilization

History

Radiography and Imaging

Secondary Neurologic Evaluation

MANAGEMENT

Admission to the Hospital

Low-risk Group

Moderate-risk Group

High-risk Group

Surgical Intervention

Intracranial Pressure Management

Intensive Care Unit Management of Severe Head Injury

Serial Neurologic Evaluation

Airway and Ventilation

Blood Pressure Management

Fluid Management

Nutrition

Analgosedation

Temperature Management

Anticonvulsants

Intensive Insulin Therapy

Steroids

Deep Vein Thrombosis Prophylaxis

Gastric Stress Ulcer Prophylaxis

Antibiotics

ACUTE COMPLICATIONS OF HEAD INJURY

Cerebrospinal Fluid Fistula

Carotid-Cavernous Fistula

Traumatic Arterial Dissection and Vascular Injuries

Cranial Nerve Injury

Infections

OUTCOMES

Postconcussion Syndrome

Seizures and Posttraumatic Epilepsy

Posttraumatic Movement Disorders

PEDIATRIC TRAUMA BRAIN INJURY

MISSILE-RELATED CEREBRAL BLAST INJURY

LEVEL 1 EVIDENCE

48 Traumatic Spinal Cord Injury

INTRODUCTION

ETIOLOGY AND EPIDEMIOLOGY

MECHANISM OF INJURY

PATHOBIOLOGY

Gross Pathology

Histopathology

Immediate Phase (Initial 1-2 H)

Acute Phase (Hours to 1-2 D)

Intermediate Phase (Days to Weeks)

Late Phase (Weeks to Months/Years)

DIAGNOSIS, NEUROLOGIC ASSESSMENT, AND CLASSIFICATION

Clinical Evaluation

Imaging Studies

Risk Stratification

Cervical X-Rays

Cervical Computed Tomography

Neurologic Assessment and Classification

Brown-Séquard Syndrome

Central Cord Syndrome

Anterior Cord Syndrome

Posterior Cord Syndrome

Conus Medullaris and Cauda Equina Syndromes

ACUTE MEDICAL MANAGEMENT

General Trauma Care

Steroids

Autonomic Dysfunction and Blood Pressure Management

Critical Care

Surgical Treatment

CHRONIC MEDICAL MANAGEMENT

Pain

Autonomic Dysreflexia

Coronary Artery Disease

Pulmonary Disease

Genitourinary Complications

Gastrointestinal Dysfunction

Abnormal Bone Metabolism

Spasticity

Psychiatric Complications

CONCLUSION

LEVEL 1 EVIDENCE

49 Traumatic Cranial and Peripheral Nerve Injury

INTRODUCTION

Anatomy and Physiology

Mechanisms of Injury

Injury Classification

Repair and Regeneration

CLINICAL FEATURES

Cranial Nerve Lesions

Cranial Nerve I: The Olfactory Nerve

Cranial Nerve II: The Optic Nerve

Cranial Nerve III, IV, VI: The Oculomotor, Trochlear, and Abducens Nerves

Cranial Nerve V: The Trigeminal Nerve

Cranial Nerve VII: The Facial Nerve

Cranial Nerve IX: The Glossopharyngeal Nerve

Cranial Nerve X: The Vagus Nerve

Cranial Nerve XI: The Spinal Accessory Nerve

Cranial Nerve XII: The Hypoglossal Nerve

UPPER EXTREMITY NERVE LESIONS

Brachial Plexus Injuries

Proximal Nerve Injuries

Median Nerve

Radial Nerve

Ulnar Nerve

Axillary Nerve

LOWER EXTREMITY NERVE LESIONS

Lumbosacral Plexus

Obturator Nerve

Iliohypogastric Nerve

Ilioinguinal Nerve

Genitofemoral Nerve

Lateral Femoral Cutaneous Nerve

Femoral Nerve

Sciatic Nerve

Common Peroneal Nerve

Tibial Nerve

DIAGNOSIS

Electrodiagnostic Findings in Peripheral Nerve Injury

Neurapraxia

Axonotmesis

Neurotmesis

Timing of Studies

Role in Surgical Planning

TREATMENT

Indications for Early Operative Management

Surgical Techniques

Neurolysis

Split Repair

End-to-End Neurorrhaphy

Graft Repair

Nerve Transfer

Tendon Transfer

OUTCOMES

SECTION VII DEMENTIA

50 Mild Cognitive Impairment

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

DIAGNOSIS

Clinical Features

Diagnostic Testing

Neuropsychological Testing

Laboratory Testing and Neuroimaging

Biomarker-Based Assessments

TREATMENT

OUTCOMES

LEVEL 1 EVIDENCE

51 Alzheimer Disease

INTRODUCTION

EPIDEMIOLOGY

Genetic Basis of Alzheimer Disease

PATHOBIOLOGY

DIAGNOSIS

Clinical Features

Diagnostic Testing

Neuropsychological Testing

Laboratory Testing and Neuroimaging

Biomarker-Based Assessments

MANAGEMENT

OUTCOME

LEVEL 1 EVIDENCE

52 Frontotemporal Dementia

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

GENETICS

CLINICAL MANIFESTATIONS

Behavioral Variant

Primary Progressive Aphasia

DIAGNOSIS

MANAGEMENT

OUTCOME

LEVEL 1 EVIDENCE

53 Lewy Body Dementias

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Neuropathology

Lewy Bodies

Lewy Neurites

Alzheimer Pathology

Spongiform Change/Microvacuolation

Distinction Between Dementia With Lewy Bodies and Parkinson Disease Dementia

Neurotransmitter Systems

Genetic Risk Factors

APOE

SNCA

GBA1

CLINICAL MANIFESTATIONS

Cognitive Impairment

Psychiatric Symptoms

Parkinsonism

Sleep Disorders

Autonomic Dysfunction

Fluctuating Attention and Concentration

DIAGNOSIS

Prodromal Dementia With Lewy Bodies

Diagnostic Testing and Evaluation

Neuropsychological Testing

Neuroimaging

Autonomic Testing

Differential Diagnosis

TREATMENT

Cognitive Symptoms

Motor Symptoms

Psychosis

Other Features

Mood Disorders

Sleep

Dysautonomia

Lifestyle

Future Directions

OUTCOME

LEVEL 1 EVIDENCE

54 Vascular Dementia and Cognitive Impairment

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

DIAGNOSIS

MANAGEMENT

Prevention and Treatment of Stroke

Cognitive and Behavioral Symptoms

OUTCOME

LEVEL 1 EVIDENCE

55 Prion Diseases

INTRODUCTION

History and Nomenclature

PATHOBIOLOGY

Prion Protein Properties

Prion Protein Functions

Prion Protein Pathogenicity

SPORADIC CREUTZFELDT-JAKOB DISEASE

Epidemiology

Clinical Presentation

Molecular Classification

Diagnosis

GENETIC PRION DISEASE

Familial Creutzfeldt-Jakob Disease

Gerstmann-Sträussler-Scheinker Disease

Fatal Familial Insomnia

Octapeptide Repeat Insertions

ACQUIRED PRION DISEASE

Variant Creutzfeldt-Jakob Disease

Iatrogenic Creutzfeldt-Jakob Disease

MANAGEMENT AND OUTCOMES

Symptomatic Treatments

Investigational Treatments

LEVEL 1 EVIDENCE

SECTION VIII HEADACHE AND PAIN SYNDROMES

56 Primary and Secondary Headache Disorders

ANATOMY AND PHYSIOLOGY OF HEADACHE

CLINICAL EVALUATION OF HEADACHE DISORDERS

SECONDARY HEADACHE

PRIMARY HEADACHE DISORDERS

MIGRAINE

Epidemiology

Pathophysiology

Diagnosis and Clinical Features

Treatment of Migraine

Trigger and Lifestyle Approaches to Management

Acute Therapies

Preventive Treatments for Migraine

TENSION-TYPE HEADACHE

Clinical Features

Pathophysiology

Treatment

TRIGEMINAL AUTONOMIC CEPHALALGIAS

Cluster Headache

Clinical Features

Pathophysiology

Treatment

Paroxysmal Hemicrania

SUNCT/SUNA

Clinical Features

Treatment

Hemicrania Continua

OTHER PRIMARY HEADACHES

Primary Cough Headache

Primary Exertional Headache

Primary Headache Associated With Sexual Activity

Cold-Stimulus Headache

External Pressure Headache

Primary Stabbing Headache

Nummular Headache

Hypnic Headache

New Daily Persistent Headache

Posttraumatic Headache

LEVEL 1 EVIDENCE

57 Facial Pain Disorders and Painful Cranial Neuralgias

INTRODUCTION

FACIAL PAIN DUE TO DISORDERS OF SINUSES, NOSE, AND EARS

Rhinosinusitis

Epidemiology and Pathobiology

Diagnosis

Management and Outcome

Facial Pain Attributed to Disorders of Intranasal Mucosal Contact Points

Epidemiology and Pathobiology

Diagnosis

Management and Outcome

Otalgia Attributed to Disorder of Ears and Referred Pain

Epidemiology and Pathobiology

Diagnosis

FACIAL PAIN DUE TO TEMPOROMANDIBULAR DISORDERS AND DISORDERS OF THE TEETH OR MOUTH

Temporomandibular Disorders

Epidemiology and Pathobiology

Clinical Manifestations and Diagnosis

Treatment and Outcome

CRANIAL NEURALGIAS

Trigeminal Neuralgia

Epidemiology and Pathobiology

Clinical Manifestations and Diagnosis

Treatment and Outcome

Glossopharyngeal Neuralgia

Nervus Intermedius Neuralgia

Occipital Neuralgia

Epidemiology and Pathobiology

Clinical Manifestations and Diagnosis

Treatment and Outcome

IDIOPATHIC FACIAL PAIN

Burning Mouth Syndrome

Persistent Idiopathic Facial Pain

Primary Headache Disorders Presenting as Facial Pain

LEVEL 1 EVIDENCE

58 Complex Regional Pain Syndrome

INTRODUCTION

EPIDEMIOLOGY

CLINICAL FEATURES AND PATHOLOGY

PATHOBIOLOGY

DIAGNOSTIC CRITERIA

Clinical Diagnostic Criteria

Research Diagnostic Criteria

MANAGEMENT

Physical Therapies

Nonopioid Pharmacotherapy

Opioid Treatment

Topical Creams

Continuous Infusion Therapy

Intravenous Therapies and Intravenous Regional Blockade

Sympathetic Blocks and Sympathectomy

Neuromodulation

Pain Prevention

FUTURE RESEARCH

CONCLUSIONS

59 Neuropathic Pain

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Normal Processing of Nociceptive Pain

Transduction and Transmission

Modulation and Perception

Mechanisms Underlying Neuropathic Pain

Peripheral Sensitization

Central Sensitization

DIAGNOSIS

MANAGEMENT

General Principles

Pharmacologic Treatments

Gabapentinoids

Serotonin-Norepinephrine Reuptake Inhibitors and Tricyclic Antidepressants

Sodium Channel Antagonists

Antiepileptics

Capsaicin

Opioids and Other Controlled Substances

Other Pharmacologic Agents

Cognitive Behavioral and Physical Therapy

Procedural Interventions

OUTCOME

LEVEL 1 EVIDENCE

SECTION IX EPILEPSY AND PAROXYSMAL DISORDERS

60 Classification of Seizures and Epilepsy

INTRODUCTION

EPIDEMIOLOGY

Gene Mutations in Epilepsy

CLASSIFICATIONS OF SEIZURES AND EPILEPSY

Classification of Seizures

Focal Seizures

Generalized Seizures

Unknown Onset Seizures

Classification of Epilepsies

Generalized Epilepsy

Focal Epilepsy

Epilepsy Syndromes

Idiopathic Generalized Epilepsy Syndromes

Selected Focal Epilepsy Syndromes

Lennox-Gastaut Syndrome (A Type of Combined Focal and Generalized Epilepsy Syndrome)

Infantile Spasms (West Syndrome)

Post-traumatic Seizures

Epilepsia Partialis Continua

LEVEL 1 EVIDENCE

61 Management of Epilepsy

INTRODUCTION

Acute Symptomatic Seizures

The Single Seizure

INITIAL DIAGNOSTIC EVALUATION

History and Examination

Electroencephalography

Brain Imaging

Other Laboratory Tests

LONG-TERM MONITORING

MEDICAL TREATMENT

Antiepileptic Drugs

Selection of Antiepileptic Drugs

Adverse Effects of Antiepileptic Drugs

Dose-Related Adverse Effects

Idiosyncratic Reactions

Antiepileptic Drug Pharmacology

Specific Drugs

Use of Generic Equivalents

Discontinuing Antiepileptic Drugs

Reproductive Health Issues

Bone Health

SURGICAL TREATMENT

Resective Procedures

Anterior Temporal Lobe Resection

Lesionectomy

Nonlesional Cortical Resections

Corpus Callosotomy

Hemispherectomy

Preoperative Evaluation

Neurostimulation in Drug-Resistant Epilepsy

Vagus Nerve Stimulation

Deep Brain Stimulation

Responsive Neurostimulation

PSYCHOSOCIAL AND PSYCHIATRIC ISSUES

Compliance

Psychogenic Seizures

LEVEL 1 EVIDENCE

62 Ménière Syndrome, Benign Paroxysmal Positional Vertigo, and Vestibular Neuritis

INTRODUCTION

MéNIèRE DISEASE

Definitions

Diagnosis

Medical Treatment

Surgical Management

Conservative Procedures

Destructive Procedures Labyrinthectomy

Other Treatment Methods

Intratympanic and Oral Corticosteroids

Intratympanic Gentamicin

Meniett Device

Streptomycin

BENIGN PAROXYSMAL POSITIONAL VERTIGO

VESTIBULAR NEURITIS

CONCLUSION

LEVEL 1 EVIDENCE

63 Transient Global Amnesia

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

CLINICAL MANIFESTATIONS

DIAGNOSIS

TREATMENT

SECTION X CENTRAL NERVOUS SYSTEM INFECTIONS

64 Acute Bacterial Meningitis

INTRODUCTION

ETIOLOGY

PATHOBIOLOGY

CLINICAL MANIFESTATIONS

DIAGNOSIS

Differential Diagnosis

Cerebrospinal Fluid Analysis for the Differential Diagnosis

TREATMENT

COMPLICATIONS

LEVEL 1 EVIDENCE

65 Brain, Spinal, and Epidural Abscess and Other Parameningeal Infections

BRAIN ABSCESS

Epidemiology

Source of Infection in Brain Abscess

Causative Organisms of Brain Abscess

Pathobiology

Clinical Manifestations

Diagnosis

Management

Outcome

SUBDURAL EMPYEMA

Epidemiology

Pathobiology

Clinical Manifestations

Diagnosis

Management

Outcome

SPINAL EPIDURAL ABSCESS

Epidemiology and Pathobiology

Clinical Manifestations

Diagnostic Testing

Management

Outcome

MALIGNANT OTITIS EXTERNA

Epidemiology and Pathobiology

Clinical Manifestations

Diagnosis

Management and Outcome

OSTEOMYELITIS OF THE SKULL BASE

Epidemiology and Pathobiology

Clinical Manifestations

Diagnosis

Management and Outcome

66 Other Bacterial Central Nervous System Infections and Toxins

INTRODUCTION

RICKETTSIAL INFECTIONS

Spotted Fever Rickettsiosis

Epidemiology

Pathophysiology

Clinical Manifestations

Diagnosis

Treatment and Outcome

Ehrlichiosis and Anaplasmosis

Epidemiology

Pathophysiology

Clinical Manifestations

Diagnosis

Treatment and Outcome

Scrub Typhus

Clinical Manifestations

Diagnosis

Treatment and Outcome

Typhus Group Rickettsiae

Epidemiology

Clinical Manifestations

Diagnosis

Treatment and Outcome

ZOONOTIC INFECTIONS

Q Fever

Epidemiology

Clinical Manifestations

Diagnosis

Treatment and Outcome

Brucellosis

Epidemiology

Clinical Manifestations

Diagnosis

Treatment and Outcome

Leptospirosis

Epidemiology

Clinical Manifestations

Diagnosis

Treatment and Outcome

Cat-Scratch Disease

Epidemiology

Clinical Manifestations

Diagnosis

Treatment and Outcome

Anthrax

Epidemiology

Clinical Manifestations

Diagnosis

Treatment and Outcome

DISEASES OF HUMAN-TO-HUMAN TRANSMISSION

Whipple Disease

Epidemiology

Clinical Manifestations

Diagnosis

Treatment and Outcome

Mycoplasma pneumoniae

Epidemiology

Clinical Manifestations

Diagnosis

Treatment and Outcome

TOXINS

Diphtheria

Epidemiology

Clinical Manifestations

Diagnosis

Treatment and Outcome

Botulism

Pathophysiology

Epidemiology

Clinical Manifestations

Diagnosis

Treatment and Outcome

Tetanus

Pathophysiology

Epidemiology

Clinical Manifestations

Diagnosis

Treatment and Outcome

67 Chronic Meningitis

INTRODUCTION

GENERAL DIAGNOSTIC APPROACH

FUNGAL MENINGITIS

Cryptococcal Meningitis

Epidemiology and Pathobiology

Clinical Features

Diagnosis

Treatment Update

Outcomes

Coccidioidomycosis

Epidemiology and Pathobiology

Clinical Features

Diagnosis

Treatment

Outcomes

Blastomycosis and Histoplasmosis

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Treatment

Aspergillosis

Epidemiology and Pathobiology

Clinical Features

Diagnosis

Treatment

TUBERCULOSIS

Epidemiology and Pathobiology

Clinical Features

Diagnosis

Treatment

NEUROSYPHILIS

Epidemiology and Pathobiology

Clinical Features

Diagnosis

Treatment

LYME DISEASE

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Treatment

HYPOTHESIS-FREE INVESTIGATIONS

LEVEL 1 EVIDENCE

68 Parasitic Infections

INTRODUCTION

Parasitic Neurologic Diseases

CEREBRAL MALARIA

Introduction

Epidemiology

Pathobiology

Diagnosis

Clinical Features

Diagnostic Methods

Management

TRYPANOSOMIASIS

African Trypanosomiasis

American Trypanosomiasis

AMEBIC MENINGOENCEPHALITIS

NEUROCYSTICERCOSIS

Introduction

Epidemiology

Pathobiology

Diagnosis

Clinical Features

Diagnostic Methods

Management

LARVA MIGRANS AND EOSINOPHILIC MENINGITIS

Introduction

Epidemiology

Pathobiology

Diagnosis

Clinical Features

Diagnostic Methods

Management

Outcome

SCHISTOSOMIASIS (BILHARZIA)

Introduction

Epidemiology

Pathobiology

Diagnosis

Clinical Manifestations

Diagnostic Methods

Management and Outcome

LEVEL 1 EVIDENCE

69 Viral Infections

INTRODUCTION

PICORNAVIRUS

Enteroviruses

Poliomyelitis

Enterovirus D68

Enteroviruses 70 and 71

Coxsackievirus and Echoviruses

ARBOVIRUSES

Flaviviruses

Dengue

Tick-Borne Encephalitis Virus

Japanese Encephalitis Virus

West Nile Virus

Murray Valley Encephalitis Virus

St. Louis Encephalitis Virus

Powassan Virus

Bunyaviruses

Lacrosse Virus

Jamestown Canyon Virus

Togaviruses

Alphavirus (Equine Encephalitis)

Rubivirus

CORONAVIRUSES: SEVERE ACUTE RESPIRATORY SYNDROME VIRUS

Epidemiology

Neurologic Clinical Features Associated With COVID-19

Cerebrospinal Fluid Analyses and Neuroradiographic Imaging in COVID-19

Histopathologic Findings Associated With COVID-19

ORTHOMYXOVIRUSES: INFLUENZA VIRUS

PARAMYXOVIRUSES

Henipaviruses

Measles Encephalitis

Subacute Sclerosing Panencephalitis

RHABDOVIRUS: RABIES

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Treatment

ARENAVIRUSES

Lymphocytic Choriomeningitis Virus

Clinical Manifestations

Diagnosis and Treatment

Adenovirus

HERPESVIRUSES

Herpes Simplex Virus 1 and 2

Herpes Simplex Virus Encephalitis

Neonatal Herpes Simplex Virus 2 Encephalitis

Benign Recurrent Lymphocytic Meningitis

Varicella-Zoster Virus

Herpes Zoster

Epstein-Barr Virus

Cytomegalovirus

Clinical Manifestations

Diagnosis and Treatment

Human Herpesvirus 6

JOHN CUNNINGHAM VIRUS

Pathobiology

Clinical Features

Diagnosis

Treatment and Prognosis

LEVEL 1 EVIDENCE

70 Human Immunodeficiency Virus (HIV) and Acquired Immunodeficiency Syndrome (AIDS)

INTRODUCTION

Classification of HIV Disease

EPIDEMIOLOGY

Epidemiology of HIV Infection in Adults

Epidemiology of HIV Infection in Children

Etiology

PATHOBIOLOGY

Primary HIV Infection

Progression to AIDs

Impact of Antiretroviral Therapy

Central Nervous System Pathogenesis

DIAGNOSIS

Initial Diagnostic Approach

Neurological HIV-Related Syndromes

HIV Associated Neurocognitive Disorder

Stroke

Seizures

Leukoencephalopathy

Movement Disorders

Myelopathy

Motor Neuron Disease

Peripheral Neuropathy

Myopathy

Opportunistic Infections

Neoplasms

Nutritional and Metabolic Consequences

MANAGEMENT

Adverse Neurologic Effects of Drug Treatment for HIV Infection

Precautions for Clinical and Laboratory Services

OUTCOMES

SECTION XI DEMYELINATING AND INFLAMMATORY DISEASES

71 Multiple Sclerosis and Related CNS Neuroimmune Diseases

INTRODUCTION

MULTIPLE SCLEROSIS

Introduction

Epidemiology

Pathobiology

Immunobiology

Pathology

Genetic Risk Factors

Environmental Influences

Diagnosis

Disease Course

Clinical Manifestations

Diagnostic Criteria

Imaging

Biomarkers

Differential Diagnosis

Management

Treatment of Acute Exacerbations

Disease-Modifying Drugs

Symptom Management

Pregnancy and Breastfeeding

Vaccination

Pediatric Multiple Sclerosis

Outcomes

NEUROMYELITIS OPTICA SPECTRUM DISORDER

Introduction

Epidemiology

Pathobiology

Diagnosis

Clinical Manifestations

Radiologic Manifestations

Biomarkers

Management

Outcomes

ANTI–MYELIN OLIGODENDROCYTE GLYCOPROTEIN SYNDROMES

Epidemiology

Pathobiology

Diagnosis

Clinical Manifestations

Diagnostic Criteria

Biomarkers

Management

LEVEL 1 EVIDENCE

72 Transverse Myelitis

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

DIAGNOSIS

Clinical Presentation

MAGNETIC RESONANCE IMAGING

Cerebrospinal Fluid

Biomarkers of Transverse Myelitis

Other Investigations

MANAGEMENT

OUTCOME

LEVEL 1 EVIDENCE

73 Neuromyelitis Optica Spectrum Disorder

INTRODUCTION

EPIDEMIOLOGY

Worldwide Distribution/Ethnicity

Sex

Familial Occurrence

Risk Factors

PATHOBIOLOGY

AQP4-IgG– and MOG-IgG–Mediated Immunopathology

T-cell Activation, Helper T Cell 17 Polarization, B-cell Recognition, and “Upstream” Pathogenesis

DIAGNOSIS

Characteristic Symptoms and Diagnostic Criteria

Serologic Testing and Cerebrospinal Fluid

Red Flags, Comorbid Conditions, and Differential Diagnosis

Neuromyelitis Optica Spectrum Disorder Versus Multiple Sclerosis

AQP4-IgG– Versus MOG-IgG–Associated NMOSD

MANAGEMENT

Acute Attacks

Maintenance Therapy for Neuromyelitis Optica Spectrum Disorder

Maintenance for Myelin Oligodendrocyte Glycoprotein Immunoglobulin G Disease

OUTCOMES

Mortality in Neuromyelitis Optica Spectrum Disorder

Disability in Neuromyelitis Optica Spectrum Disorder

LEVEL 1 EVIDENCE

74 Autoimmune Encephalitis and Meningitis

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Immunobiology

Immunopathology

DIAGNOSIS

Clinical Manifestations

Autoimmune Meningitis

Autoimmune Encephalitis: General Features

Classical Encephalitis Syndromes

Clinically Restricted Forms

Nonlimbic Encephalitis: Serologically Defined Entities

Neural IgG Seronegative Autoimmune Encephalopathy

Imaging

MRI Brain Imaging

Positron Emission Tomography

Electroencephalography

Antibody Testing

Cancer Workup

MANAGEMENT

Acute Therapy

Chronic Therapy

PREDICTIVE SCORES

OUTCOME

75 Neurosarcoidosis

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

CLINICAL MANIFESTATIONS

Cranial Nerves

Meninges

Brain Parenchyma

Spinal Cord

DIAGNOSIS

Magnetic Resonance Imaging Findings

Cerebrospinal Fluid Findings

Peripheral Nervous System and Muscle Presentations

MANAGEMENT

OUTCOMES

SECTION XII MOVEMENT DISORDERS

76 Essential Tremor

INTRODUCTION

EPIDEMIOLOGY

ETIOLOGY

PATHOBIOLOGY

CLINICAL MANIFESTATIONS

Introduction

Tremor

Other Motor Features

Nonmotor Features

Natural History

Associated Morbidity and Mortality

DIAGNOSIS

TREATMENT

OUTCOME

LEVEL 1 EVIDENCE

77 Tics and Tourette Syndrome

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Neurobiology

Motor Circuits

Neurotransmitters

Genetics

Immune System

CLINICAL MANIFESTATIONS

DIAGNOSIS

DSM-5 Tic Disorder Diagnoses

Scales

Coexisting Conditions

Attention Deficit Hyperactivity Disorder

Obsessive-Compulsive Behaviors and Obsessive-Compulsive Disorder

Anxiety and Depression

Other Psychopathologies

Academic Issues

Sleep Disorders

MANAGEMENT

Initial Management

Behavioral Treatment for Tics

Habit Reversal Training

Comprehensive Behavioral Intervention for Tics

Exposure Response Prevention

Complementary and Alternative Medications

Pharmacotherapy

Tier 1 Medications

Tier 2 Medications

Other Medications

Neuromodulation

Noninvasive Neuromodulation

Invasive Neuromodulation

OUTCOME

CONCLUSION

LEVEL 1 EVIDENCE

78 Restless Legs Syndrome

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

DIAGNOSIS

MANAGEMENT

OUTCOME

LEVEL 1 EVIDENCE

79 The Dystonias

INTRODUCTION

Defining Features of Dystonia

Discrimination of Dystonia From Related Disorders

Additional Relevant Terminology

Classification of Dystonia Subtypes

Axis I: Clinical Manifestations

Axis II: Etiologies

EPIDEMIOLOGY

Isolated Dystonia Syndromes

Other Syndromes With Dystonia

PATHOBIOLOGY

Anatomic Underpinnings of Dystonia

Physiologic Underpinnings of Dystonia

Muscle Activity

Neuronal Physiology

Network Physiology

Developmental or Degenerative Processes

Etiologies for Dystonia

Idiopathic Dystonias

Acquired Dystonias

Inherited Dystonias

DIAGNOSIS

Approach to Clinical Evaluation

Diagnostic Imaging

Routine Laboratory Investigations

Genetic Testing

MANAGEMENT

Counseling

Oral Medications

Drugs Targeting Acetylcholine Pathways

Drugs Targeting Dopamine Pathways

Drugs Targeting γ-Aminobutyric Acid Pathways

Miscellaneous Drugs

Special Populations

Botulinum Neurotoxins

Applications

Contraindications and Side Effects

Surgical Treatments

Deep Brain Stimulation

Procedures Involving Focal Ablation

Procedures Involving Peripheral Nerve or Muscle

Physical and Occupational Therapy

OUTCOMES

ACKNOWLEDGMENTS

80 Hemifacial Spasm

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

CLINICAL FEATURES

DIAGNOSIS

MANAGEMENT

Medication

Botulinum toxin injections

Surgery

OUTCOME

Medication

Botulinum toxin injections

Surgery

LEVEL 1 EVIDENCE

81 Myoclonus

INTRODUCTION

GENERAL CONSIDERATIONS

Epidemiology

Phenomenologic Characterization of Myoclonus

CORTICAL MYOCLONUS

Pathobiology

Clinical Manifestations

Diagnosis

SUBCORTICAL INCLUDING BRAINSTEM MYOCLONUS

Pathobiology

Clinical Manifestations

Diagnosis

SPINAL MYOCLONUS

Pathobiology

Clinical Manifestations

Diagnosis

PERIPHERAL MYOCLONUS

Pathobiology

Clinical Manifestations

Diagnosis

MANAGEMENT

OUTCOME

82 Hereditary and Acquired Ataxias

INTRODUCTION

Neuroanatomy of the Cerebellum

CEREBELLAR NEUROPHYSIOLOGY

Cerebellar Circuits

Control of Movement by the Cerebellum

EPIDEMIOLOGY

CLINICAL APPROACH TO CEREBELLAR ATAXIA

Signs and Symptoms of Ataxia

Neurologic Examination

Laboratory Testing

Neuroimaging

Other Diagnostic Tests

ACQUIRED ATAXIA

Cerebellar Stroke

Paraneoplastic and Autoimmune Ataxia

Classic Paraneoplastic Cerebellar Ataxia

Cerebellar Ataxias Resulting from Ion Channel Antibodies

Ataxia Due to Other Accessible/Cell-Surface Antigens

Gluten Ataxia

Neurosarcoidosis

INHERITED ATAXIAS

Spinocerebellar Ataxia

Introduction

Genetic Testing

Autosomal Recessive Ataxia

Cerebellar Ataxia With Predominant Sensory Neuropathy (Friedreich Ataxia)

Cerebellar Ataxia with Sensorimotor Axonal Neuropathy

Cerebellar Ataxia Without Neuropathy

Other Inherited Ataxias

X-Linked Ataxia

Mitochondrial Ataxia

Episodic Ataxia

Cerebellar Ataxia, Neuropathy, and Vestibular Areflexia Syndrome

Degenerative Ataxia

Multiple System Atrophy

Idiopathic Late-Onset Cerebellar Ataxia

Psychogenic Ataxia

MANAGEMENT AND FUTURE DEVELOPMENTS

83 Tardive Dyskinesia and Other Neuroleptic-Induced Syndromes

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

DIAGNOSIS

Acute Dystonic Reaction

Acute Akathisia

Drug-Induced Parkinsonism

Neuroleptic Malignant Syndrome

Tardive Dyskinesia Syndromes

Withdrawal Emergent Syndrome

Classic Tardive Dyskinesia

Tardive Dystonia

Tardive Akathisia

Other Tardive Syndromes

MANAGEMENT

Acute Dystonic Reaction

Acute Akathisia

Drug-Induced Parkinsonism

Neuroleptic Malignant Syndrome

Tardive Dyskinesia Syndromes

OUTCOME

LEVEL 1 EVIDENCE

84 Choreas

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

CAUSES OF CHOREA

Autoimmune Choreas

Sydenham Disease

Systemic Lupus Erythematosus

Antiphospholipid Antibody Syndrome

Chorea due to Other Autoantibodies

Structural and Vascular Chorea and Ballism

Genetic Causes of Chorea

Autosomal Recessive

X Linked

Autosomal Dominant

Other Choreas

DIAGNOSIS

MANAGEMENT

OUTCOME

LEVEL 1 EVIDENCE

85 Huntington Disease

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Genetics

Neuropathology

Neurochemistry

DIAGNOSIS

Differential Diagnosis

Clinical Manifestations

Motor Signs

Cognitive Impairment

Behavioral Signs

Measures of Disease Progression

Clinical Progression Composite Measures

Structural and Molecular Imaging

Fluid Biomarkers

MANAGEMENT

Treatment of Movement Disorder

Antidopaminergic Agents for Chorea

Antiglutamatergic Therapy for Chorea

Treatment of Dystonia

Treatment of Voluntary Motor Impairment (Gait and Swallowing)

Treatment of Weight Loss

Treatment of Cognitive Impairment

Treatment of Psychiatric Symptoms

Disease-Modifying Therapies

Improving Mitochondrial Function and Reducing Oxidative Stress

Immune Modulation

mHTT Huntingtin-Lowering Strategies

Nonpharmacologic Treatments

OUTCOME

LEVEL 1 EVIDENCE

86 Parkinson Disease

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Pathology

Etiology

Genetics

Pathogenesis

CLINICAL MANIFESTATIONS

Signs and Symptoms

Tremor

Bradykinesia

Rigidity

Postural Instability

Dystonia

Speaking and Swallowing

Freezing

Parkinson Mimics

Clinical Course

Non-Motor Manifestations

DIFFERENTIAL DIAGNOSIS

OVERVIEW OF PARKINSON DISEASE TREATMENT

Levodopa

Levodopa-Induced Dyskinesias

Levodopa and Adjunctive Therapies

COMT Inhibitors

MAO-B Inhibitors

Amantadine

Anticholinergic (Antimuscarinic) Drugs

Dopaminergic Agonists

Adenosine A2A Antagonists

TREATING THE NONMOTOR PROBLEMS OF PARKINSON DISEASE

Mental and Behavioral Complications

Other Non-Motor Symptoms

SURGICAL THERAPY

OUTCOME

LEVEL 1 EVIDENCE

87 Parkinson-Plus Syndromes

INTRODUCTION

PARKINSON-PLUS SYNDROMES

Progressive Supranuclear Palsy

History

Pathobiology

Symptoms and Signs

Multiple System Atrophy

History

Pathobiology

Symptoms and Signs

Corticobasal Degeneration

History

Pathobiology

Symptoms and Signs

EPIDEMIOLOGY

DIAGNOSIS

TREATMENT

OTHER PARKINSON-LIKE SYNDROMES

Drug/Toxin-Induced Parkinsonism

Dopamine Receptor–Blocking Drugs

1-Methyl-4-Phenyl-1,2,3,6-Tetrahydropyridine

Other Drugs Besides Dopamine Receptor Blockers and 1-Methyl-4-Phenyl-1,2,3,6-Tetrahydropyridine

Lytico-Bodig (Parkinson-Dementia-Amyotrophic Lateral Sclerosis Complex of Guam)

Hemiparkinsonism-Hemiatrophy Syndrome

Normal Pressure Hydrocephalus

Parkinson-Dementia Syndromes

Postencephalitic Parkinsonism

Vascular Parkinsonism

SECTION XIII NEUROMUSCULAR DISEASES

88 Amyotrophic Lateral Sclerosis and Motor Neuron Diseases

INTRODUCTION

Amyotrophic Lateral Sclerosis

EPIDEMIOLOGY

PATHOBIOLOGY

Genetics

Neuropathology

Amyotrophic Lateral Sclerosis and Frontotemporal Dementia

CLINICAL MANIFESTATIONS

DIAGNOSIS

Amyotrophic Lateral Sclerosis Diagnostic Criteria

Motor Neuron Disease Variants

Progressive Muscular Atrophy

Primary Lateral Sclerosis

Progressive Bulbar Palsy

Monomelic Muscular Atrophy

Bibrachial Amyotrophy

Diagnostic Testing

Electrodiagnostic Testing

Neuroimaging

Lab Testing

Muscle Biopsy

Differential Diagnoses

Multifocal Motor Neuropathy

Myasthenia Gravis

Spinal Stenosis

Myopathy

Postpolio Syndrome

Benign Fasciculations

Spinobulbar Muscular Atrophy (Kennedy Disease)

MANAGEMENT

Pharmacotherapy

Multidisciplinary Supportive Care

OUTCOME

LEVEL 1 EVIDENCE

89 Bell’s Palsy and Cranial Neuropathies

INTRODUCTION

THE OLFACTORY NERVE (CRANIAL NERVE 1)

THE TRIGEMINAL NERVE (CRANIAL NERVE V)

Trigeminal Neuralgia

Epidemiology and Pathobiology

Diagnosis

Management and Outcome

THE FACIAL NERVE (CRANIAL NERVE VII)

Bell’s Palsy

Epidemiology and Pathobiology

Diagnosis

Treatment and Outcome

Ramsay Hunt Syndrome

Other Causes of Facial Nerve Injury

THE GLOSSOPHARYNGEAL NERVE (CRANIAL NERVE IX)

Glossopharyngeal Neuralgia

THE VAGUS NERVE (CRANIAL NERVE X)

THE SPINAL ACCESSORY NERVE (CRANIAL NERVE XI)

THE HYPOGLOSSAL NERVE (CRANIAL NERVE XII)

LEVEL 1 EVIDENCE

90 Mononeuropathies and Compression Neuropathies

APPROACH TO PERIPHERAL NERVE DISORDERS

Epidemiology

Clinical Features

Etiology and Diagnosis

Outcome

THE SPINAL ROOTS AND BRACHIAL PLEXUS

Trunk and Root Injury

Upper Radicular Syndrome

Middle Radicular Syndrome

Lower Radicular Syndrome (Klumpke Palsy)

Cord Injury

Diffuse Plexus Injury

Thoracic Outlet Syndrome

Pathology

Clinical Features

Diagnosis and Treatment

THE SPINAL ROOTS AND LUMBAR AND SACRAL PLEXUSES

The Lumbar Plexus

The Sacral Plexus

Radiation Plexopathy

THE PROXIMAL NERVES OF THE ARM

The Axillary Nerve

The Long Thoracic Nerve

The Brachial Cutaneous and Antebrachial Cutaneous Nerves

The Suprascapular Nerve

THE PERIPHERAL NERVES

The Radial Nerve

The Median Nerve

The Ulnar Nerve

The Musculocutaneous Nerve

THE NERVES OF THE LEG

The Obturator Nerve

The Iliohypogastric Nerve

The Ilioinguinal Nerve

The Genitofemoral Nerve

The Lateral Femoral Cutaneous Nerve of the Thigh

The Femoral Nerve

The Sciatic Nerve

The Common Fibular Nerve

The Tibial Nerve

MONONEUROPATHIES IN DIABETES MELLITUS

91 Acquired Peripheral Neuropathies

APPROACH TO PERIPHERAL NERVE DISORDERS

Epidemiology

Clinical Features

Etiology and Diagnosis

Treatment

Outcome

SPECIFIC ACQUIRED POLYNEUROPATHIES

Guillain-Barré Syndrome

Epidemiology

Pathobiology

Clinical Features

Diagnosis and Differential Diagnosis

Treatment

Outcome

Chronic Inflammatory Demyelinating Polyneuropathy

Multifocal Motor Neuropathy

Sensory Neuronopathy and Neuropathy

Idiopathic Autonomic Neuropathy

Vasculitic and Cryoglobulinemic Neuropathies

Neuropathies Associated With Myeloma and Nonmalignant Immunoglobulin G or Immunoglobulin A Monoclonal Gammopathies

Neuropathies Associated With Immunoglobulin M Monoclonal Antibodies That React With Peripheral Nerve Glycoconjugate Antigens

Progressive Inflammatory Neuropathy (Among Swine Slaughterhouse Workers)

Amyloid Neuropathy

Neuropathy Associated With Carcinoma (Paraneoplastic Neuropathy)

Hypothyroid Neuropathy

Acromegalic Neuropathy

Hyperthyroid Neuropathy

Celiac Neuropathy

Uremic Neuropathy

Neuropathy Associated With Hepatic Disease

Neuropathies Associated With Infection

Neuropathy of Leprosy

Diphtheritic Neuropathy

HIV-Related Neuropathies

Neuropathy of Herpes Zoster

Lyme Neuropathy

Sarcoid Neuropathy

Polyneuropathy Associated With Dietary States

Critical Illness Polyneuropathy

Neuropathies Caused by Heavy Metals

Arsenic

Lead

Mercury

Thallium

Other Chemicals

Neuropathies Caused by Therapeutic Drugs

Alcoholic Neuropathy

Diabetic Neuropathy

Generalized Polyneuropathies

Idiopathic Neuropathy

LEVEL 1 EVIDENCE

92 Inherited Peripheral Neuropathies

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

CLINICAL PRESENTATION

DIAGNOSIS

Genetic Testing

Age of Onset and Progression

Associated Symptoms and Signs

Neurophysiologic Pattern

Inheritance Pattern

At-Risk Populations

MANAGEMENT

Gene Therapy

Normalization of Gene Dosage

Neurotrophic Support

Reduction of Neurotoxic Aggregates/Misfolded Proteins

Targeting Transport Defects

Cellular Reprogramming and High-Throughput Therapeutic Screening

OUTCOME

93 Myasthenia Gravis and Other Disorders of the Neuromuscular Junction

INTRODUCTION

MYASTHENIA GRAVIS

Epidemiology

Pathobiology

Autoimmune Antibodies

T Cell Autoimmunity

New Insights

Diagnosis

Clinical Features

Laboratory Testing and Electrophysiologic Testing

Management

Acetylcholinesterase Inhibitors

Glucocorticoids

Steroid-Sparing Agents

Plasma Exchange and Intravenous Immunoglobulins

Other Immunomodulatory Treatments

Thymectomy

Myasthenic Crisis

Vaccination in Myasthenia Gravis

Pregnancy and Myasthenia Gravis

Outcome

CONGENITAL MYASTHENIC SYNDROMES

Neonatal Myasthenia Gravis

Congenital Myasthenic Syndromes

Presynaptic Congenital Myasthenic Syndromes

Synaptic Basal Lamina-Associated Syndromes

Defects in the Acetylcholine Receptor

Defects in Postsynaptic Membrane

Defects in Glycosylation

Other Congenital Myasthenic Syndromes

Treatment of Congenital Myasthenic Syndromes

DRUG-INDUCED MYASTHENIA GRAVIS

Penicillamine

Immune Checkpoint Inhibitors

Medications Which may Exacerbate Myasthenia Gravis

LAMBERT-EATON MYASTHENIC SYNDROME

Introduction

Epidemiology

Pathobiology

Diagnosis

Clinical Features

Electrodiagnosis and Laboratory Testing

Management

BOTULISM

Epidemiology and Pathobiology

Diagnosis

Clinical Features

Laboratory and Electrodiagnostic Testing

Management

ACKNOWLEDGMENT

LEVEL 1 EVIDENCE

94 Inflammatory and Autoimmune Myopathies

INTRODUCTION

DERMATOMYOSITIS

Introduction

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

ANTISYNTHETASE SYNDROME AND OVERLAP MYOSITIS

Introduction

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

SPORADIC INCLUSION BODY MYOSITIS

Introduction

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

IMMUNE-MEDIATED NECROTIZING MYOPATHY

Introduction

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

POLYMYOSITIS

MUSCLE PATHOLOGY

Dermatomyositis

Antisynthetase Syndrome

Polymyositis and Inclusion Body Myositis

Immune-Mediated (Autoimmune) Necrotizing Myopathy

LEVEL 1 EVIDENCE

95 Critical Illness Myopathy and Neuropathy

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

DIAGNOSIS

Clinical Manifestations

Laboratory Testing and Electrophysiology

Muscle Biopsy

MANAGEMENT

OUTCOME

LEVEL 1 EVIDENCE

96 Endocrine and Toxic Myopathies

INTRODUCTION

ENDOCRINE MYOPATHIES

Epidemiology

Pathobiology

Diagnosis

Clinical Features

Diagnostic Testing

Management

Outcome

TOXIC MYOPATHIES

Epidemiology

Pathobiology

Diagnosis

Clinical Features

Diagnostic Testing

Management

Outcome

97 Periodic Paralysis and Other Channelopathies

INTRODUCTION

HYPOKALEMIC PERIODIC PARALYSIS

Epidemiology and Pathobiology

Clinical Manifestations and Diagnosis

Management and Outcome

HYPERKALEMIC PERIODIC PARALYSIS

Epidemiology and Pathobiology

Clinical Manifestations and Diagnosis

Management and Outcome

ANDERSEN-TAWIL SYNDROME

Epidemiology and Pathobiology

Clinical Manifestations and Diagnosis

Management and Outcome

NONDYSTROPHIC MYOTONIAS

Epidemiology and Pathobiology

Clinical Manifestations and Diagnosis

Management and Outcome

ACKNOWLEDGMENT

LEVEL 1 EVIDENCE

98 Stiff Person Syndrome and Peripheral Nerve and Muscle Hyperexcitability

INTRODUCTION

STIFF PERSON SYNDROME

Epidemiology

Pathobiology

Clinical Manifestations

Diagnosis

Laboratory Data

Electrophysiology

Imaging

Treatment and Outcomes

OTHER CENTRAL NERVOUS SYSTEM DISORDERS OF MUSCLE STIFFNESS

PERIPHERAL NERVE AND MUSCLE HYPEREXCITABILITY

Acquired Neuromyotonia (Isaac Syndrome)

Epidemiology

Pathobiology

Clinical Manifestations

Diagnosis

Treatment

Tetany

Neuroleptic Malignant Syndrome and Malignant Hyperthermia

Muscle Cramps

Epidemiology

Pathobiology

Clinical Manifestations

Diagnosis

Treatment

Outcome

LEVEL 1 EVIDENCE

99 Metabolic and Mitochondrial Myopathies in Adults

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

DIAGNOSIS

Disorders of Glycogen Metabolism (Glycogenoses)

Disorders of Lipid Metabolism

Mitochondrial Diseases

Approach to Diagnostic Testing

MANAGEMENT

OUTCOME

LEVEL 1 EVIDENCE

SECTION XIV NEUROONCOLOGY

100 Gliomas

INTRODUCTION

Epidemiology

Pathobiology

Molecular Features of Gliomas

Diagnosis

Management

Surgery

Radiation Therapy

Chemotherapy

Outcome

ASTROCYTOMAS

Pilocytic Astrocytoma

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

Diffuse Astrocytoma

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

Anaplastic Astrocytoma and Glioblastoma

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

OLIGODENDROGLIOMAS

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Management of WHO Grade II Diffuse Oligodendrogliomas

Management of WHO Grade III Anaplastic Oligodendrogliomas

Outcome

LEVEL 1 EVIDENCE

101 Metastatic Tumors

INTRODUCTION

OVERVIEW OF METASTATIC TUMORS

Epidemiology

Pathobiology

Diagnosis

History and Physical Examination

Laboratory Studies

Imaging

Tissue Diagnosis

Management

Corticosteroids and Antiepileptics

Surgery

Radiation Therapy

Chemotherapy

BRAIN METASTASIS

Epidemiology and Clinical Manifestations

Diagnosis

Management

Focal Treatment

Whole Brain Radiation Therapy in Addition to Focal Treatment

Reirradiation

Chemotherapy, Targeted Therapy, and Immunotherapy

EPIDURAL SPINAL COLUMN METASTASIS

Epidemiology and Clinical Manifestations

Diagnosis

Management

LEPTOMENINGEAL METASTASIS

Epidemiology and Clinical Presentation

Diagnosis

Management

CONCLUSIONS

LEVEL 1 EVIDENCE

102 Meningiomas

INTRODUCTION

MENINGIOMAS

Epidemiology

Pathobiology

Clinical Manifestations

Diagnosis

Treatment

Surgery

Radiotherapy

Chemotherapy

Outcomes

World Health Organization Grade I Meningioma

World Health Organization Grade II Meningioma

World Health Organization Grade III Meningioma

Incidental Meningioma

OTHER DURAL DISEASES

Hemangiopericytomas

Dural Metastases

Hypertrophic Pachymeningitis

103 Primary Central Nervous System Lymphoma

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

CLINICAL FEATURES AND DIAGNOSIS

TREATMENT

Newly Diagnosed Primary Central Nervous System Lymphoma

Treatment of Primary Central Nervous System Lymphoma in Elderly Patients

Relapsed and Refractory Primary Central Nervous System Lymphoma

Neurotoxicity

OUTCOME

LEVEL 1 EVIDENCE

104 Tumors of the Pituitary Gland

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

CLINICAL MANIFESTATIONS

DIAGNOSIS

Radiographic Features

Endocrine Evaluation

Differential Diagnosis

MANAGEMENT

Surgery

Radiation Therapy

Recurrent Tumors

OUTCOME

Surgery

Radiation Therapy

Medical Therapy

105 Pineal Region Tumors

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Histologic Subtypes

Germ Cell Tumors

Pineal Cell Tumors

Gliomas

Meningiomas

Metastasis and Other Miscellaneous Tumors

Pineal Cysts

CLINICAL FEATURES

DIAGNOSIS

MANAGEMENT

Management of Hydrocephalus

Surgery

Postoperative Staging

Radiotherapy

Chemotherapy

OUTCOME

106 Acoustic Neuroma and Other Skull Base Tumors

VESTIBULAR SCHWANNOMA (ACOUSTIC NEUROMA)

Epidemiology

Pathobiology

Diagnosis

Management and Outcomes

Observation/Expectant Management

Stereotactic Radiosurgery

Surgical Resection

Chemotherapy

MALIGNANT TUMORS OF THE SKULL AND SKULL BASE

Metastasis to the Skull Base

Epidemiology

Diagnosis

Management and Outcomes

Extension of Malignant Tumors to the Skull Base

Primary Malignant Tumors of the Skull and Skull Base

Chondrosarcoma

Chordoma

Osteogenic Sarcoma

Soft-Tissue Sarcoma

Glomus Jugulare Tumors

BENIGN TUMORS OF THE SKULL

Osteoma

Chondroma

Hemangioma

Dermoid and Epidermoid Tumors

NEOPLASTIC-LIKE LESIONS OF THE SKULL

Hyperostosis

Fibrous Dysplasia

Paget Disease (Osteitis Deformans)

Mucocele

Miscellaneous

107 Spinal Tumors

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

CLINICAL FEATURES

Extramedullary Tumors

Extradural (Epidural) Tumors

Epidural Spinal Cord Metastasis

Intradural Extramedullary Tumors

Intramedullary Tumors

Primary Intramedullary Tumors

Intramedullary Metastases

Regional Syndromes

Foramen Magnum Tumors

Cervical Tumors

Thoracic Tumors

Lumbar Tumors

Tumors of the Conus and Cauda Equina

DIAGNOSIS

Radiography

Cerebrospinal Fluid

MANAGEMENT

Intradural Extramedullary Tumors

Intramedullary Tumors

Epidural Disease From Metastatic Cancer

OUTCOME

LEVEL 1 EVIDENCE

108 Paraneoplastic Syndromes

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

CLINICAL MANIFESTATIONS

Encephalitis

Epilepsy

Myelitis

Cerebellar Ataxia

Neuropathies

Sensory Neuronopathy

Sensory-Motor Neuropathies

Autonomic Neuropathies

Motor Neuropathies

Peripheral Hyperexcitability

Neuromuscular Junction Disorders

Myopathy

Movement Disorders

Paraneoplastic Disorders Affecting the Eyes

MANAGEMENT

Diagnosis

Treatment

OUTCOMES

LEVEL 1 EVIDENCE

109 Complications of Cancer Therapy

INTRODUCTION

NEUROLOGIC COMPLICATIONS OF RADIOTHERAPY

Introduction

Pathobiology

Clinical Manifestation, Epidemiology, and Diagnosis

Acute Encephalopathy

Pseudoprogression or Worsening of Preexisting Symptoms

Focal Radionecrosis

Leukoencephalopathy and Cognitive Impairment

Radiation-Induced Myelopathy

Cranial Neuropathies

Brachial and Lumbosacral Plexopathies

Indirect Radiation-Induced Complications to Nervous System

Management and Outcome

NEUROLOGIC COMPLICATIONS OF CHEMOTHERAPY

Introduction

Pathobiology

Clinical Manifestations, Epidemiology, and Diagnosis

Acute or Subacute Encephalopathy

Acute Cerebellar Syndrome

Posterior Reversible Encephalopathy Syndrome

Chronic Leukoencephalopathy

Peripheral Neuropathy

Management and Outcome

NEUROLOGIC COMPLICATIONS OF BIOLOGIC AGENTS

Monoclonal Antibodies

Small Molecule Inhibitors

NEUROLOGIC COMPLICATIONS OF IMMUNOTHERAPY

Introduction, Epidemiology, and Pathobiology

Clinical Manifestations and Diagnosis

Management and Outcome

NEUROLOGIC COMPLICATIONS OF HEMATOPOIETIC STEM CELL TRANSPLANTATION

Introduction and Epidemiology

Pathobiology, Clinical Manifestations, and Diagnosis

Management and Outcome

CONCLUSION

LEVEL 1 EVIDENCE

SECTION XV HYDROCEPHALUS AND CEREBRAL EDEMA

110 Hydrocephalus

INTRODUCTION

CLASSIFICATION

CEREBROSPINAL FLUID DIVERSION SYSTEMS

ACUTE OBSTRUCTIVE HYDROCEPHALUS

Epidemiology

Pathobiology

Stroke and Intraventricular Hemorrhage

Tumor Related (Ball and Valve)

Clinical Features

Diagnosis

Treatment and Outcomes

CONGENITAL OBSTRUCTIVE HYDROCEPHALUS

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Treatment and Outcomes

COMMUNICATING HYDROCEPHALUS

NORMAL-PRESSURE HYDROCEPHALUS

Epidemiology

Pathobiology

Clinical Manifestations

Diagnosis

Treatment and Outcomes

LOW-PRESSURE HYDROCEPHALUS

Epidemiology

Pathobiology

Clinical Diagnosis

Treatment and Outcome

EXTERNAL HYDROCEPHALUS

GENERAL COMPLICATIONS AFTER SHUNT PLACEMENT

LEVEL 1 EVIDENCE

111 Brain Edema and Increased Intracranial Pressure

INTRODUCTION

BRAIN EDEMA

Pathobiology

Vasogenic Edema

Cytotoxic Edema

Osmotic Edema

Interstitial (Hydrocephalic) Edema

Selected Causes of Brain Edema

Cerebral Infarction

Intracerebral Hemorrhage

Subarachnoid Hemorrhage

Venous Infarction

Cardiac Arrest

Traumatic Brain Injury

Brain Tumors

Fulminant Hepatic Encephalopathy

Reye Syndrome

Brain Abscess

Viral Encephalitis

Meningitis

Posterior Reversible Encephalopathy Syndrome

High-Altitude Cerebral Edema

Postendarterectomy Hyperperfusion Syndrome

Intracranial Pressure Management

General Approach

Tiered Approach to Intracranial Pressure Management in a Monitored Patient

Advanced Treatment Options for Medically Refractory Intracranial Pressure

Salvage Interventions for Super-Refractory Intracranial Pressure

Specific Treatments for Brain Edema

Glucocorticoids

Mannitol

Hypertonic Saline

IDIOPATHIC INTRACRANIAL HYPERTENSION

Causes of Idiopathic Intracranial Hypertension

Endocrine and Metabolic Disorders

Drugs and Toxins

Hematologic and Connective Tissue Disorders

Pulmonary Encephalopathy

Spinal Cord Diseases

Pathobiology

Clinical Features

Diagnosis

Treatment

LEVEL 1 EVIDENCE

112 Spontaneous Intracranial Hypotension

INTRODUCTION

SPONTANEOUS INTRACRANIAL HYPOTENSION

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

General Management

Outcomes

POSTCRANIOTOMY BRAIN SAGGING

Clinical Features

Diagnosis

Management

SECTION XVI SPINAL CORD DISORDERS

113 Intervertebral Disk Disease and Radiculopathy

INTRODUCTION

EPIDEMIOLOGY

PATHOPHYSIOLOGY

Intervertebral Disk Disease

CLINICAL FEATURES

Radicular Pain and Lumbar Disk Disease

Thoracic Disk Rupture

Cervical Disk Disease

Myelopathy

DIAGNOSIS

Initial Management and Evaluation

Considerations in the Cervical Spine

Other Diagnostic Considerations

Imaging and Electrodiagnostic Studies

Magnetic Resonance Imaging

Myelography and X-ray

Electromyography and Nerve Conduction Studies

TREATMENT

Expert Referral

Lumbar Diskectomy

Cervical Diskectomy

SUMMARY

LEVEL 1 EVIDENCE

114 Cervical and Lumbar Spinal Stenosis

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Spondylosis

Congenital Stenosis

Myelopathy

Pain

DIAGNOSIS

Clinical Manifestations

Cervical Stenosis

Lumbar Stenosis

Physical Examination

Radiographic Studies

Plain Radiography

Computed Tomography and Computed Tomography Myelography

Magnetic Resonance Imaging

Additional Tests

MANAGEMENT

Cervical Stenosis

Lumbar Stenosis

OUTCOMES

LEVEL 1 EVIDENCE

115 Acquired and Hereditary Myelopathies

INTRODUCTION

SYRINGOMYELIA

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

NEOPLASTIC MYELOPATHY

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

INFECTIOUS MYELOPATHY

Viral Myelitis

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

Human T-cell Lymphotropic Virus-Associated Myelopathy (Tropical Spastic Paraparesis)

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

INFLAMMATORY MYELOPATHY

Clinical Features

Diagnosis

Management

Outcome

VASCULAR MYELOPATHY

Spinal Cord Infarction

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

Spinal Arteriovenous Malformations and Fistulas

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

TOXIC/METABOLIC CAUSES OF MYELOPATHY

Myelopathy due to Radiation or Chemotherapy

Nitrous Oxide Toxicity

Hepatic Myelopathy

GENETIC CAUSES OF MYELOPATHY

Hereditary Spastic Paraplegia

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Outcome

LEVEL 1 EVIDENCE

SECTION XVII AUTONOMIC AND SLEEP DISTURBANCES

116 Autonomic Failure, Autonomic Neuropathy, and Orthostatic Intolerance

INTRODUCTION

EPIDEMIOLOGY

ACUTE AND SUBACUTE AUTONOMIC DISORDERS

Paraneoplastic Autonomic Neuropathy

Orthostatic Intolerance

CHRONIC NEUROGENIC ORTHOSTATIC HYPOTENSION AND AUTONOMIC NEUROPATHY

Parkinsonian Syndromes and Pure Autonomic Failure

Chronic Autonomic Neuropathies

DIAGNOSIS AND MANAGEMENT OF AUTONOMIC DISORDERS

HEREDITARY SENSORY AND AUTONOMIC NEUROPATHY AND FAMILIAL DYSAUTONOMIA

Familial Dysautonomia (Riley-Day Syndrome)

Clinical Manifestations

Diagnosis

Biochemical and Pathologic Data

Treatment

Outcome

Other Hereditary Autonomic Disorders

ACKNOWLEDGMENT

117 Paroxysmal Sympathetic Hyperactivity After Acute Brain Injury

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

DIAGNOSIS

Clinical Manifestations

Diagnostic Criteria

MANAGEMENT

OUTCOME

118 Sleep Disorders

INTRODUCTION

PHYSIOLOGY OF NORMAL SLEEP

NEUROANATOMY AND NEUROCHEMISTRY OF SLEEP

SPECIFIC DISORDERS OF SLEEP

Insomnia

Diagnosis

Management

The Hypersomnias

Narcolepsy

Kleine-Levin Syndrome

Sleep-Related Breathing Disorders

Snoring

Sleep Apnea

Hypoventilatory and Hypoxemic Disorders

Catathrenia

SLEEP-RELATED MOVEMENT DISORDERS

Restless Legs Syndrome and Periodic Limb Movement Disorder

Sleep-Onset Movements

Sleep-Related Movements

Circadian Rhythm Disorders

Delayed Sleep Phase

Advanced Sleep Phase

Nonentrained and Irregular Sleep Phase

Nocturnal Behaviors

Parasomnias

Non–Rapid Eye Movement Parasomnias

Rapid Eye Movement Parasomnias

CONCLUSION

LEVEL 1 EVIDENCE

SECTION XVIII SYSTEMIC ORGAN FUNCTION AND THE NERVOUS SYSTEM

119 Heart-Brain Interactions

INTRODUCTION

CEREBRAL COMPLICATIONS OF CARDIAC PROCEDURES

Epidemiology

Pathobiology

Diagnosis

Management

Prevention

Acute Ischemic Stroke

Intracranial Hemorrhage

Outcome

CARDIOVASCULAR COMPLICATIONS OF NEUROLOGIC INJURY

Neurogenic Stunned Myocardium

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Treatment and Outcome

Paroxysmal Sympathetic Storming

Epidemiology

Clinical Features

Diagnosis

Management

Outcome

LEVEL 1 EVIDENCE

120 Sepsis-Associated Encephalopathy

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Brain Signaling

Neuroinflammation and Glial Cell Activation

Astrocytes and Blood-Brain Barrier Dysfunction

Ischemic Processes

Neurotransmitter Dysfunction

DIAGNOSIS

Clinical Features

Brain Imaging

Continuous Electroencephalogram Monitoring

MANAGEMENT

OUTCOME

Impact of Sepsis-Associated Encephalopathy on Survival

Impact on Sepsis-Associated Encephalopathy on Long-term Outcome

Societal Cost of Sepsis-Associated Encephalopathy

CONCLUSION

LEVEL 1 EVIDENCE

121 Respiratory Support for Neurologic Diseases

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Pulmonary Gas Exchange

Respiratory Failure

Effects of Respiratory Compromise on Neurologic Injury

DIAGNOSIS

Examination

Pulmonary Function Testing

Blood Gas Analysis and Monitoring

MANAGEMENT OF RESPIRATORY FAILURE

Noninvasive Positive Pressure Ventilation

Endotracheal Intubation and Mechanical Ventilation

Manipulation of Ventilation: Effects on Intracranial Pressure and Cerebral Blood Flow

Prevention of Ventilator-Associated Pneumonia

Weaning From Mechanical Ventilation

Tracheostomy

Long-Term Ventilation

OUTCOMES

LEVEL 1 EVIDENCE

122 Endocrine Diseases and the Brain

INTRODUCTION

PITUITARY DISORDERS

Hypopituitarism

Epidemiology

Pathobiology

Clinical Features, Diagnosis, and Treatment

Hyperpituitarism

Epidemiology

Pathobiology

Clinical Features, Diagnosis, and Treatment

Pituitary Tumors

Pituitary Adenomas

Other Pituitary Tumors

Other Pituitary Disorders

Pituitary Apoplexy

Empty Sella

THYROID DISEASE

Hypothyroidism

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Hyperthyroidism

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

DIABETES MELLITUS

Epidemiology

Etiology

Clinical Features

Neurologic Complications

Diagnosis

Management

ADRENAL DISEASE

Adrenal Insufficiency

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Hyperadrenalism

Cushing Syndrome

Hyperaldosteronism

Pheochromocytoma

PARATHYROID DISEASE

Hypoparathyroidism

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

Hyperparathyroidism

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Management

123 Hematologic Diseases and the Brain

RED BLOOD CELL DISORDERS

Anemia

Anemia and Transfusion in Hospitalized and Critically Ill Neurologic Patients

Sickle Cell Disease

Polycythemia

Polycythemia Vera

WHITE BLOOD CELL DISORDERS

Malignancies of Lymphoid Cells

Lymphoma, Lymphoblastic Leukemia, and Posttransplant Lymphoproliferative Disorders

Plasma Cell Malignancies

Acute Myeloid Leukemia

Hematopoietic Cell Transplantation

Epidemiology

Pathobiology

Clinical Manifestations, Diagnosis, and Treatment

PLATELET DISORDERS

Thrombocytopenia

Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome

Heparin-Induced Thrombocytopenia

Impaired Platelet Function

Thrombocytosis

Essential Thrombocythemia

COAGULATION DISTURBANCES

Normal Coagulation

Impaired Coagulation

Warfarin-Induced Intracranial Hemorrhage

Reversal of Novel Oral Anticoagulants

Bleeding Associated With Thrombolytic Therapy

Bleeding Associated With Unfractionated or Low Molecular Weight Heparin

Hypercoagulable States

Familial Thrombophilia

Antiphospholipid Antibody Syndrome

LEVEL 1 EVIDENCE

124 Hepatic Disease and the Brain

INTRODUCTION

CIRRHOSIS AND HEPATIC ENCEPHALOPATHY

Epidemiology

Pathobiology

Clinical Features

Cirrhosis

Minimal Hepatic Encephalopathy

Overt Hepatic Encephalopathy

Hepatic Myelopathy

Persistent Hepatic Encephalopathy

Diagnosis

Minimal Hepatic Encephalopathy

Overt Hepatic Encephalopathy

Treatment

Minimal Hepatic Encephalopathy

Overt Hepatic Encephalopathy

Outcome

ACUTE LIVER FAILURE AND CEREBRAL EDEMA

Epidemiology

Pathobiology

Multiple Organ Failure

Encephalopathy and Cerebral Edema

Clinical Features

Diagnosis

Treatment

General Management

High-Volume Plasma Exchange

Intracranial Pressure and Transcranial Doppler Monitoring

Intracranial Pressure Management

Seizures

Ammonia Clearance

Artificial Liver Support Systems

Transplantation

Outcome

LEVEL 1 EVIDENCE

125 Renal Disease, Electrolyte Disorders, and the Nervous System

INTRODUCTION

UREMIC ENCEPHALOPATHY

Pathobiology

Diagnosis

Treatment

DIALYSIS DISEQUILIBRIUM SYNDROME

DIALYSIS DEMENTIA

CEREBROVASCULAR DISEASE

RESTLESS LEGS SYNDROME

PERIPHERAL NEUROPATHY

Pathobiology

Diagnosis

Management

MYOPATHY

NEUROLOGIC COMPLICATIONS OF RENAL TRANSPLANTATION

Posterior Reversible Encephalopathy Syndrome

Primary Central Nervous System Lymphoma

Opportunistic Infections

HYPONATREMIA

Clinical Features

Diagnosis

Management

Acute Symptomatic Hyponatremia

Chronic Hyponatremia

OSMOTIC DEMYELINATION SYNDROME

Epidemiology

Pathobiology

Clinical Manifestations

Diagnosis

Prevention and Treatment

Outcome

126 Gastric and Genitourinary Function and the Brain

INTRODUCTION

NEUROANATOMY

GASTROINTESTINAL MANIFESTATIONS OF NEUROLOGIC DISEASE

Dysphagia

Esophageal Dysmotility

Gastroparesis

Stress Gastric Ulcer

Bowel Dysmotility

Incontinence

URINARY MANIFESTATIONS OF NEUROLOGIC DISEASE

Voiding Dysfunction

Storage Dysfunction

SEXUAL MANIFESTATIONS OF NEUROLOGIC DISEASE

Female Sexual Dysfunction

Male Sexual Dysfunction

NEUROLOGIC DISORDERS AND THE GASTROINTESTINAL AND GENITOURINARY SYSTEMS

Peripheral Neuropathy

Spinal Cord Injury

Multiple Sclerosis

Parkinsonism

Cortical and Subcortical Disease

NEUROLOGIC CONSEQUENCES OF PRIMARY GASTROINTESTINAL DISEASE

THE MICROBIOME-GUT-BRAIN AXIS AND NEUROLOGIC DISEASE

LEVEL 1 EVIDENCE

127 Bone Diseases and the Central Nervous System

PAGET DISEASE

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

FIBROUS DYSPLASIA

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

ACHONDROPLASIA

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

ANKYLOSING SPONDYLITIS

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

ATLANTOAXIAL DISLOCATION

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

FRACTURES AND SECONDARY OSTEOPOROSIS: NEUROLOGIC CAUSES

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

LEVEL 1 EVIDENCE

128 Malnutrition, Malabsorption, and Vitamin Deficiencies

INTRODUCTION

CAUSES OF NUTRITIONAL DEFICIENCIES

VITAMIN DEFICIENCY AND EXCESS SYNDROMES

Vitamin B12 (Cobalamin) Deficiency

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

Vitamin B1 (Thiamine) Deficiency

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

Vitamin B6 (Pyridoxine) Deficiency or Excess

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

Vitamin B9 (Folic Acid) Deficiency

Vitamin B3 (Niacin) Deficiency

Vitamin A Deficiency or Toxicity

Vitamin D Deficiency

Vitamin E Deficiency

MINERAL DEFICIENCY AND EXCESS SYNDROMES

Copper Deficiency and Toxicity

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

Wilson Disease

Other Mineral Deficiencies and Toxicities

NEUROLOGIC COMPLICATIONS OF BARIATRIC SURGERY

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

NEUROLOGIC COMPLICATIONS OF GLUTEN-RELATED DISORDERS

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

129 Neurologic Disease During Pregnancy

INTRODUCTION

Biology of Pregnancy

EPILEPSY

Epidemiology

Seizure Control and Monitoring Antiseizure Medication Concentrations

Teratogenicity

Effects of Antiseizure Medications on Cognition

Management of Epilepsy During Pregnancy

Breastfeeding

PREECLAMPSIA AND ECLAMPSIA

Epidemiology

Clinical Features

Diagnosis

Treatment

STROKE

Epidemiology

Ischemic Stroke

Cerebral Venous Thrombosis

Diagnosis

Treatment

Intravenous Thrombolysis

Mechanical Thrombectomy

Unfractionated and Low Molecular Weight Heparin

CEREBRAL HEMORRHAGE

Epidemiology

Diagnosis and Treatment

MULTIPLE SCLEROSIS

Epidemiology

Clinical Features

Pathobiology

Treatment

MIGRAINE

Epidemiology

Clinical Features

Pathobiology

Diagnosis

Treatment

NEOPLASMS

Epidemiology

Clinical Features and Diagnosis

Treatment

NEUROPATHIES

Carpal Tunnel Syndrome

Bell Palsy

Meralgia Paresthetica

MYASTHENIA GRAVIS

Epidemiology

Clinical Features

Treatment

MOVEMENT DISORDERS

Restless Legs Syndrome

Chorea Gravidarum

Parkinsonism and Idiopathic Parkinson Disease

LEVEL 1 EVIDENCE

130 Neurologic Complications of Organ Transplantation

INTRODUCTION

GENERAL CONSIDERATIONS

Central Nervous System Infections

Encephalopathy and Delirium

Drug Toxicity

ORGAN-SPECIFIC COMPLICATIONS

Lung Transplantation

Cardiac Transplantation

Liver Transplantation

Intestinal Transplantation

LONG-TERM EFFECTS OF IMMUNOSUPPRESSION

CONCLUSIONS

SECTION XIX NEUROLOGIC TOXIDROMES

131 Alcoholism

INTRODUCTION

ACUTE ETHANOL SYNDROMES

Intoxication

Ethanol-Drug Interactions

Ethanol Withdrawal

Clinical Manifestations

Pathobiology

Management

CHRONIC ETHANOL SYNDROMES

Wernicke-Korsakoff Syndrome

Clinical Manifestations

Pathobiology

Management and Outcome

Alcoholic Cerebellar Degeneration

Alcoholic Polyneuropathy

Alcoholic Amblyopia

Pellagra

Alcoholic Liver Disease

Hypoglycemia

Alcoholic Ketoacidosis

Infection in Alcoholics

Trauma in Alcoholics

Alcohol and Cancer

Alcohol and Stroke

Alcoholic Myopathy

Central Pontine Myelinolysis and Marchiafava-Bignami Disease

Alcoholic Dementia

Fetal Alcohol Spectrum Disorders

TREATMENT OF CHRONIC ALCOHOLISM

132 Drug Intoxication and Withdrawal

INTRODUCTION

DRUGS OF DEPENDENCE

Opioids

Psychostimulants

Sedatives

Marijuana

Hallucinogens

Inhalants

Phencyclidine

Anticholinergics

COMPLICATIONS OF DRUG TOXIDROMES

Trauma

Infection

Seizures

Stroke

Altered Mentation

Fetal Effects

Miscellaneous Effects

133 Neurotoxicology

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

CLINICAL FEATURES

DIAGNOSIS

TREATMENT

OUTCOME

COMMON NEUROTOXIDROMES

Anticholinergic Toxicity

Clinical Features

Treatment

Sympathomimetic Toxicity and Acute Excited State

Clinical Features

Diagnosis

Treatment

Cholinergic Toxicity: Organophosphates and Carbamates

Epidemiology

Pathobiology

Clinical Features

Diagnosis

Treatment

Outcome

Neuroleptic Malignant Syndrome

Clinical Features

Diagnosis

Treatment

Outcome

Serotonin Syndrome

Pathobiology

Diagnosis

Management

Carbon Monoxide Poisoning

Clinical Features

Diagnosis

Treatment

Malignant Hyperthermia

Strychnine Poisoning

Methanol (Methyl Alcohol)

Volatile Organic Compounds

Nitrous Oxide Myelopathy

Heavy Metals

Lead

Mercury

Arsenic

Thallium

Manganese

Aluminum

Plant, Animal, and Marine Neurotoxins

ACKNOWLEDGMENT

134 Radiation Injury

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

DIAGNOSIS

Acute Radiation Sickness

Cerebral Edema and Early Delayed Leukoencephalopathy

Radiation Necrosis

Myelopathy

Radiation-Induced Vasculopathy and SMART Syndrome

Neuropathy and Plexopathy

Optic Neuropathy and Ocular Neuromyotonia

Neuroendocrine Dysfunction

Neurocognitive Effects

MANAGEMENT

OUTCOME

LEVEL 1 EVIDENCE

135 Electrical and Lightning Injury

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Electrical Injuries

Lightning Strikes

CLINICAL FEATURES

DIAGNOSIS

MANAGEMENT

OUTCOME

136 Decompression Illness

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

DIAGNOSIS

MANAGEMENT

OUTCOME

LEVEL 1 EVIDENCE

SECTION XX PEDIATRIC NEUROLOGY

137 Neonatal Neurology

GERMINAL MATRIX-INTRAVENTRICULAR HEMORRHAGE

Epidemiology

Pathobiology

Clinical Manifestations

Diagnosis

Management

Prevention

Therapeutic Intervention

Outcome

PERINATAL ASPHYXIA/HYPOXIC-ISCHEMIC ENCEPHALOPATHY

Epidemiology

Pathobiology

Clinical Manifestations

Diagnosis

Management

Outcome

NEONATAL SEIZURES

Epidemiology

Pathobiology

Clinical Manifestations

Diagnosis

Management

Outcome

STROKE AND SINUS VENOUS THROMBOSIS

Epidemiology

Pathobiology

Clinical Manifestations

Diagnosis

Management

Outcome

PERIVENTRICULAR LEUKOMALACIA

Epidemiology

Pathobiology

Clinical Manifestations

Diagnosis

Management

Outcome

LEVEL 1 EVIDENCE

138 Nervous System Formation and Malformation

OVERVIEW

FORMATION OF NEUROECTODERM

NEURAL TUBE CLOSURE

Primary Neurulation

Specific Neural Tube Defects

Encephaloceles

Meckel Syndrome

EMBRYONIC NERVOUS SYSTEM SEGMENTATION

Transcriptional Factors and Homeobox Genes

Septo-Optic Dysplasia

VENTRAL INDUCTION

Normal Prosencephalon Division

Disorders of Ventral Induction

Holoprosencephaly

NEURONAL AND GLIAL PROLIFERATION

Normal Cell Proliferation

Disorders of Neuronal and Glial Proliferation

Microcephaly

Megalencephaly and Hemimegalencephaly

NEURONAL DIFFERENTIATION

Normal Differentiation

NEURONAL MIGRATION

Normal Migration

Cortical Neuronal Migration Disorders

Lissencephaly

Kallmann Syndrome

Zellweger Syndrome

Polymicrogyria

Congenital Bilateral Perisylvian Syndrome

Heterotopias

Cortical Dysgenesis

POSTERIOR FOSSA FORMATION AND MALFORMATION

Joubert, Meckel-Gruber, and Other Ciliopathies

Rhombencephalosynapsis

Dandy-Walker

PROCESS OUTGROWTH AND SYNAPSE FORMATION

Corpus Callosum

Normal Synapse Formation

Disorders of Process Outgrowth

CONCLUSIONS

139 Inherited Metabolic Diseases

INTRODUCTION

SMALL MOLECULE DISORDERS

Glucose Transporter Type 1 Deficiency Syndrome

Clinical Syndrome

Laboratory Data

Molecular Genetics and Pathogenesis

Diagnosis

Management

Hyperammonemia

Neonatal Hyperammonemia

Hyperammonemia in Older Children and Adults

Valproate-Associated Hyperammonemia

Disorders of Amino Acid Metabolism

Phenylketonuria

Other Amino Acid Disorders

Organic Acidurias

Disorders of Purine and Pyrimidine Metabolism

Introduction

Lesch-Nyhan Syndrome

Other Purine Disorders

Porphyrias

Disorders of Metal Metabolism

Disorders of Copper Metabolism

Disorders of Iron Metabolism

Disorders Associated With Acanthocytosis

LARGE MOLECULE DISORDERS

Lysosomal and Associated Large Molecule Disorders

Lipidoses

Mucopolysaccharidoses

Mucolipidoses

Neuronal Ceroid Lipofuscinoses

Leukodystrophies

Disorders of Carbohydrate Metabolism

Glycogen Storage Diseases

Peroxisomal Diseases: Adrenoleukodystrophy, Zellweger Syndrome, and Refsum Disease

Zellweger Spectrum Disorder

Adrenoleukodystrophy

Refsum Disease

140 Chromosomal Disorders and Disorders of DNA

INTRODUCTION

CHROMOSOMAL DISORDERS

Genomic Imprinting

Autism Spectrum Disorder and Genomic Imprinting

Intellectual Disability Associated With Subtelomeric Chromosomal Deletion

Chromosomal Microarray

Array Comparative Genomic Hybridization

DISORDERS OF DNA MAINTENANCE, TRANSCRIPTION, AND TRANSLATION

DNA Maintenance and Transcription

DNA Translation and Modification

Cell Cycle Control

Specific Disorders

141 The Floppy Infant

INTRODUCTION

CENTRAL HYPOTONIA

Vascular Causes

Structural Causes

Metabolic Causes

Systemic Causes

Chromosomal Disorders

Spinal Cord

PERIPHERAL HYPOTONIA

Anterior Horn Cell Disorders

Brachial Plexus Palsy

Peripheral Neuropathy

Demyelinating Neuropathies

Axonal Neuropathies

Mixed Axonal and Demyelinating Neuropathy

Neuromuscular Junction Disorders

Muscle Disorders

142 Disorders of Motor and Cognitive Development

INTRODUCTION

DISORDERS OF MOTOR DEVELOPMENT

Approach to the Child With Motor Delay

Cerebral Palsy

Spastic Diplegia

Spastic Quadriplegia

Spastic Hemiparesis

Dyskinetic Cerebral Palsy

Ataxic Cerebral Palsy

Management of Cerebral Palsy

Developmental Coordination Disorder

DISORDERS OF HIGHER CEREBRAL FUNCTIONS

Approach to the Child With Language, Cognitive, or Academic Delays

Developmental Language Disorders

Intellectual Disability

Learning Disabilities

Dyslexia

Nonverbal Learning Disabilities

Dyscalculia

Attention Deficit Hyperactivity Disorder

LEVEL 1 EVIDENCE

143 Autism Spectrum Disorders

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Genetics

Neuropathology

DIAGNOSIS

Evaluation

Neurologic Examination

Cognition and Language

Medical and Psychiatric Comorbidities

Epilepsy

Neurodiagnostic Testing

MANAGEMENT

OUTCOME

144 Mitochondrial Encephalomyopathies

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Pathobiology of mtDNA Mutations: Mitochondrial Genetics Versus Mendelian Inheritance

Mendelian Inheritance

Direct Hits Mutations

Indirect Hits Mutations

Defects of Mitochondrial Ribonucleic Acid (mtRNA) Translation

Defects of the Inner Mitochondrial Membrane Lipid Milieu

Defects of Mitochondrial Dynamics

Defects of Mitochondrial DNA Maintenance

Disorders of Mitochondrial Protein Importation

DIAGNOSIS

Mitochondrial DNA-Related Diseases

Mendelian Inheritance

Defects of the Inner Mitochondrial Membrane Lipid Milieu

Defects of Mitochondrial Translation

Defects of Mitochondrial Dynamics

Defects of Mitochondrial DNA Maintenance

Mitochondrial DNA Depletion Syndromes

Syndromes due to Multiple Mitochondrial DNA Deletions

Coexistence of Mitochondrial DNA Depletion and Mitochondrial DNA Multiple Deletions

DIAGNOSTIC WORKUP

Laboratory

Neuroradiology

Exercise Physiology

PATHOLOGY

Skeletal Muscle

Neuropathology

BIOCHEMICAL FINDINGS

MANAGEMENT

Mitochondrial Transfer

Shifting Heteroplasmy

Enhancement of Oxidative Phosphorylation Function

Elimination of Noxious Compounds

Small Molecule Therapies

Alteration of Mitochondrial Dynamics

145 Neurocutaneous Syndromes

INTRODUCTION

NEUROFIBROMATOSIS

Introduction

Epidemiology

Molecular Genetics and Pathogenesis

Neuropathology

Symptoms and Signs

Cutaneous Manifestations

Ocular Findings

Neurologic Symptoms

Skeletal and Limb Manifestations

Miscellaneous Manifestations

Diagnosis

Laboratory Data

Management

TUBEROUS SCLEROSIS COMPLEX

Introduction

Epidemiology

Genetics

Pathology and Pathogenesis

Symptoms and Signs

Cutaneous Findings

Neurologic Findings

Ophthalmic Findings

Visceral and Skeletal Findings

Laboratory Data

Diagnosis

Course and Prognosis

Management

ENCEPHALOTRIGEMINAL ANGIOMATOSIS

Introduction

Epidemiology

Genetics

Neuropathology

Symptoms and Signs

Cutaneous Manifestations

Neurologic Manifestations

Ophthalmologic Manifestations

Laboratory Data

Diagnosis

Management

INCONTINENTIA PIGMENTI

Introduction

Genetics

Neuropathology

Cutaneous Findings

Neurologic, Ophthalmic, and Other Symptoms

Laboratory Data

Management

INCONTINENTIA PIGMENTI ACHROMIANS (HYPOMELANOSIS OF ITO)

Introduction

Epidemiology and Genetics

Pathogenesis and Pathology

Clinical Manifestations

Diagnosis and Laboratory Data

Management

146 Spinal Muscular Atrophies of Childhood

INTRODUCTION

EPIDEMIOLOGY

CLINICAL CHARACTERISTICS

Type I Spinal Muscular Atrophy

Type II Spinal Muscular Atrophy

Type III Spinal Muscular Atrophy

Outliers

Other “Spinal Muscular Atrophies”

GENETICS

The SMN Gene

Newborn Screening

PATHOBIOLOGY

MANAGEMENT

Agents That Upregulate SMN2 Gene Expression and Promote Exon 7 Inclusion

Small Molecules

Other Approaches

Antisense Oligonucleotides

Gene Therapy

CARE OF THE PATIENT WITH SPINAL MUSCULAR ATROPHY

SUMMARY

LEVEL 1 EVIDENCE

147 Muscular Dystrophies and Congenital Myopathies

INTRODUCTION

DEFINITIONS

Muscular Dystrophy Classification

Congenital Myopathy Classification

Laboratory Diagnosis

CONGENITAL MUSCULAR DYSTROPHIES

Definitions

Epidemiology

Diagnosis

Merosin-Deficient Congenital Muscular Dystrophy

COL6-Related Dystrophies

Dystroglycanopathies

Management

DYSTROPHINOPATHIES

Definitions

Epidemiology

Duchenne and Becker Muscular Dystrophies

Duchenne Muscular Dystrophy

Becker Muscular Dystrophy

Intermediate Phenotype

Manifesting Carriers

Diagnosis

Molecular Genetics

Management

SCAPULOPERONEAL MYOPATHIES

Emery-Dreifuss Muscular Dystrophy

LMNA-Related Myopathies

FACIOSCAPULOHUMERAL MUSCULAR DYSTROPHY

Definition

Molecular Genetics

Clinical Manifestations

Associated Disorders

Differential Diagnosis

Laboratory Studies

Management

MYOTONIC MUSCULAR DYSTROPHY AND PROXIMAL MYOTONIC MYOPATHY

Definitions

Epidemiology

Diagnosis

Clinical Features

Electrodiagnostic Features

Other Clinical Features

Genetics

Myotonia Variants

Congenital Myotonic Dystrophy

Proximal Myotonic Myopathy

Management

LIMB-GIRDLE MUSCULAR DYSTROPHY

Definition

Epidemiology

Diagnosis

Clinical Manifestations

Autosomal Recessive Limb-Girdle Muscular Dystrophy

Lgmdr1/Lgmdd4 (Lgmd2a)

Lgmdr2 (Lgmd2b)

Lgmdr3-6 (Sarcoglycanopathies) (Lgmd2c-F)

Lgmdr9

Lgmdr12

Other Recessive Limb-Girdle Muscular Dystrophies

Autosomal Dominant Limb-Girdle Muscular Dystrophy

Limb-Girdle Muscular Dystrophy 1A

Limb-Girdle Muscular Dystrophy 1B

Limb-Girdle Muscular Dystrophy 1C

Limb Girdle Muscular Dystrophy 1D

Limb-Girdle Muscular Dystrophy D2

Limb-Girdle Muscular Dystrophy 1G Through Limb-Girdle Muscular Dystrophy 1H

Differential Diagnosis

Management

OCULOPHARYNGEAL MUSCULAR DYSTROPHY

OTHER RARE DYSTROPHIES

Distal Myopathies (Distal Muscular Dystrophies)

NONDYSTROPHIC MYOPATHIES—CONGENITAL MYOPATHIES

Definitions

Epidemiology

Diagnosis

X-Linked Myotubular Myopathy

Other Centronuclear Myopathies

Nemaline Myopathy

Core Myopathies—RYR1-Related Myopathies

Core Myopathies—SELENON-Related Myopathy

TTNopathies

Management

Outcomes

LEVEL 1 EVIDENCE

148 Seizures and Epilepsies in Neonates and Children

INTRODUCTION

AGE-DEPENDENT SEIZURES NOT TRADITIONALLY DIAGNOSED AS EPILEPSY

Neonatal Seizures

Febrile Seizures

AGE-DEPENDENT EPILEPSIES AND EPILEPSY SYNDROMES IN NEONATES AND CHILDREN

Benign Epilepsies in Neonates and Infants

Benign Familial Neonatal Epilepsy

Benign Familial Neonatal-Infantile Epilepsy

Benign Familial Infantile Epilepsy

Epileptic Encephalopathy Syndromes in Neonates and Infants

Ohtahara Syndrome and Early Myoclonic Encephalopathy

West Syndrome

Genetic Developmental and Epileptic Encephalopathies in Neonates and Infants

KCNQ2 Encephalopathy

STXBP1 Encephalopathy

GNAO1 Encephalopathy

ARX Disorders

SCN2A Encephalopathies

CDKL5 Encephalopathy

Epilepsy of Infancy With Migrating Focal Seizures

SCN8A Encephalopathy

Genetic Epilepsy With Febrile Seizures Plus

Dravet Syndrome

PCDH19 Girls Clustering Epilepsy

Toddler-Onset Epilepsy Syndromes

Lennox-Gastaut Syndrome

Epilepsy With Myoclonic-Atonic Seizures

Landau-Kleffner Syndrome and Continuous Spikes and Waves During Slow Sleep

Idiopathic Focal Epilepsies of Childhood

Childhood Epilepsy With Centrotemporal Spikes

Panayiotopoulos Syndrome

Gastaut Syndrome

Idiopathic Generalized Epilepsies of Childhood

Childhood Absence Epilepsy

Jeavons Syndrome

Epilepsy with Myoclonic Absences

Juvenile myoclonic epilepsy

Juvenile Absence Epilepsy

Epilepsy with Generalized Tonic-Clonic Seizures Alone

Rasmussen Encephalitis

INBORN ERRORS OF METABOLISM CAUSING SEIZURES AND EPILEPSY IN NEONATES AND CHILDREN

Pyridoxine-dependent Epilepsy

Glut1 Deficiency Syndrome

Progressive Myoclonic Epilepsies

EPILEPSIES ASSOCIATED WITH BRAIN MALFORMATIONS

Cortical Dysgenesis

Focal Cortical Dysplasia

Tuberous Sclerosis Complex

Hemimegalencephaly and Dysplastic Megalencephaly

Periventricular Nodular Heterotopias

Lissencephaly

Hypothalamic Hamartomas

Sturge-Weber Syndrome

LEVEL 1 EVIDENCE

149 Pediatric Stroke

INTRODUCTION

PERINATAL STROKE

Epidemiology

Perinatal Arterial Ischemic Stroke

Perinatal Cerebral Sinovenous Thrombosis

Perinatal Intracranial Hemorrhage

Pathobiology

Perinatal Arterial Ischemic Stroke

Perinatal Cerebral Sinovenous Thrombosis

Perinatal Intracranial Hemorrhage

Clinical Features

Perinatal Arterial Ischemic Stroke

Perinatal Cerebral Sinovenous Thrombosis

Perinatal Intracranial Hemorrhage

Diagnosis and Imaging

Perinatal Arterial Ischemic Stroke

Perinatal Cerebral Sinovenous Thrombosis

Perinatal Intracranial Hemorrhage

Management

Perinatal Arterial Ischemic Stroke

Perinatal Cerebral Sinovenous Thrombosis

Perinatal Intracranial Hemorrhage

Outcome

Perinatal Arterial Ischemic Stroke

Perinatal Cerebral Sinovenous Thrombosis

Perinatal Intracranial Hemorrhage

Epidemiology

Childhood Arterial Ischemic Stroke

Childhood Cerebral Sinovenous Thrombosis

Childhood Intracranial Hemorrhage

Pathobiology

Childhood Arterial Ischemic Stroke

Childhood Cerebral Sinovenous Thrombosis

Childhood Intracranial Hemorrhage

Diagnosis

Childhood Arterial Ischemic Stroke

Childhood Cerebral Sinovenous Thrombosis

Childhood Intracranial Hemorrhage

Management

Childhood Arterial Ischemic Stroke

Childhood Cerebral Sinovenous Thrombosis

Childhood Intracranial Hemorrhage

Outcome

Childhood Arterial Ischemic Stroke

Childhood Cerebral Sinovenous Thrombosis

Childhood Intracranial Hemorrhage

SICKLE CELL ANEMIA

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

Neurologic Deficits

Recurrence

Mortality

GENETICS OF STROKE IN CHILDREN

Disorders with Abnormal Angiogenesis

Disorders of Vessel Wall Stenosis

Connective Tissue Disorders

Thrombophilic Disorders

Genetic or Metabolic Stroke or Stroke-like Episodes

LEVEL 1 EVIDENCE

150 Brain Tumors in Children

INTRODUCTION

EPIDEMIOLOGY

Incidence

Histologies

Risk Factors

Survival

PATHOBIOLOGY

Sonic Hedgehog, Wnt Signaling, Cerebellar Development, and Medulloblastoma

Astrocytes, Growth Factors, Tumor Suppressors, and Astrocytoma

Ependymal Cells, RELA Fusions, and Ependymoma

Integrated Classification of Central Nervous System Tumors

CLINICAL FEATURES

Symptoms

Diagnosis

TREATMENT

Surgery

Radiation Therapy

Chemotherapy

SPECIFIC TYPES OF PEDIATRIC CENTRAL NERVOUS SYSTEM TUMORS

Congenital Tumors

Craniopharyngioma

Epidermoid Cysts

Teratoma and Chordoma

Choroid Plexus Tumors

Colloid Cysts

Ganglioglioma and Related Glioneuronal Tumors

Dysplastic Gangliocytoma

Dysembryoplastic Neuroepithelial Tumor

Schwannoma (Neurilemmoma)

Astrocytoma

Low-Grade Astrocytoma

High-Grade Astrocytoma

Brainstem Glioma

Spinal Cord Astrocytoma

Oligodendroglioma

Medulloblastoma and Embryonal Tumors

Medulloblastoma

Nonmedulloblastoma Embryonal Tumors

Pineoblastoma and Trilateral Retinoblastoma

Atypical Teratoid/Rhabdoid Tumor

Ependymoma and Other Tumors

Ependymoma

Central Nervous System Germ Cell Tumors

Central Nervous System Lymphoma

Meningioma and Mesenchymal Tumors

Malignant Melanoma

LATE EFFECTS OF TREATMENT AND QUALITY OF LIFE

Radiation Effects

Endocrine Dysfunction

Cardiovascular and Pulmonary Effects

Neurologic Sequelae

Secondary Malignancies

151 Pediatric Brain Injury and Shaken Baby Syndrome

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Primary Brain Injury

Secondary Brain Injury

Macroscopic Secondary Injury

Microscopic Secondary Injury

DIAGNOSIS AND MANAGEMENT

Minor Traumatic Brain Injury

Indications for Head Computed Tomography Imaging in Minor Traumatic Brain Injury

Severe Traumatic Brain Injury

Intracranial Pressure in Children

Hypothermia Following Severe Traumatic Brain Injury

Seizure Prophylaxis

Repeat Imaging

Decompressive Craniectomy

OUTCOME

LEVEL 1 EVIDENCE

152 Human Immunodeficiency Virus, Fetal Alcohol Syndrome, and Drug Effects

PEDIATRIC AIDS AND HUMAN IMMUNODEFICIENCY VIRUS INFECTION

Introduction

Epidemiology

Pathobiology

Clinical Manifestations

Human Immunodeficiency Virus Encephalopathy

Primary Central Nervous System Lymphoma

Stroke

Opportunistic Central Nervous System Infection

Less Common Manifestations

Diagnostic Testing

Human Immunodeficiency Virus Testing

Neuroimaging

Cerebrospinal Fluid

Management

Antiretroviral Therapy

Outcome

FETAL ALCOHOL SPECTRUM DISORDERS

Introduction

Epidemiology

Pathobiology

Diagnosis

Partial Fetal Alcohol Syndrome

Alcohol-Related Neurodevelopmental Disorder

Alcohol-Related Birth Defects and Other Outcomes

Postnatal Alcohol Exposure

Alcohol Withdrawal Syndrome

Management and Outcome

PRENATAL AND PERINATAL EFFECTS OF SUBSTANCE ABUSE

Cocaine

Neurobehavior

Strokes

Seizures

Malformations

Neurodevelopmental Impact

Cocaine Exposure in Childhood

Withdrawal Symptoms

Opiates

Withdrawal Symptoms

LEVEL 1 EVIDENCE

SECTION XXI PSYCHIATRY AND NEUROLOGY

153 Psychosis

INTRODUCTION

SCHIZOPHRENIA AND OTHER PSYCHOTIC DISORDERS

Epidemiology

Pathobiology

Diagnosis

Management and Outcome

Antipsychotic Medication

Nonpharmacologic Treatment

OTHER CAUSES OF PSYCHOSIS

CONCLUSION

LEVEL 1 EVIDENCE

154 Mood Disorders

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

Pathophysiology of Major Depressive Disorder

Pathophysiology of Bipolar Disorder

CLINICAL MANIFESTATIONS

Depressive Disorders

Bipolar Disorders

Seasonal Affective Disorder

DIAGNOSIS

Differential Diagnosis

Differential Diagnosis With Neurologic Disorders

Differential Diagnosis With Other Psychiatric Disorders

TREATMENT

Treatment of Depressive Disorders

Treatment of Bipolar Disorders

Treatment of Mood Disorders Comorbid With Neurologic Disorders

OUTCOME

LEVEL 1 EVIDENCE

155 Anxiety Disorders, Posttraumatic Stress Disorder, and Obsessive-Compulsive Disorder

INTRODUCTION

EPIDEMIOLOGY

PATHOBIOLOGY

DIAGNOSIS

Clinical Features Across Anxiety Disorders

Panic Disorder and Agoraphobia

Social Anxiety Disorder (Social Phobia)

Posttraumatic Stress Disorder and Acute Stress Disorder

Obsessive-Compulsive Disorder

Generalized Anxiety Disorder

Specific Phobias

Separation Anxiety Disorder

Selective Mutism

Anxiety and Neurologic Disease: Comorbidity and Differential Diagnosis

MANAGEMENT

General Approaches to Anxiety Disorders

Panic Disorder and Agoraphobia

Social Anxiety Disorder

Posttraumatic Stress Disorder and Acute Stress Disorder

Obsessive-Compulsive Disorder

Generalized Anxiety Disorder

Specific Phobias

Separation Anxiety Disorder and Selective Mutism

Treatment of Anxiety Disorders Comorbid With Neurologic Disorders

OUTCOME

LEVEL 1 EVIDENCE

156 Somatic Symptom Disorders

INTRODUCTION

Somatic Symptom Disorders in Diagnostic and Statistical Manual of Mental Disorders (Fifth Edition)

SOMATIC SYMPTOM DISORDER

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

ILLNESS ANXIETY DISORDER

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

CONVERSION DISORDER (FUNCTIONAL NEUROLOGIC SYMPTOM DISORDER)

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

FACTITIOUS DISORDER

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

PSYCHOLOGICAL FACTORS AFFECTING OTHER MEDICAL CONDITIONS

Introduction

Epidemiology

Pathobiology

Diagnosis

Management

Outcome

CONCLUSIONS

LEVEL 1 EVIDENCE

SECTION XXII RECOVERY AND END-OF-LIFE CARE

157 Neurologic Rehabilitation

INTRODUCTION

COMPENSATION AND RECOVERY

NEUROPLASTICITY AND RECOVERY

Injury-Induced Plasticity

Activity-Dependent Plasticity

LEVELS OF CARE

THE DISCIPLINES

Physician

Rehabilitation Nursing

Physical Therapy

Occupational Therapy

Speech, Language, and Cognitive Therapy

Psychology and Neuropsychology

Social Work

Recreational Therapy

SPECIALIZED NEUROLOGIC AND MEDICAL MANAGEMENT

Dysphagia

Bladder and Bowel Dysfunction

Medical Care

LEVEL 1 EVIDENCE

158 End-of-Life and Palliative Care in Neurology

INTRODUCTION

Disease Trajectories

PALLIATIVE CARE

ADVANCE DIRECTIVES AND CARE PLANNING

Orders for Life-Sustaining Treatment

Informed Consent and Decision Making

END-OF-LIFE CARE

Life-Sustaining Therapies

Ineffective, Inappropriate, and Futile Therapies

Withholding and Withdrawing

Death, Dying, and Hospice Care

Refusal of Therapy, Physician-Assisted Suicide, and Euthanasia

Other Neurologic Issues With Death

CONCLUSION

Index

 

An aparitie	25/07/2021
Autor	Elan D. Louis MD, MS, Stephan A. Mayer MD, Dr. James M. Noble
Dimensiuni	21.29 x 27.64 cm
Editura	LWW
Format	Hardcover
ISBN	9781975141226
Limba	Engleza
Nr pag	1760