bluuebunny/crossref_metadata_2025_split huggingface dataset:
Supranuclear Ocular Motor Systems
Authors: Eggers Scott D. 
Published: November 2021

Abstract
Properly functioning eye movements facilitate a clear, stable view of the environment. Saccadic eye movements and nystagmus fast phases are 2 types of fast eye movements. Slow eye movements include smooth pursuit, vestibular, optokinetic, and vergence. Reflexive and voluntary conjugate eye movements incorporate cortical, subcortical (basal ganglia), and vestibulocerebellar input to the final common pathways of horizontal and vertical eye movements. The present chapter reviews the anatomy and dysfunction of the supranuclear input to conjugate gaze.
Thalamus
Authors: Flemming Kelly D. , Benarroch Eduardo E. 
Published: November 2021

Abstract
The diencephalon has 4 components: 1) the epithalamus, which includes the pineal body; 2) the dorsal thalamus, which is commonly considered to be the thalamus proper; 3) the ventral thalamus, consisting of the reticular nucleus and subthalamic nucleus; and 4) the hypothalamus. This chapter focuses on the dorsal thalamus and the ventral thalamus. The thalamus has 3 main functions: 1) relay input from subcortical structures to the cortex, 2) control (gate) which sensory information reaches the cortex, and 3) synchronize cortical activity that relates to consciousness.
Principles of Neuroendocrinology and Hypothalamic Function
Authors: Coon Elizabeth A. , Benarroch Eduardo E. 
Published: November 2021

Abstract
The hypothalamus is the neural center of the endocrine system, the regulator of the autonomic nervous system, and the circadian and seasonal clock for behavioral and sleep-wake functions. The hypothalamus maintains homeostasis by integrating cortical, limbic, and spinal inputs and by affecting hormone release, temperature regulation, intake of food and water, sexual behavior and reproduction, emotional responses, and diurnal rhythms. As the link from the nervous system to the endocrine system, the hypothalamus synthesizes and secretes neurohormones that stimulate or inhibit the secretion of pituitary hormones.
Limbic System
Authors: Ramanan Vijay K. , Benarroch Eduardo E. 
Published: November 2021

Abstract
Unlike the lobes of the cortex, which are regionally defined, the limbic “lobe” includes highly interconnected circuits that span cortical, subcortical, and brainstem components. The major functions of the limbic system include mediating emotional and behavioral responses, memory, and learning. From a functional standpoint, the limbic system can be conceptualized as including an anterior circuit, centered in the amygdala and primarily involved in emotion and behavioral drive, and a posterior circuit, centered in the hippocampus and crucial for encoding and retrieving declarative (explicit) memory.
Autonomic Nervous System
Authors: Cutsforth-Gregory Jeremy K. 
Published: November 2021

Abstract
The autonomic nervous system is involved in many important unconscious body functions. It is critical for maintaining the internal environment in response to changes in the external environment. The autonomic nervous system consists of peripheral components (sympathetic and parasympathetic nerves and ganglia) and central components (ventrolateral medulla, nucleus ambiguus, nucleus of the solitary tract, periaqueductal gray, anterior cingulate gyrus, insular cortex, amygdala, and hypothalamus). This chapter briefly reviews the anatomy and functional components of the autonomic nervous system and several anatomical clinical correlations.
Cortex: Topography and Organization
Authors: Caselli Richard J. , Jones David T. 
Published: November 2021

Abstract
The cerebral cortex is involved in various simple and complex activities. It consists of layers of neuronal cell bodies (ie, gray matter) that are organized into gyri (convolutions).The cortex can be divided into functional components in several ways. Various schemes are based on function, cytoarchitecture, topography, or Brodmann areas. The terminology can be confusing because the same area of cortex could be designated by several names. For instance, Brodmann area 17 is also called the primary visual cortex, the striate cortex, and the calcarine cortex. Brodmann designated 52 regions of the cerebral cortex according to cytoarchitecture.
Cellular Processes
Authors: Staff Nathan P. , Madigan Nicolas N. 
Published: November 2021

Abstract
Although cells in the nervous system contain all the cellular machinery that exists in other cells throughout the body, nervous system cells have many specialized functions that present unique challenges in the maintenance of cellular functionality and homeostasis. The unique morphology of neurons demands elaborate cytoarchitecture, energy production, and cellular processing machinery that are unparalleled in other parts of the body. This chapter presents a broad overview of cellular processes related to the cytoskeleton, axonal transport, protein processing, and energy metabolism within the nervous system. Breakdowns of these intricate processes are involved in many diseases of the nervous system, which are discussed in subsequent chapters.
Sleep Pathophysiology
Authors: Anders Gabriel , Lipford Melissa C. 
Published: November 2021

Abstract
Sleep is a natural, reversible, and periodic behavioral state characterized by perceptual inattention and decreased responsiveness to external stimuli. The processes governing sleep, sleep-wake transitions, and maintenance of wakefulness are mediated by complex physiologic mechanisms, the primary neurobiological substrates of which include the neocortex, basal forebrain, thalamus, hypothalamus, pontine tegmentum, and brainstem monoaminergic nuclei. Moreover, the integrity of brainstem autonomic respiratory control networks becomes critical in the maintenance of ventilation during sleep. Pathologic insults to these systems may result in a broad constellation of clinical deficits.
Patterns of Inheritance in Neurogenetic Disease
Authors: Gavrilova Ralitza H. 
Published: November 2021

Abstract
The human genome consists of approximately 22,000 genes that are encoded within the nuclear DNA and embedded in the chromosome. Mitochondria are the only cytoplasmic organelles that have their own DNA. Nuclear gene disorders and mitochondrial inheritance are discussed in this chapter. Nuclear gene disorders follow the patterns of inheritance originally described by Gregor Mendel. They often are referred to as single-gene disorders because 1 or more alleles of only 1 locus are the major determinants of phenotype.
Mood Disorders
Authors: Kung Simon 
Published: November 2021

Abstract
Mood disorders—depressive and bipolar disorders—are the second most common set of psychiatric disorders, behind anxiety disorders. The lifetime prevalence of any mood disorder in US adults is approximately 20%, and the 12-month prevalence is approximately 10%. Although depressive disorders and bipolar disorder have been split into 2 chapters in the Diagnostic and Statistical Manual of Mental Disorders (Fifth Edition), the criteria have stayed the same as in its Fourth Edition, Text Revision while the terminology has changed slightly.
Psychotic Disorders
Authors: Rasmussen Keith G. 
Published: November 2021

Abstract
Common psychotic disorders and their treatment are reviewed in this chapter. The prevalence of schizophrenia is approximately 1%, a figure strikingly similar across countries and cultures. Mean age at onset for men is typically the early 20s, but for women, it is the late 20s. Schizophrenia only rarely remits entirely and is most often a chronic, disabling disorder characterized by exacerbations and partial remissions. It is associated with profound financial costs in terms of long-term needs for all aspects of functioning, including housing, medication, and medical care.
Impaired Consciousness and Coma
Authors: Hawkes Maximiliano A. , Wijdicks Eelco F. M. 
Published: November 2021

Abstract
The diagnosis of impaired consciousness or coma requires a rapid but thorough evaluation. Although impaired consciousness has many causes, clues can be gleaned from history and examination factors to deduce potential causes. Ancillary tests, including brain imaging and laboratory studies, may further aid in the diagnosis of impaired consciousness. Coma has various gradations. When all brain and brainstem functions cease and a known, irreversible brain injury is responsible, the condition is referred to as brain death.
Acute Spinal Cord Compression, Spinal Cord Trauma, and Peripheral Neural Injury
Authors: Marcellino Christopher R. , Wijdicks Eelco F. M. 
Published: November 2021

Abstract
Acute spinal cord compression with myelopathy is a neurologic emergency. Recognition of spinal cord compression, timely imaging, and treatment are important to restore and preserve neurologic function. This chapter reviews the causes and clinical approach to spinal cord compression. Traumatic and nontraumatic causes of spinal cord compression are addressed together because of their overlapping symptoms and management. The chapter concludes with a brief discussion of peripheral nerve injury.
Neuromuscular Disease in the Neuroscience Intensive Care Unit
Authors: Hawkes Maximiliano A. , Wijdicks Eelco F. M. 
Published: November 2021

Abstract
Neurologists may meet patients with neuromuscular disease and subsequent respiratory failure in the inpatient setting. Common clinical conditions include Guillain-Barré syndrome (GBS) and myasthenia gravis (MG). Recognition of impending respiratory failure for such patients is of great importance. This chapter describes general features of neuromuscular weakness and neuromuscular respiratory failure. In addition, it reviews the clinically important aspects of GBS, MG, and botulism. Finally, the chapter outlines the clinical symptoms, diagnosis, and treatment of critical illness polyneuropathy, a commonly encountered condition in the medical intensive care unit.
Diagnosis of Multiple Sclerosis
Authors: Tobin W. Oliver 
Published: November 2021

Abstract
Multiple sclerosis is the most common idiopathic inflammatory demyelinating disease of the central nervous system (CNS), with a prevalence of 1 in 500 to 1 in 2,000 people, depending on geography and various other factors. Idiopathic inflammatory demyelinating diseases are a group of related disorders that include acute disseminated encephalomyelitis, neuromyelitis optica spectrum disorder, and myelin oligodendrocyte glycoprotein–immunoglobulin G–associated CNS demyelinating disease.
Atypical Parkinsonian Syndromes
Authors: Chiu Shannon Y. , Cutsforth-Gregory Jeremy K. 
Published: November 2021

Abstract
The cardinal characteristics of parkinsonism are represented in the mnemonic TRAP: tremor at rest, rigidity, akinesia and bradykinesia, and postural instability. The parkinsonian phenotype encompasses a broad range of clinical and pathologic disorders; the most common (about 55% of cases) is idiopathic (sporadic) Parkinson disease. Rapid disease progression, poor initial response to dopaminergic therapy, or the early presence of certain other signs may suggest an atypical parkinsonian syndrome, sometimes called parkinsonism-plus syndrome.
Hyperkinetic Movement Disorders: Tremor and Myoclonus
Authors: Ali Farwa , Cutsforth-Gregory Jeremy K. 
Published: November 2021

Abstract
Hyperkinetic movement disorders are characterized by excess movement. Tremor and myoclonus are the most common hyperkinetic movement disorders and signify various possible underlying causes or diagnoses. Tremor is an involuntary, rhythmic, oscillatory movement of a body part. It can be distinguished from myoclonus by its regular frequency and from chorea by its stereotyped nature. The subtypes of tremor vary in frequency, conditions of activation and relief, and associated signs and symptoms. Tremor can be primary, being the only manifestation of a condition, or secondary, being symptomatic of a metabolic state or other underlying disease.
Hyperkinetic Movement Disorders: Chorea, Tic, and Dystonia
Authors: Coon Elizabeth A. 
Published: November 2021

Abstract
Hyperkinetic movement disorders are characterized by an excess of movement. This chapter outlines the classification, causes, and treatment of chorea, tic disorders, and dystonia. The term chorea refers to dancelike irregular, arrhythmic, rapid, involuntary movements that appear to flow between muscle groups. Athetosis, a slower and twisting dystonialike movement, may be superimposed with chorea, leading to choreoathetosis. Ballism is a form of high-amplitude and proximal chorea that has the appearance of flailing movement.
Childhood Movement Disorders
Authors: Youssef Paul E. , Mack Kenneth J. 
Published: November 2021

Abstract
The phenomenology and classification of movement disorders are similar for adults and children, but the causes and approach are distinct. In children, hyperkinetic disorders are more common than hypokinetic disorders. Furthermore, some disorders are present exclusively in infants and children; others may begin in childhood and persist into adulthood. This chapter focuses primarily on movement disorders that begin in childhood. Disorders that occur in children or adults without a specific predilection are covered in individual movement disorders sections.
Frontotemporal Dementias
Authors: Townley Ryan , Botha Hugo 
Published: November 2021

Abstract
Frontotemporal dementia (FTD) is a clinically and pathologically heterogeneous group of disorders caused by progressive degeneration of the frontal and temporal lobes. Frontal lobe functions include behavioral and personality control, executive function, and verbal expression. The temporal lobe is associated with behavior and language perception. Thus, the common clinical characterization of FTD is progressive changes in personality and behavior in addition to deficits in executive and language functions. Memory problems are typically not prominent during the early stage of illness.
Parkinsonism and Related Dementias
Authors: Savica Rodolfo , Turcano Pierpaolo , Boeve Bradley F. 
Published: November 2021

Abstract
The differential diagnosis for dementia is wide. A slowly progressive course for parkinsonism suggests a degenerative cause and helps to narrow the differential diagnosis considerably. In patients with dementia in combination with parkinsonism (often collectively termed the parkinsonism-related dementias), the 4 most common neurodegenerative entities are 1) Lewy body dementias (which include dementia with Lewy bodies and Parkinson disease with dementia); 2) corticobasal syndrome or corticobasal degeneration; 3) Richardson syndrome or progressive supranuclear palsy; and 4) frontotemporal dementia with parkinsonism.
Classification of Epilepsy
Authors: Khattak Jamal F. , Burkholder David B. 
Published: November 2021

Abstract
An understanding of the definitions, classification, and key characteristics of seizures and epilepsy is vital in the initial approach to a patient presenting with seizures. Classifying seizures appropriately helps narrow the differential diagnosis and guide further testing, treatment, and prognosis. This chapter reviews the basic definitions and classifications of seizures and epilepsy and summarizes select epilepsy syndromes. A seizure is a transient occurrence of signs or symptoms due to abnormal, excessive, or synchronous neuronal activity in the brain.
Evaluation of Seizures and Seizurelike Events
Authors: Parsons Angela M. , Drazkowski Joseph F. 
Published: November 2021

Abstract
Correctly diagnosing seizures and seizurelike events is important for numerous reasons, including safety issues, social consequences, and therapy. Patients with transient neurologic events of unknown cause are commonly admitted to hospitals, and an estimated 10% of the people in the United States have a seizure in their lifetime. These facts highlight the importance of diagnostic accuracy. History taking is imperfect, but it is still a cornerstone in making the proper diagnosis of transient neurologic events. Focused, supporting diagnostic tests may add accuracy in arriving at the proper diagnosis, but even with a good history, diagnostic testing, and physical examination findings, the diagnosis may be inaccurate. Self-reports of seizure frequency are notoriously inaccurate and often miss more than 50% of focal-onset seizures, especially if the seizures begin in the dominant hemisphere (largely because the effects of the event cause an altered level of consciousness).
Myelopathies
Authors: Kumar Shivram , Flemming Kelly D. 
Published: November 2021

Abstract
A myelopathy is a disorder of the spinal cord. The symptoms and signs of myelopathy can vary depending on which part of the spinal cord is affected and on the cause of the myelopathy. Certain causes of myelopathy tend to affect distinct areas of the spinal cord. The severity of myelopathies can vary even when the causes are identical. This chapter reviews the clinical features and evaluation of myelopathies in general and the specific forms of myelopathy.
Motor Neuron Diseases
Authors: Sorenson Eric J. 
Published: November 2021

Abstract
The motor neuron disorders are a clinically diverse group of diseases that share a pathologic loss of the motor neurons. The most common adult-onset disorder is amyotrophic lateral sclerosis (ALS). Other forms include the spinal muscular atrophies, infectious motor neuronopathies, and rare focal forms of anterior horn cell loss.Overall, the incidence rate of ALS is believed to be 1.5 to 2.0 cases per 100,000 person-years, and the prevalence rate is 4 to 6 cases per 100,000 population. Other than in sparsely populated geographic clusters (eg, Guam and the Kii Peninsula of Japan), the incidence rate seems consistent across ethnic and geographic boundaries.
Inherited Muscle Disorders
Authors: Liewluck Teerin , Milone Margherita 
Published: November 2021

Abstract
Inherited muscular disorders can manifest at any age, from prenatal life to adulthood. The broad differential diagnosis includes muscular dystrophies, congenital myopathies, disorders of glycogen and lipid metabolism, channelopathies, and mitochondrial disorders. Muscular dystrophies may present at any age, are inherited, and involve progressive degeneration of muscle, which is often replaced by connective tissue. Muscular dystrophies result from defects in the sarcolemmal proteins of muscle, including dystrophin-associated muscle membrane protein complex, muscle intracellular proteins (eg, nuclear envelope proteins), and extracellular matrix proteins (eg, collagen VI).
Neuro-ophthalmology: Disorders of Visual Perception, Pupils, and Eyelids
Authors: Kumar Shivram , Flemming Kelly D. 
Published: November 2021

Abstract
Visual loss may develop acutely, subacutely, or insidiously. The course may be transient, static, or progressive. This chapter reviews the causes, diagnosis, and treatment of various disorders resulting in visual loss or abnormal visual perception. In addition, it reviews clinical disorders of the eyelids and pupils. Disorders of visual perception involve visual acuity, color perception, visual field defects, and other visual changes. Historical information and physical findings on examination can help to localize the problem and define the cause.
Neuro-ophthalmology: Extraocular Muscles and Cranial Nerves III, IV, and VI
Authors: Brazis Paul W. , Flemming Kelly D. 
Published: November 2021

Abstract
Conjugate eye movement requires voluntary and reflexive input to the final common pathway (cranial nerves III, IV, and VI and their respective muscles). This chapter briefly repeats information about the pertinent anatomy of the extraocular muscles and cranial nerves III, IV, and VI but focuses on related clinical syndromes or diseases. Supranuclear input to these structures and dysfunction are discussed in other chapters. Six muscles move each eye globe: 4 recti (superior, inferior, medial, and lateral) and 2 oblique (superior and inferior) muscles.
Introduction and Approach to Headache
Authors: Halker Singh Rashmi B. , VanderPluym Juliana H. 
Published: November 2021

Abstract
Headache is experienced by 93% of men and 99% of women at some time in their lives. It is the most common complaint treated by neurologists and the seventh most common complaint treated by primary care providers. Although most headaches in the general population meet the criteria for tension-type headache, migraine is the most common headache treated in clinical practice and affects 18% of women and 6% of men, typically in their peak years of productivity. Evaluation of headache should include a thorough history and general, musculoskeletal, and neurologic examinations. Paramount in the headache evaluation is the proper identification of red flags that may indicate an underlying secondary cause of headache.
Nonglial Central Nervous System Tumors
Authors: Sharma Akanksha , Porter Alyx B. 
Published: November 2021

Abstract
Primary central nervous system lymphoma (PCNSL) is a rare variant of extranodal non-Hodgkin lymphoma that may involve the brain, leptomeninges, eyes, or spinal cord and accounts for up to 5% of all adult primary brain neoplasms. Age-adjusted incidence has increased in the past 3 decades. Infection with HIV and acquired immunodeficiency syndrome increase the risk of PCNSL by 3,600-fold. However, with highly active antiretroviral therapy, the frequency of immune system compromise sufficient for HIV-associated PCNSL (CD4 count ≤50) is dramatically reduced, along with the risk of this disorder.
Spinal Cord Tumors
Authors: Carabenciov I. Darin , Uhm Joon H. 
Published: November 2021

Abstract
Spinal tumors can be broadly classified on the basis of the anatomical compartment they occupy. These tumors can be intramedullary, intradural extramedullary, or extradural. Regardless of location, significant neurologic morbidity can occur as a result of their effects on the spinal cord or the cauda equina, or both. This chapter summarizes the pathophysiologic factors, clinical characteristics, imaging characteristics, and treatment of each tumor type.
DNA and RNA Viral Infections of the Nervous System
Authors: Toledano Michel , Aksamit Jr Allen J. 
Published: November 2021

Abstract
Viruses may cause acute, subacute, or, rarely, chronic infection of the nervous system. The most common acute syndromes of nervous system infections are meningitis and encephalitis, but viruses can also affect the spinal cord and the peripheral nervous system. Viruses can be classified in many ways. One classification scheme assesses the type of nucleic acid (DNA or RNA), whether it is double or single stranded, its sense, and its method of replication.
Fungal Infections of the Central Nervous System
Authors: Wilson John W. 
Published: November 2021

Abstract
Fungal infections within the central nervous system (CNS) occur more commonly in patients with immunosuppressive conditions but occasionally are diagnosed in immunocompetent patients as well. This may reflect the route of infection and the specific pathogen. Fungal infections resulting from hematogenous seeding are typically encountered in patients with profound and prolonged immunosuppression (including neutropenia); however, pathogens such as Cryptococcus gattii and some dimorphic fungi can also produce CNS disease in otherwise healthy persons. A common avenue of infection in immunocompetent patients is traumatic or nosocomial inoculation, including neurosurgical procedures, foreign body implants, and contaminated spinal fluid injections.
Prion Disorders: Creutzfeldt-Jakob Disease and Related Disorders
Authors: Cutsforth-Gregory Jeremy K. 
Published: November 2021

Abstract
Prion disorders, or transmissible spongiform encephalopathies (TSEs), are universally fatal human and animal diseases that cause rapid degeneration of brain neurons by way of a conformational change in the prion protein that autocatalyzes further conformational change and selective neuronal toxicity. TSEs may occur sporadically, be inherited, or, least frequently, spread like an infectious agent. Mounting evidence suggests that degenerative proteinopathies such as Alzheimer disease and Parkinson disease may also involve prionlike spread of abnormal proteins between neurons but not between organisms as in the prion disorders discussed in this chapter.